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Sample records for carcinoides gastrointestinales tratamiento

  1. Carcinoid Tumor

    MedlinePlus

    ... are here Home > Types of Cancer > Carcinoid Tumor Carcinoid Tumor This is Cancer.Net’s Guide to Carcinoid Tumor. Use the menu below to choose the ... social workers, and patient advocates. Cancer.Net Guide Carcinoid Tumor Introduction Statistics Medical Illustrations Risk Factors Symptoms ...

  2. Carcinoid Tumors

    MedlinePlus

    Carcinoid tumors are rare, slow-growing cancers. They usually start in the lining of the digestive tract or in the lungs. They grow ... trouble breathing. Surgery is the main treatment for carcinoid tumors. If they haven't spread to other parts of the body, surgery can cure the cancer.

  3. What Are Lung Carcinoid Tumors?

    MedlinePlus

    ... Carcinoid Tumor About Lung Carcinoid Tumors What Are Lung Carcinoid Tumors? Lung carcinoid tumors (also known as ... lungs, as well as the neuroendocrine system. The lungs The lungs are 2 sponge-like organs in ...

  4. [Carcinoid papillomatosis].

    PubMed

    Cardama, J E; Gatti, J C; Cabrera, H N; Bianchi, O; Garófalo, M

    1976-01-01

    Papilomatosis Carcinoides, presents clinical and histological characteristics that may be authentically described as a transition state between the benign papillomatous proliferations and spindle cell carcinoma. It involves a series of clinical conditions, that are described with different names that correspond to the condyloma accuminatum gigantum. [Buschke and Lowenstein) wartic carcinoma [Ackerman], Papillomatosis oral florid [Rock and Fisher], carcinomatoid [Gilbert] profuse conjuntival papillomatosis [Bazex], etc. These latter only refer to partial localizations or a special clinical form. That is why we believe the denonmination Papillomatosis Carcinoides, is preferable, which is its cutaneous, mucose or semimucose forms involves all of them. The treatments applied to the 18 cases presented have been very diverse: they vary from the elimination of the lesions by the application of topical podophylin to large surgical ablations. But most of them showed a regression or cure of their lesions by citostatics [methotrexate] not prevent the proliferative advance of the lessions. These therapeutic data can also contribute to its nosologic classification. In some cases they have an evolutive character with slow progression for years, still being curable with podophylin and in others that due to their chronic evolution with added supuration, and complications of the general health, make surgery necessary. In most cases, the antimetabolities are the therapy of choice the radiations seem inoperative. It may be noted that tumoral papillomatous lesions produced by virus, exist in veterinatian pathology (fibropapillomatosis in the bovine genitals, equine sarcoid, papilomatosis of goats, monkeys, etc.). Noteworthy are those of the oral mucosa of rabbits which are white greyish sesiles or pedunculated small nodules localized mostly on the inferior surface of tongue with a similar histology to the papilomatosis carcinoides. These observations would suggest a viral ethiology

  5. Carcinoid Cancer Foundation

    MedlinePlus

    ... size). The Latest News Project Zebra Report: Real-World Insights from the Carcinoid and NET Patient Community ... release the first ever Project Zebra Report: Real-World Insights from the Carcinoid and Neuroendocrine Tumor Patient ...

  6. Primary renal carcinoid tumor.

    PubMed

    Kanodia, K V; Vanikar, A V; Patel, R D; Suthar, K S; Kute, V B; Modi, P R; Trivedi, H L

    2013-09-01

    Primary renal carcinoid tumor is extremely rare and, therefore, its pathogenesis and prognosis is not well known. We report a primary renal carcinoid in a 26-year-old man treated by radical nephrectomy.

  7. Carcinoid heart disease.

    PubMed

    Hassan, Saamir A; Banchs, Jose; Iliescu, Cezar; Dasari, Arvind; Lopez-Mattei, Juan; Yusuf, Syed Wamique

    2017-10-01

    Rare neuroendocrine tumours (NETs) that most commonly arise in the gastrointestinal tract can lead to carcinoid syndrome and carcinoid heart disease. Patients with carcinoid syndrome present with vasomotor changes, hypermotility of the gastrointestinal system, hypotension and bronchospasm. Medical therapy for carcinoid syndrome, typically with somatostatin analogues, can help control symptoms, inhibit tumour progression and prolong survival. Carcinoid heart disease occurs in more than 50% of these patients and is the initial presentation of carcinoid syndrome in up to 20% of patients. Carcinoid heart disease has characteristic findings of plaque-like deposits composed of smooth muscle cells, myofibroblasts, extracellular matrix and an overlying endothelial layer which can lead to valve dysfunction. Valvular dysfunction can lead to oedema, ascites and right-sided heart failure. Medical therapy of carcinoid heart disease is limited to symptom control and palliation. Valve surgery for carcinoid heart disease should be considered for symptomatic patients with controlled metastatic carcinoid syndrome. A multidisciplinary approach is needed to guide optimal management. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  8. [Metastatic bronchial carcinoid tumors].

    PubMed

    Bouledrak, K; Walter, T; Souquet, P J; Lombard-Bohas, C

    2016-02-01

    Bronchial carcinoids are uncommon pulmonary neoplasms and represent 1 to 2 % of all lung tumors. In early stage of disease, the mainstay and only curative treatment is surgery. Bronchial carcinoids are generally regarded as low-grade carcinomas and metastatic dissemination is unusual. The management of the metastatic stage is not currently standardized due to a lack of relevant studies. As bronchial carcinoids and in particular their metastatic forms are rare, we apply treatment strategies that have been evaluated in gastrointestinal and pancreatic neuroendocrine tumors. However, bronchial carcinoids have their own characteristic. A specific therapeutic feature of these metastatic tumors is that they require a dual approach: both anti-secretory for the carcinoid syndrome, and anti-tumoral.

  9. How Are Lung Carcinoid Tumors Staged?

    MedlinePlus

    ... Tumor Early Detection, Diagnosis, and Staging How Are Lung Carcinoid Tumors Staged? The stage of a cancer ... Your Doctor About Lung Carcinoid Tumors? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  10. How Are Lung Carcinoid Tumors Diagnosed?

    MedlinePlus

    ... Tumor Early Detection, Diagnosis, and Staging How Are Lung Carcinoid Tumors Diagnosed? Certain signs and symptoms might ... Your Doctor About Lung Carcinoid Tumors? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  11. Pazopanib Hydrochloride in Treating Patients With Progressive Carcinoid Tumors

    ClinicalTrials.gov

    2017-10-09

    Atypical Carcinoid Tumor; Foregut Carcinoid Tumor; Hindgut Carcinoid Tumor; Lung Carcinoid Tumor; Metastatic Carcinoid Tumor; Metastatic Digestive System Neuroendocrine Tumor G1; Midgut Carcinoid Tumor; Recurrent Digestive System Neuroendocrine Tumor G1; Regional Digestive System Neuroendocrine Tumor G1

  12. Primary hepatic carcinoid tumor.

    PubMed

    Gao, Jinbo; Hu, Zhijian; Wu, Junwei; Bai, Lishan; Chai, Xinqun

    2011-11-19

    Primary hepatic carcinoid tumor is rare and poses a challenge for diagnosis and management. We presented a case of primary hepatic carcinoid tumor in a 53-year-old female with a complaint of right upper abdominal pain. Computer tomography scans revealed a hypervascular mass in segment 4 of the liver. An ultrasonography-guided biopsy showed a carcinoid tumor. No other lesions were found by the radiological investigations. Surgery resection was performed and histopathological examination revealed a primary hepatic carcinoid tumor. Three years later, recurrence was found and transcatheter arterial chemoembolization was performed. After transcatheter arterial chemoembolization, the patient has been free of symptom and had no radiological disease progression for over 6 months. Surgical resection combination with transcatheter arterial chemoembolization is effective to offer excellent palliation.

  13. Endobronchial Carcinoid and Concurrent Carcinoid Syndrome in an Adolescent Female

    PubMed Central

    Swanson, Jonathan; Ong, Thida

    2016-01-01

    Endobronchial carcinoid tumors are the most common intrabronchial tumors in children and adolescents. Common signs and symptoms include persistent cough and wheezing not responsive to bronchodilators, hemoptysis, and recurrent fever. Diagnosis is frequently made by imaging and direct visualization with flexible bronchoscopy; surgery remains the gold standard treatment, and lung-sparing resections should be performed whenever possible. Though carcinoid syndrome—characterized by flushing, palpitations, wheezing, shortness of breath, and diarrhea—has been found in association with adult bronchial carcinoid tumors, to our knowledge only one previous study has reported the presence of carcinoid syndrome in a pediatric patient with an endobronchial carcinoid. Here, we report a case of a 14-year-old girl with chronic cough found to have an endobronchial carcinoid tumor and signs and symptoms consistent with carcinoid syndrome. PMID:27895950

  14. What Are the Key Statistics for Lung Carcinoid Tumors?

    MedlinePlus

    ... Carcinoid Tumors What Are the Key Statistics About Lung Carcinoid Tumors? About 1% to 2% of all ... Lung Carcinoid Tumor Research and Treatment? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  15. Treatment of Lung Carcinoid by Type and Extent of Disease

    MedlinePlus

    ... Carcinoid Tumor Treating Lung Carcinoid Tumors Treatment of Lung Carcinoid, by Type and Extent of Disease The ... those that can’t be removed completely Resectable lung carcinoid tumors Resectable carcinoid tumors haven’t spread ...

  16. What's New in Lung Carcinoid Tumor Research and Treatment?

    MedlinePlus

    ... Tumor About Lung Carcinoid Tumors What’s New in Lung Carcinoid Tumor Research and Treatment? Many medical centers ... Lung Carcinoid Tumor Research and Treatment? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  17. What Happens after Treatment for Lung Carcinoid Tumors?

    MedlinePlus

    ... Tumor After Treatment What Happens After Treatment for Lung Carcinoid Tumors? For many people with carcinoid tumors, ... Lung Carcinoid Tumor Treatment Stops Working More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  18. I-131 MIBG imaging of carcinoid tumors

    SciTech Connect

    Blinder, R.A.; Feldman, J.M.; Coleman, R.E.

    1985-05-01

    The potential of I-131 MIBG to image carcinoid tumors was evaluated in nine patients with pathologically proven carcinoid. Three patients with foregut carcinoid tumors (2 bronchial and one pancreatic), had abnormal urinary 5-HIAA, and one had the carcinoid syndrome. Six patients had midgut carcinoid tumors (5 ileal and 1 cecal), 5 had abnormal urinary 5-HIAA, and 4 had the carcinoid syndrome. All of the patients with midgut carcinoids had metestatic disease to the liver and/or mesentery. Two hundred microcuries of I-131 MIBG were injected intravenously and the patients were studied at 24 and 48 hours. In all patients except those with bronchial carcinoids Tc-99m sulfur colloid liver exams with computer subtraction were performed during the study. The patients with bronchial carcinoids showed no uptake in the tumors. The patient with a pancreatic carcinoid who had carcinoid syndrome demonstrated tumor uptake in hepatic metastases. Of the six patients with midgut carcinoids, 4 demonstrated MIBG uptake in tumors. One patient with mesenteric disease did not have definitive tumor uptake. A second patient with liver involvement at surgery was negative on the MIBG study. Hepatic CT and scintigraphy were negative and the patient's 5-HIAA was normal. The 4 patients with carcinoid syndrome had abnormal MIBG scans. I-131 MIBG demonstrated tumor in 4 of six patients with midgut carcinoids. MIBG was not concentrated in two carcinoids of bronchial origin. The five patients with carcinoid syndrome had positive scans. MIBG has potential as a diagnostic agent in carcinoid tumors.

  19. Multiple rectal carcinoid tumors in monozygotic twins.

    PubMed

    Doi, Momoko; Ikawa, Osamu; Taniguchi, Hiroki; Kawamura, Takuji; Katsura, Kanade

    2016-08-01

    We report multiple rectal carcinoid tumors in monozygotic twins who, respectively, had 42 and 36 carcinoid tumors in the lower rectum. This is the first report about carcinoid tumors in monozygotic twins. Both twins developed a similar number of rectal carcinoids with a similar distribution. Investigation of their genetic background may provide information about the origin of these tumors.

  20. Disseminated typical bronchial carcinoid tumor.

    PubMed

    Novković, Dobrivoje; Skuletić, Vesna; Vuković, Jelena; Cerović, Snezana; Tomić, Ilija; Karlicić, Vukojica; Stojisavljević, Marko

    2013-05-01

    Bronchial carcinoids belong to a rare type of lung tumors. If they do not expose outstanding neuroendocrine activity, they develop without clearly visible symptoms. They are often detected during a routine examination. According to their clinical pathological features, they are divided into typical and atypical tumors. Typical bronchial carcinoids metastasize to distant organs very rarely. Localized forms are effectively treated by surgery. The methods of conservative treatment should be applied in other cases. We presented a 65-year-old patient with carcinoid lung tumor detected by a routine examination. Additional analysis (chest X-ray, computed tomography of the chest, ultrasound of the abdomen, skeletal scintigraphy, bronhoscopy, histopathological analysis of the bioptate of bronchial tumor, as well as bronchial brushing cytology and immunohistochemical staining performed with markers specific for neuroendocrine tumor) proved a morphologically typical lung carcinoid with dissemination to the liver and skeletal system, which is very rarely found in typical carcinoids. The presented case with carcinoid used to be showed morphological and pathohistological characteristics of typical bronchial carcinoid. With its metastasis to the liver and skeletal system it demonstrated unusual clinical course that used to be considered as rare phenomenon. Due to its frequent asymptomatic course and varied manifestation, bronchial carcinoid could be considered as a diagnostic challenge requiring a multidisciplinary approach.

  1. What Are the Key Statistics about Gastrointestinal Carcinoid Tumors?

    MedlinePlus

    ... About Gastrointestinal Carcinoid Tumors What Are the Key Statistics About Gastrointestinal Carcinoid Tumors? Although the exact number ... a Gastrointestinal Carcinoid Tumor? What Are the Key Statistics About Gastrointestinal Carcinoid Tumors? What’s New in Gastrointestinal ...

  2. What Are the Risk Factors for Lung Carcinoid Tumors?

    MedlinePlus

    ... and Prevention What Are the Risk Factors for Lung Carcinoid Tumors? A risk factor is anything that ... Can Lung Carcinoid Tumors Be Prevented? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  3. What Should You Ask Your Doctor about Lung Carcinoid Tumors?

    MedlinePlus

    ... Staging What Should You Ask Your Doctor About Lung Carcinoid Tumors? It is important to have honest, ... Your Doctor About Lung Carcinoid Tumors? More In Lung Carcinoid Tumors About Lung Carcinoid Tumors Causes, Risk ...

  4. What Should You Ask Your Doctor about Gastrointestinal Carcinoid Tumors?

    MedlinePlus

    ... What Should You Ask Your Doctor About Gastrointestinal Carcinoid Tumors? It is important to have honest, open ... Doctor About Gastrointestinal Carcinoid Tumors? More In Gastrointestinal Carcinoid Tumors About Gastrointestinal Carcinoid Tumors Causes, Risk Factors, ...

  5. Carcinoid heart disease.

    PubMed

    Flaherty, Anne Marie C

    2014-11-01

    The patient, C.P., is a 59-year-old woman who was diagnosed with metastatic carcinoid of the terminal ileum in May 2003. In June 2003, she underwent an extensive resection including hemicolectomy, cholecystectomy, distal pancreatectomy, and splenectomy with metastatic disease in her pancreas, mesentery, and liver. She had been treated with octreotide, everolimus, oxaliplatin, and multiple hepatic artery embolizations in the past eight years and, most recently, capecitabine and bevacizumab with monthly octreotide. She has had intermittent pleural effusions not requiring intervention and a trace pericardial effusion. Her tumor is functional, meaning it demonstrates hormonal hypersecretion which causes flushing, diarrhea, bronchospasm, and abdominal pain.

  6. Carcinoid Heart Disease in a Primary Ovarian Carcinoid

    PubMed Central

    T., Kolouch; H., Linkova; O., Lang; V., Ciprova; L., Brunerova

    2016-01-01

    Ovarian carcinoids are very rare, and only their insular form is associated with carcinoid syndrome. We herein describe a case report of an elderly woman who presented with typical carcinoid syndrome, which is routinely characterized by right-sided heart failure, diarrhoea, flushes, and other common manifestations. Further examination and biochemical testing of the patient confirmed suspected carcinoid tumor. However, the tumor was surprisingly localized in the left ovary. The presence of the patient’s severe combined tricuspid valve disease would create impossible surgical management conditions, so we decided to first perform cardio-surgery with tricuspid valve replacement. After tumor removal, levels of hydroxyindolacetic acid did not normalize and although the patient was asymptomatic, a small lesion was detected by tectrotyd scan paravertebrally. Treatment with lanreotide led to complete remission with negative biochemical and imaging signs of tumor. Thus, to summarize, carcinoid tumor even in an atypical localization (ovary) should be considered in elderly female patients with severe combined tricuspid valve disease due to carcinoid syndrome. PMID:27122940

  7. Carcinoid Tumor: Frequently Asked Questions

    MedlinePlus

    ... the middle ages in Europe. Centuries of anecdotal observation and more recent experience in carcinoid patients under ... Dr. Kjell Oberg (as well as myself through observation) reports greater than 60% effectiveness from CHRONIC treatment ...

  8. Stages of Gastrointestinal Carcinoid Tumors

    MedlinePlus

    ... cancer cells have spread within the stomach and intestines or to other parts of the body. Staging ... the NCI website . Carcinoid Tumors in the Small Intestine It is not clear what the best treatment ...

  9. Intramedullary spinal metastasis of a carcinoid tumor.

    PubMed

    Kumar, Jay I; Yanamadala, Vijay; Shin, John H

    2015-12-01

    We report an intramedullary spinal cord metastasis from a bronchial carcinoid, and discuss its mechanisms and management. Intramedullary spinal cord metastases from any cancer are rare, and bronchial carcinoids account for only a small fraction of lung cancers. To our knowledge, an intramedullary spinal cord metastasis from a bronchial carcinoid has been described only once previously.

  10. Multiple carcinoid tumors of the rectum: report of two cases suggesting the origin of carcinoid tumors.

    PubMed

    Sasou, Shunichi; Suto, Takeshi; Satoh, Toshihiko; Tamura, Gen; Kudara, Norihiko

    2012-10-01

    Two cases of multiple carcinoid tumors of the rectum with numerous micronests of carcinoid tumors are reported. The patients were 51- and 58-year-old males. Many carcinoid tumors and numerous carcinoid micronests were found in the resected rectum; the total number of carcinoid tumors, groups of micronests, and solitary micronests was 69 in the first case and 62 in the second case. The micronests, consisting of a few to many endocrine cells, were observed in the lamina propria, muscularis mucosa, and/or submucosa. Micronests increased in number, gathered and formed carcinoid tumors, which were up to 8 mm in diameter. It was found that a nest of the carcinoid tumors in the lamina propria showed continuity with the endocrine cells of a crypt in the different carcinoid tumors in both cases. The carcinoid tumor and micronest infiltrated the nerves and ganglions in the muscularis mucosa and submucosa. Nests of the carcinoid tumors and micronests were surrounded by S-100-positive cells. Lymph node metastases of the carcinoid tumor were found in both cases. Rectal carcinoid tumors may originate from endocrine cells of the crypts, and multiple carcinoid tumors may occur heterogeneously. © 2012 The Authors. Pathology International © 2012 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  11. What Happens after Treatment for Gastrointestinal Carcinoid Tumors?

    MedlinePlus

    ... for Gastrointestinal Carcinoid Tumors? For some people with gastrointestinal (GI) carcinoid tumor, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. ...

  12. Duodenal carcinoid tumor - a case report.

    PubMed

    Debnath, C R; Debnath, M R; Haque, M A; Das, S N; Moshwan, M M; Karim, R; Uddoula, M S

    2014-01-01

    Carcinoid tumors are well differentiated neuroendochrine tumors which most frequently involve the gastrointestinal tract; however duodenal carcinoid tumors are rare. They can present with various clinical symptoms and are difficult to diagnose. A 52 years old lady presented with the symptoms of recurrent upper abdominal pain, burning sensation of whole body and passage of loose stool. On endoscopy of upper GIT, there was a duodenal polyp. Polyp was removed by endoscopic resection and tissue was taken for biopsy. Histological findings of biopsy specimen shows carcinoid tumor. As duodenal carcinoid tumor is a rare presentation so we are going to present this case in this article.

  13. [Bronchial carcinoid tumor: study of 60 patients].

    PubMed

    Madrid-Carbajal, Claudia; García-Clemente, Marta; Pando-Sandoval, Ana; Cubillas Martín, Hugo; González-Budiño, Teresa; Casan-Clarà, Pere

    2013-07-21

    To describe the casuistry of bronchial carcinoid tumor in the last 20 years in our hospital and determine survival after surgical treatment. We retrospectively reviewed the medical records from January 1992 to June 2012 of patients diagnosed with carcinoid tumor by the pulmonary service. Fifty-two patients (87%) had typical carcinoid and 8 (13%) atypical carcinoid. The mean age at diagnosis was 60 years (SD: 14.4). There was no relationship between consumption of tobacco and carcinoid tumor. Twenty-two per cent were asymptomatic radiographic finding (incidental finding) Three patients showed carcinoid syndrome and one patient had Cushing syndrome. There was a right dominance and the mean lesion size was between 2.1 and 5 cm. Nine per cent had lymph node involvement, predominantly in atypical carcinoid. Overall survival at 3.5 and 10 years was 94%, 86% and 82%. Survival at 5 years was 90% for typical and 86% for atypical and survival at 10 years was 85% for typical and 57% for atypical carcinoids. Carcinoid tumors are malignant tumors by their ability to metastasize. In our study, both histological type and staging were predictors of survival. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  14. [Intestinal carcinoid tumour: Case report].

    PubMed

    Mussan-Chelminsky, Gil; Vidal-González, Pablo; Núñez-García, Edgar; Valencia-García, Luis César; Márquez-Ugalde, Miguel Ángel

    2015-01-01

    Carcinoid of the small intestine, is a well-differentiated neuroendocrine tumor that rarely presents with clinical signs. This tumour can be associated with other conditions, such as inflammatory bowel disease, presenting a wide range of symptoms. In some cases they have an aggressive and highly symptomatic behaviour; thus, clinical suspicion must be high to make an early diagnosis. A 60 year-old male patient with Crohn's disease and gastrointestinal symptoms attributed to this disease within the last year. He presented with intestinal obstruction initially treated with conservative management with no improvement. Exploratory laparotomy was performed finding a mesenteric tumour that caused the bowel obstruction. Bowel resection with primary anastomosis was performed. The pathology report showed an intestinal carcinoid tumour with lymph node metastases. The patient recovered well, and was discharged without complications to continue medical treatment and follow-up by the Oncology department. In almost 42% of the cases, the most common site of carcinoid tumours is the small intestine, and of these, 41% are presented as locoregional disease. Patients with Crohn's disease present a higher incidence. In these cases, the most common presentation is an acute intestinal obstruction (90%). Surgery is usually curative, and follow up is important as the symptoms of Crohn's disease can hide any recurrence. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  15. Scintigraphic imaging of carcinoid tumors

    SciTech Connect

    Fischer, M.; Kamanabroo, D.

    1985-05-01

    131-1-metaiodobenzylguanidine (131-1-MIBG) is used for scintigraphic localization and treatment of pheochromocytoma and neuroblastoma. Several other tumors, deriving from neuroectoderm (APUD tumors) may also produce catecholamines. 4 patients with surgically proven carcinoid tumors were studied by 131-1-MIBG scintigraphy. Scintigraphic images were performed with a computer assisted gamma camera 2.24, 48 and 72 hours after IV injection of 26 MBq 131-I-MIBG. In one patient single photon emission computed tomography (SPECT) with 185 Mgq 123-I-MIBG was performed additionally. Catecholamines were determined in 24-hours-urinary samples by HPLC. Serotonine was determined in plasma. Catecholamine excretion was normal in all patients, whereas serotonine was elevated in all of them. In 2 of 4 patients slight tracer uptake was observed in some of liver metastases, whereas other metastases in the liver and the primary tumor did not show 131-1-MIBG uptake. In one patient with a carcinoid tumor of the pancreas 131-1-MIBG scintigraphy and SPECT with 123-1-MIBG was positive. In one patient scintigraphy was false negative. MIBG scintigraphy is not only suitable for imaging pheochromocytoma and neuroblastoma, but may also localize carcinoid tumors and their metastases.

  16. Pulmonary carcinoid tumor associated with nephrotic syndrome.

    PubMed

    DePace, N L; Elquezabal, A; Hardenburg, H C

    1980-04-01

    A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid tumor of the lung has previously been reported, to our knowledge.

  17. Liver metastasis secondary to primary mesenteric carcinoid.

    PubMed

    Juanmartiñena Fernández, José Francisco; Fernández Urién, Ignacio; Amat Villegas, Irene; Prieto Martínez, Carlos

    2017-03-01

    Carcinoid tumors are rare, slow-growing neoplasms that display neuroendocrine properties. The term carcinoid is generally applied to well-differentiated neoplasms, while neuroendocrine carcinoma is reserved only for poorly differentiated lesions. They can be found throughout the different regions of the gastrointestinal system, although they have predilection for the ileum.

  18. Combined classical carcinoid and goblet cell carcinoid tumor: a new morphologic variant of carcinoid tumor of the appendix.

    PubMed

    Chetty, Runjan; Klimstra, David S; Henson, Donald E; Albores-Saavedra, Jorge

    2010-08-01

    Carcinoid tumors are the most common neoplasms of the appendix. Histologically they have been categorized as classical, tubular, or goblet cell types. Goblet cell carcinoid has been regarded as a distinctive tumor type, not related to classic carcinoids, and to our knowledge combinations of these 2 tumor types have not been described in detail. In this report, we describe 5 cases of combined classical carcinoid and goblet cell carcinoid (GCC) tumors of the appendix. Four men and 1 woman, (mean age 53.4 y) presented with acute appendicitis (4 cases), whereas 1 presented with a pelvic mass owing to widespread pelvic disseminated disease. The tumors (0.6 to 6.0 cm) were located in the mid-portion and the tip of the appendix. Four patients were treated with right hemicolectomies (the patient with disseminated pelvic and ovarian metastases also had a pelvic exenteration), and 1 was treated with an appendectomy only. Four patients are alive and asymptomatic, whereas the patient with disseminated pelvic disease died 6 months after surgery. All 5 appendiceal tumors had microscopic features of both classical carcinoid and GCC, either intimately admixed or separate but closely apposed. The extent of the 2 components varied, with classical carcinoid representing 60% to 90% of the tumor. Both components stained for the general neuroendocrine markers, however, staining in the classic component was greater. The Mib-1 proliferation index varied from 1-15%, again with higher Mib-1 indices seen in the GCC component of all 5 cases. The pelvic soft tissue and ovarian metastases in case 4 consisted predominantly of a signet ring cell carcinoma with a minor component of goblet cells and was interpreted as an adenocarcinoma ex-GCC. In view of the fact that these combined carcinoid tumors appear to behave more as goblet cell carcinoids, detailed microscopic examination of classical carcinoid tumors of the appendix is suggested and larger series with longer follow-up is required to

  19. [Localised bronchiectasis revealing carcinoid tumor].

    PubMed

    Janah, Hind; Jabri, Hasna; Bopaka, Régis Gothard; Khattabi, Wiam El; Afif, Hicham

    2016-01-01

    We here report the case of a 32-year old female patient with a 5-year history of recurrent purulent bronchial syndrome. Radiological assessment showed a left basithoracic finely ringed bright areas related to cylindrical bronchiectasis located in the left lower lobe. Flexible bronchoscopy showed smooth surface, reddish tumor obstructing the entrance of the left basal pyramid. The patient underwent left lower lobectomy associated with mediastinal lymph node dissection. Histological examination of the surgical specimen was in favor of typical carcinoid tumor. Postoperative outcome was uncomplicated and clinical and radiological evolution was good.

  20. Metastatic carcinoid tumor--atypical presentation.

    PubMed

    Pleşa, Alina; Sarca, Emanuela; Maxim, Roxana

    2014-01-01

    Carcinoid tumor is a slow-growing type of neuroendocrine tumor, originating in the enterochromaffin cells and secreting mainly serotonin. Neuroendocrine tumors (NETs) are found throughout the intestinal tract, the appendix and terminal ileum being the most common locations, and are classified by site of origin and by degree of differentiation, with well-differentiated lesions representing those tumors formerly referred to as carcinoid tumors. The clinical symptoms are characterized by flushing, diarrhea, abdominal pain, and/or bronchial constriction and occur almost exclusively in patients with liver metastases due to the release of bioactive peptides and amines directly into the systemic circulation. We report the clinical, serological and histological diagnosis of a 67-years-old male patient with congestive heart failure secondary to carcinoid heart disease in the context of liver metastases of an ileum carcinoid tumor.

  1. Treatment Option Overview (Gastrointestinal Carcinoid Tumors)

    MedlinePlus

    ... cancer cells have spread within the stomach and intestines or to other parts of the body. Staging ... the NCI website . Carcinoid Tumors in the Small Intestine It is not clear what the best treatment ...

  2. General Information about Gastrointestinal Carcinoid Tumors

    MedlinePlus

    ... cancer cells have spread within the stomach and intestines or to other parts of the body. Staging ... the NCI website . Carcinoid Tumors in the Small Intestine It is not clear what the best treatment ...

  3. Treatment Options for Gastrointestinal Carcinoid Tumors

    MedlinePlus

    ... cancer cells have spread within the stomach and intestines or to other parts of the body. Staging ... the NCI website . Carcinoid Tumors in the Small Intestine It is not clear what the best treatment ...

  4. Primary carcinoid tumor of the cavernous sinus.

    PubMed

    Hood, Brian; Bray, Eric; Bregy, Amade; Norenberg, Michael; Weed, Donald; Morcos, Jacques J

    2014-01-01

    Intracranial carcinoid tumors belong to the class of neuroendocrine tumors and their incidence is extremely rare. The pathogenesis and clinical manifestations of carcinoid tumors of the skull base are outlined in this case report. A 61-year-old multimorbid woman presented with transient memory loss. Computed tomographic and magnetic resonance imaging scan of the brain demonstrated a left cavernous sinus mass extending into the infratemporal fossa. The lesion was biopsied using the Caldwell-Luc approach, and histology showed a low-grade neuroendocrine tumor. The tumor was subtotally resected with a neurosurgery/head and neck combined preauricular infratemporal and subtemporal extradural approaches to the cavernous sinus. Further histologic evaluation revealed that the tumor was of carcinoid differentiation with no other primary or metastatic sites detectable. Primary intracranial carcinoid tumors, though rare, should be included in the differential diagnosis of extradural and dural-based lesions. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. [Appendiceal carcinoid tumors. Evaluation of long-term outcomes in a tertiary level].

    PubMed

    Suárez-Grau, Juan Manuel; García-Ruiz, Salud; Rubio-Chaves, Carolina; Bustos-Jiménez, Manuel; Docobo-Durantez, Fernando; Padillo-Ruiz, Francisco Javier

    2014-01-01

    Antecedentes: los tumores apendiculares se encuentran en cerca de 1% de las apendicectomías y representan 0.5% de las neoplasias intestinales. El tipo de tumor más frecuente es el carcinoide apendicular, que casi siempre es un hallazgo durante la apendicectomía por otro motivo. Su pronóstico es excelente y la supervivencia es mayor de 95% a cinco años de la intervención. Objetivo: reportar una serie de casos y analizar la supervivencia media a cinco años posteriores a la identificación el tumor. Material y métodos: análisis retrospectivo (1990-2010) de pacientes con tumor carcinoide apendicular intervenidos en el servicio de Cirugía General y del Aparato Digestivo del Hospital Universitario Virgen del Rocío, Sevilla, España. Se analizaron: la supervivencia a cinco años, la necesidad de tratamiento complementario y las pruebas para seguimiento en la consulta. Resultados: se encontraron 42 pacientes intervenidos por tener un tumor carcinoide apendicular. En 38 pacientes la operación fue de urgencia, la mayoría por sospecha de apendicitis aguda, sin que en ninguno se hubiera establecido el diagnóstico de tumor carcinoide antes de la operación. El síntoma predominante al ingreso fue el dolor abdominal. El tratamiento quirúrgico fue: apendicectomía en 34 pacientes (12 por laparoscopia), en el intraoperatorio siete pacientes requirieron resecciones colónicas mayores debido a la afectación del colon; sólo uno requirió la reintervención para completar la hemicolectomía derecha. Al momento del diagnóstico dos pacientes tenían enfermedad diseminada (metástasis hepáticas). La supervivencia a cinco años fue superior a 95%, sin recidivas o tratamiento posterior de la enfermedad. Conclusiones: el tumor carcinoide apendicular difícilmente se diagnostica antes del procedimiento quirúrgico. La apendicectomía suele ser suficiente aunque en algunos pacientes las resecciones colónicas son necesarias por diseminación. La supervivencia a 5 años es

  6. Bioprosthetic tricuspid valve replacement in carcinoid heart disease from primary ovarian carcinoid tumor.

    PubMed

    Tsugu, Toshimitsu; Iwanaga, Shiro; Murata, Mitsushige; Fukuda, Keiichi

    2015-07-01

    Carcinoid heart disease (CHD) commonly occurs in association with primary gastrointestinal tract carcinoid tumors with hepatic metastases. Unlike primary gastrointestinal tract carcinoid tumors, primary ovarian carcinoid tumors may cause CHD without hepatic metastases, accounting for only 0.3 % of all carcinoid tumors. Only 37 cases of CHD from primary ovarian carcinoid tumors have been reported. We present a case of CHD in which tricuspid valve thickening and shortening led to reduced valve mobility with the resulting severe tricuspid regurgitation. Considering these characteristics of an abnormal tricuspid valve, we suspected CHD, but prosthetic valve replacement was performed without sufficient systemic examination before surgery. Two years after valve replacement, the patient underwent excision of a mass in the lower abdomen, which was diagnosed as an ovarian carcinoid tumor by histopathological examination. The patient has been observed for more than 3 years after tricuspid valve replacement. She has not experienced bioprosthetic valve leaflet degeneration or dysfunction, although it has been reported that bioprosthetic valves may degenerate in patients with carcinoid tumors. Sufficient systemic examinations should be performed to explore the cause of disease.

  7. Primary pulmonary carcinoid tumor: a long-term single institution experience/Primary pulmonary carcinoid tumor

    PubMed Central

    Herde, Ryan F.; Kokeny, Kristine E.; Reddy, Chakravarthy B.; Akerley, Wallace L.; Hu, Nan; Boltax, Jonathan P.; Hitchcock, Ying J.

    2015-01-01

    Objectives Primary carcinoid tumors of the lung are rare tumors which comprise approximately 0.5 to 5% of all lung malignancies in adults and roughly 20 to 30% of all carcinoid tumors. The purpose of this retrospective, descriptive study was to describe the incidence, characteristics, and outcomes of patients treated for primary pulmonary carcinoid tumor at a single institution. Methods All patients with a diagnosis of primary pulmonary carcinoid tumor treated from 1989 to 2009 were reviewed. Data collected included demographics, pathology, tobacco use, clinical presentation, tumor location, tumor spread, treatment and survival. Results There were 59 cases of pulmonary carcinoid tumors: 47 typical (80%) and 12 atypical (20%). All but 4 patients underwent surgery, including 54 (92%) lung-sparing resections and 1 pneumonectomy. Five out of 55 patients received concurrent adjuvant chemoradiation therapy; 4 patients with atypical and 1 with typical histology. Three additional patients with atypical carcinoid were treated only with adjuvant radiotherapy, palliative radiotherapy, or palliative chemotherapy, respectively. The Kaplan-Meier 5- and 10-year overall survivals were both 80% within the entire population. In the 88% of patients who achieved complete remission, disease-free survival was 98%. A review of a large series from the literature is also presented. Conclusions Surgical resection was primary and adequate therapy for most typical carcinoid tumors with high overall survival and disease-free survival. Adjuvant chemotherapy or radiotherapy might be considered for patients with atypical carcinoid tumors who present with adverse pathological findings. PMID:26270444

  8. Métastases gastro-intestinales du cancer du sein: à propos de 2 cas

    PubMed Central

    Loubna, Mezouar; Mohamed, El Hfid; Tijani, El Harroudi; Fouzia, Ghadouani; Hanane, Haj Kacem; Zouhour, Bourhaleb; Asmae, Ouabdelmoumen

    2013-01-01

    Le cancer du sein est le cancer le plus fréquent chez la femme, notamment au Maroc, avec un taux de mortalité élevé. Les métastases gastro-intestinales d'un carcinome canalaire du sein sont rares. Leur diagnostic est difficile du fait de la nature non spécifique des symptômes. Nous rapportons deux observations originales de métastases gastroduodénales d'un cancer canalaire infiltrant du sein. Les métastases gastro-intestinales du cancer du sein sont très rares; la présence de symptômes gastro-intestinaux chez une malade ayant un antécédent de cancer du sein doit faire suspecter une atteinte métastatique gastro-intestinale. PMID:24198876

  9. CDX2 may be a useful marker to distinguish primary ovarian carcinoid from gastrointestinal metastatic carcinoids to the ovary.

    PubMed

    Desouki, Mohamed Mokhtar; Lioyd, Joshua; Xu, Haodong; Cao, Dengfeng; Barner, Ross; Zhao, Chengquan

    2013-11-01

    Primary ovarian carcinoids and metastatic tumors share similar morphologic features. Metastatic carcinoids must be excluded from primary ones for prognostic and therapeutic reasons. Gastrointestinal neuroendocrine (carcinoid) tumors are much more common with the majority arising from small intestine and appendix. The aim of this study is to evaluate the role of immunohistochemistry for CDX2 in differentiating primary ovarian from metastatic carcinoids of primary gastrointestinal origin. Thirty primary pure ovarian carcinoids, 16 primary ovarian carcinoids arising in association with benign teratomas, 10 ovarian carcinoids metastatic from primary gastrointestinal tract and 70 gastrointestinal neuroendocrine tumors were studied for the expression of CDX2 by immunohistochemistry. CDX2 expression revealed that 40 (57.1%) of 70 cases of gastrointestinal carcinoids and 9 (90%) of 10 ovarian metastatic carcinoids showed positive nuclear staining (diffuse or focal). On the other hand, 3 (18.8%) of 16 primary carcinoids with teratomatous elements showed weak positivity. Among the 70 gastrointestinal carcinoids, CDX2 was positive in 38 (90.5%) of 42 cases in the duodenum, small intestine, appendix, and only in 2 (11.8%) of 17 cases of colorectal carcinoids and none of the 11 cases in the stomach. It is concluded that CDX2 may be a useful marker to distinguish primary ovarian carcinoid from metastasis from small intestinal and appendiceal neuroendocrine tumors.

  10. Do We Know What Causes Gastrointestinal Carcinoid Tumors?

    MedlinePlus

    ... and Prevention Do We Know What Causes Gastrointestinal Carcinoid Tumors? Researchers have made great progress in understanding ... than having been inherited. The mutations that cause carcinoid tumors often affect the MEN1 gene, the same ...

  11. Carcinoid tumor with bilateral renal involvement in a child.

    PubMed

    Warrier, Raj P; Varma, Aditi Vian; Chauhan, Aman; Ward, Ken; Craver, Randal

    2011-12-01

    Carcinoid tumors are uncommon in children. Kidneys are rarely involved as they do not possess neuro-endocrine cells. Work up of painless hematuria after abdominal trauma in a 10-year-old boy revealed primary carcinoid tumors with metastasis to both kidneys. We were unable to find any previous reports of renal involvement by carcinoid tumor in children.

  12. An Unusual Case of Rectal and Ileal Carcinoid Tumors

    PubMed Central

    Abdulsamad, Molham; Abbas, Naeem; Balar, Bhavna

    2016-01-01

    Carcinoid tumor is the most common neuroendocrine tumor affecting the gastrointestinal tract. The coexistence of multifocal carcinoid lesions is a well-established phenomenon. Although intubation of the terminal ileum is not routinely attempted during colonoscopy, it can occasionally reveal the presence of some incidental findings. We present a patient with known rectal carcinoid, who was found to have another carcinoid lesion in the terminal ileum during surveillance colonoscopy. The patient underwent right hemicolectomy, and no chemotherapy was required as the patient was found to have stage 1 carcinoid tumor. PMID:28203126

  13. PHACES syndrome associated with carcinoid endobronchial tumor.

    PubMed

    Mama, Nadia; H'mida, Dorra; Lahmar, Imen; Yacoubi, Mohamed Tahar; Tlili-Graiess, Kalthoum

    2014-05-01

    PHACES syndrome consists of the constellation of manifestations including posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), hemangiomas of the face and scalp, arterial abnormalities, cardiac defects, eye anomalies and sternal defects. We present a case with a possible PHACES syndrome including sternal cleft and supraumbilical raphé, precordial skin tag, persistent left superior vena cava and subtle narrowing of the aorta with an endobronchial carcinoid tumor. All these anomalies were discovered on chest multi-detector CT. This is a unique case of PHACES syndrome associated with carcinoid tumor. Review of the literature revealed 3 cases of PHACES syndrome with glial tumor. The authors tried to find the relationship between PHACES syndrome and carcinoid tumors or gliomas, which all derive from the neural crest cells.

  14. Metastatic carcinoid tumor obstructing left ventricular outflow.

    PubMed

    Chrysant, George S; Horstmanshof, Douglas A; Guniganti, Uma M

    2011-01-01

    Cardiac tumors are rare and usually indicate metastatic disease. Characterizing a tumor and reaching an exact diagnosis can be difficult. Diagnosis has been aided greatly by advances in imaging, such as cardiovascular magnetic resonance with the use of gadolinium-pentetic acid. Carcinoid tumors are neuroendocrine neoplasms that are found most often in the intestinal tract, although they can also develop in the lung, stomach, or heart. Herein, we report the case of a 72-year-old woman with a history of intestinal carcinoid disease and presenting symptoms of dizziness, fatigue, and chest pain. We used cardiovascular magnetic resonance with gadolinium enhancement to identify a large mass obstructing left ventricular outflow. The histopathologic results of an endomyocardial biopsy confirmed that the mass was a left-sided metastatic carcinoid cardiac tumor. To our knowledge, we are reporting the 1st combined use of clinical evaluation, cardiovascular magnetic resonance, and histopathologic studies to reach such a diagnosis.

  15. Primary renal carcinoid tumor: A radiologic review

    PubMed Central

    Lamb, Leslie; Shaban, Wael

    2015-01-01

    Carcinoid tumor is the classic famous anonym of neuroendocrine neoplasms. Primary renal carcinoid tumors are extremely rare, first described by Resnick and colleagues in 1966, with fewer than a total of 100 cases reported in the literature. Thus, given the paucity of cases, the clinical and histological behavior is not well understood, impairing the ability to predict prognosis. Computed tomography and (occasionally) octreotide studies are used in the diagnosis and followup of these rare entites. A review of 85 cases in the literature shows that no distinctive imaging features differentiate them from other primary renal masses. The lesions tend to demonstrate a hypodense appearance and do not usually enhance in the arterial phases, but can occasionally calcify. Octreotide scans do not seem to help in the diagnosis; however, they are more commonly used in the postoperative followup. In addition, we report a new case of primary renal carcinoid in a horseshoe kidney. PMID:27186242

  16. Metastatic Carcinoid Tumor Obstructing Left Ventricular Outflow

    PubMed Central

    Chrysant, George S.; Horstmanshof, Douglas A.; Guniganti, Uma M.

    2011-01-01

    Cardiac tumors are rare and usually indicate metastatic disease. Characterizing a tumor and reaching an exact diagnosis can be difficult. Diagnosis has been aided greatly by advances in imaging, such as cardiovascular magnetic resonance with the use of gadolinium-pentetic acid. Carcinoid tumors are neuroendocrine neoplasms that are found most often in the intestinal tract, although they can also develop in the lung, stomach, or heart. Herein, we report the case of a 72-year-old woman with a history of intestinal carcinoid disease and presenting symptoms of dizziness, fatigue, and chest pain. We used cardiovascular magnetic resonance with gadolinium enhancement to identify a large mass obstructing left ventricular outflow. The histopathologic results of an endomyocardial biopsy confirmed that the mass was a left-sided metastatic carcinoid cardiac tumor. To our knowledge, we are reporting the 1st combined use of clinical evaluation, cardiovascular magnetic resonance, and histopathologic studies to reach such a diagnosis. PMID:21720473

  17. Carcinoid Heart Disease without Liver Involvement Caused by a Primary Ovarian Carcinoid Tumour.

    PubMed

    Agarwal, Chirag; Goel, Sunny; Stern, Eric; Warner, Richard; Castillo, Javier; Croft, Lori; Lavine, Ronald; Zacks, Jerome

    2015-07-01

    Carcinoid heart disease, caused by primary ovarian carcinoid tumour, is a rare form of valvular heart disease. This form of heart disease usually presents with symptoms of right-sided valvular dysfunction, ultimately leading to right-sided heart failure. This entity is unique as it develops in the absence of liver metastasis. We report a case of 75 year-old woman with primary ovarian carcinoid tumour who presented with symptoms of severe right-sided heart failure and successfully underwent pulmonic and tricuspid valve replacement along with a right ventricular (RV) outflow patch enlargement. This patient later underwent uneventful resection of the primary ovarian carcinoid tumour, with complete resolution of her symptoms.

  18. Carcinoid Syndrome and Carcinoid Heart Disease as Manifestations of Non-Metastatic Ovarian Neuroendocrine Tumour.

    PubMed

    Simões-Pereira, Joana; Wang, Lai Mun; Kardos, Attila; Grossman, Ashley

    2017-05-31

    The carcinoid syndrome is rare but it is associated with carcinoid heart disease in more than a half of the cases. Carcinoid heart disease is typically characterised by morphological and functional modifications of right-sided valves. Its aetiology is probable multifactorial but serotonin appears to play a key role in the development of this valvular disease. Unlike gastrointestinal neuroendocrine tumours, ovarian neuroendocrine tumours can present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases; such ovarian neuroendocrine tumours are a unique clinical entity. The additional burden of cardiac impairment in these patients represents a significant reduction in survival. Early recognition and surgical valve replacement before advanced heart failure is established may improve the clinical outcome. We report the case of a woman with an ovarian neuroendocrine tumour and highly symptomatic carcinoid heart disease who was submitted to tumour resection followed by valvuloplasty. She demonstrated an outstanding clinical improvement and has remained free of tumour and symptomatology.

  19. Therapeutic options for gastrointestinal carcinoids.

    PubMed

    Modlin, Irvin M; Latich, Igor; Kidd, Mark; Zikusoka, Michelle; Eick, Geeta

    2006-05-01

    Although wide surgical resection is the optimal curative therapy for carcinoid tumors, in most patients the presence of metastatic disease at diagnosis usually renders excision a palliative procedure. This nevertheless decreases tumor burden, facilitates symptom control, and prevents complications caused by bleeding, perforation, or bowel obstruction resulting from fibrosis. In the stomach (types I and II) and rectum endoscopic excision may be adequate provided the lesion(s) are local. Long-term therapy is focused on symptom alleviation and improvement of quality of life using somatostatin analogues, particularly in a subcutaneous depot formulation. In some instances interferons may have a role but their usage often is associated with substantial adverse events. Conventional chemotherapy and external radiotherapy either alone or in a variety of permutations are of minimal efficacy and should be balanced against the decrease in quality of life often engendered by such agents. Hepatic metastases may be amenable to surgery, radiofrequency ablation, or embolization either alone or in combination with chemotherapeutic agents or isotopically loaded microspheres. Rarely hepatic transplantation may be of benefit although controversy exists as to its actual use. Peptide-receptor-targeted radiotherapy for advanced disease using radiolabeled octapeptide analogs (111In/90Yt/177Lu-octreotide) appear promising but data are limited and its status remains investigational. A variety of antiangiogenesis and growth factor-targeted agents have been evaluated, but as yet have shown little promise. The keystone of current therapy remains the long-acting somatostatin analogues that alleviate symptomatology and substantially improve quality of life with minimal adverse effects.

  20. [Case of primary renal carcinoid tumor with hemorrhage].

    PubMed

    Kubota, Yasuaki; Seike, Kensaku; Maeda, Shinichi; Tashiro, Kazuhiro

    2010-04-01

    Carcinoid tumors are low-grade malignant tumors that arise from neuroendocrine cells. Primary renal carcinoid tumors are extremely uncommon. A 63-year-old woman presented with a right abdominal mass and fever. Abdominal computed tomography demonstrated a mass in the right kidney; the mass measured 120 mm in diameter and showed hemorrhage. The patient underwent an uneventful right radical nephrectomy, and histological appearance was typical of carcinoid tumor. Immunohistochemistry demonstrated strong cytoplasmic labeling for neuron-specific enolase and synaptophysin. Additional examinations of the gastrointestinal tract did not show any evidence of carcinoid tumors. The patient remains free from disease recurrence at 8 months after the operation. The prognosis for primary renal carcinoid tumor is relatively optimistic. Complete surgical excision is the only recommended treatment for localized renal carcinoid tumor.

  1. Limitation of liver function tests in metastatic carcinoid tumors

    SciTech Connect

    Moinuddin, M.; Dean, P.; Vander Zwaag, R.; Dragutsky, M.

    1987-04-01

    To evaluate the utility of liver function tests (LFT) as indicators of metastatic carcinoid tumors, a retrospective study was performed. The LFT results of 17 patients with carcinoid tumors metastatic to the liver were compared with 17 patients with other malignant tumors. In the noncarcinoid group, 82.4% of the patients had elevated alkaline phosphatase (AP) or gamma glutamyl transpeptidase (GGTP), whereas only 28.6% of carcinoid patients had abnormal enzymes. The medians of all LFT values were significantly higher in noncarcinoid patients than in the carcinoid group, except for glutamic pyruvic transaminase (SGPT). Our data indicate that LFT are helpful in screening for liver metastases in patients with noncarcinoid tumors, but are unreliable in carcinoid tumors. Imaging tests should be used to rule out liver metastases in carcinoid tumors, irrespective of LFT results.

  2. Peptide YY producing strumal carcinoid tumor of the ovary.

    PubMed

    Matsunami, K; Takagi, H; Ichigo, S; Murase, T; Ikeda, T; Imai, A

    2011-01-01

    Primary ovarian carcinoid tumor is uncommon and represents less than 0.1% of ovarian malignancies. The strumal carcinoid may be complicated by carcinoid syndrome induced by peptide YY (PYY). We describe a 45-year-old woman with a bilateral ovarian tumor diagnosed through periodical gynecological examination. She presented with severe constipation. Right ovarian cyst laparoscopically resected was diagnosed as a strumal carcinoid tumor; the left one was mature cystic teratoma. No metastatic findings were seen macroscopically on the ovarian surface and pelvic peritoneum. Constipation was drastically improved by resecting the tumor. The carcinoid tumor cells were positive for tumor-producing PYY by mRNA analysis. It is important to be aware of this entity in the pathological diagnosis of ovarian tumors, in the presence of any clinical indicator of carcinoid tumor/syndrome, as it carries a markedly better prognosis and clinical outcome in comparison with most other malignant ovarian tumors.

  3. Carcinoid tumor and intravenous octreotide infusion during labor and delivery.

    PubMed

    Le, B T; Bharadwaj, S; Malinow, A M

    2009-04-01

    There are limited numbers of reports concerning the management of pregnancy complicated by carcinoid tumors. Octreotide, the synthetic analogue of somatostatin, has been found to be beneficial in preventing the perioperative exacerbation of carcinoid syndrome. We present a case of the successful use of neuraxial analgesia/anesthesia for labor and vaginal delivery in a symptomatic parturient afflicted with carcinoid syndrome, who received an intravenous infusion of octreotide throughout labor and vaginal delivery.

  4. Epidemiology of carcinoid neoplasms in Vaud, Switzerland, 1974–97

    PubMed Central

    Levi, F; Te, V-C; Randimbison, L; Rindi, G; La Vecchia, C

    2000-01-01

    In Vaud, Switzerland, the incidence of carcinoids based on 218 malignant and 215 benign cases rose from 19.6/106in 1974–85 to 28.2/106in 1986–97, more so among males and malignant neoplasms. Lung was the commonest site for malignant and large intestine for benign carcinoids. Sixty-eight (16%) carcinoids had another neoplasm. © 2000 Cancer Research Campaign PMID:10970700

  5. Fine needle aspiration cytology of thymic carcinoid tumor.

    PubMed

    Wang, D Y; Kuo, S H; Chang, D B; Yang, P C; Lee, Y C; Hsu, H C; Luh, K T

    1995-01-01

    Carcinoid tumors of the thymus are very rare, and their cytologic findings have not been reported previously in English. Retrospective study of fine needle aspiration (FNA) cytologic features in four histopathologically verified thymic carcinoid tumors are described here in detail. The FNA cytology of thymic carcinoids is characterized by predominantly single and some loose clusters of small, round to oval cells with scanty cytoplasm, interspersed with some larger cells with moderate to abundant, granular cytoplasm. The differential diagnosis of the cytologic features between carcinoid tumor and other mediastinal tumors is also discussed.

  6. Valvular Disorders in Carcinoid Heart Disease

    PubMed Central

    Yuan, Shi-Min

    2016-01-01

    Carcinoid heart disease is a rare but important cause of intrinsic right heart valve disorders leading to right heart failure. Occasionally, left-sided heart valves may also be involved. The characteristic cardiac pathological findings of carcinoid heart disease are endocardial thickening as a result of fibrous deposits on the endocardium. Echocardiographic examination and right heart catheterization are very useful for the diagnosis of the lesion. If more cardiac valves are affected, multiple valve replacement should be considered. The management of the pulmonary valve lesion depends on the extent of the diseased valve, either by valvulotomy, valvectomy, or valve replacement. Percutaneous valve implantations in the pulmonary and in the inferior vena cava positions have been advocated for high-risk patients. PMID:27982350

  7. Primary Hepatic Carcinoid Tumor with Poor Outcome.

    PubMed

    Parkash, Om; Ayub, Adil; Naeem, Buria; Najam, Sehrish; Ahmed, Zubair; Jafri, Wasim; Hamid, Saeed

    2016-03-01

    Primary Hepatic Carcinoid Tumor (PHCT) represents an extremely rare clinical entity with only a few cases reported to date. These tumors are rarely associated with metastasis and surgical resection is usually curative. Herein, we report two cases of PHCT associated with poor outcomes due to late diagnosis. Both cases presented late with non-specific symptoms. One patient presented after a 2-week history of symptoms and the second case had a longstanding two years symptomatic interval during which he remained undiagnosed and not properly worked up. Both these cases were diagnosed with hepatic carcinoid tumor, which originates from neuroendocrine cells. Case 1 opted for palliative care and expired in one month’s time. Surgical resection was advised to the second case, but he left against medical advice.

  8. Metastatic carcinoid tumour with spinal cord compression.

    PubMed

    Scott, Si; Antwi-Yeboah, Y; Bucur, Sd

    2012-07-01

    Carcinoid tumours are rare with an incidence of 5.25/100,000. They predominantly originate in the gastrointestinal tract (50-60%) or bronchopulmonary system (25-30%). Common sites of metastasis are lymph nodes, liver, lungs and bone. Spinal metastasis are rare, but has been reported in patients with symptoms of spinal cord compression including neurological deficits. We report a rare case of carcinoid metastasis with spinal cord compression, in a 63-year-old man, presenting with a one-year history of back pain without any neurological symptoms. The patient underwent a two-level decompressive laminectomy of T10 and T11 as well as piecemeal tumour resection. Post-operatively the patient made a good recovery without complications.

  9. Metastatic carcinoid tumour with spinal cord compression

    PubMed Central

    Scott, SI; Antwi-Yeboah, Y; Bucur, SD

    2012-01-01

    Carcinoid tumours are rare with an incidence of 5.25/100,000. They predominantly originate in the gastrointestinal tract (50-60%) or bronchopulmonary system (25-30%). Common sites of metastasis are lymph nodes, liver, lungs and bone. Spinal metastasis are rare, but has been reported in patients with symptoms of spinal cord compression including neurological deficits. We report a rare case of carcinoid metastasis with spinal cord compression, in a 63-year-old man, presenting with a one-year history of back pain without any neurological symptoms. The patient underwent a two-level decompressive laminectomy of T10 and T11 as well as piecemeal tumour resection. Post-operatively the patient made a good recovery without complications. PMID:24960730

  10. Carcinoid Tumor in Accidental, Asymptomatic Meckel's Diverticulum.

    PubMed

    Baranyai, Zsolt; Jósa, Valeria; Merkel, Keresztely; Zolnai, Zsofia

    2013-01-01

    Although Meckel's diverticulum is the most common congenital gastrointestinal disorder, it is controversial whether asymptomatic diverticula in adults should be respected. The authors report the case of a patient who was operated due to ileus caused by adhesions and a Meckel's diverticulum without any sign of inflammation was accidentally noted and removed. As a surprise, the pathological examination of the diverticulum proved carcinoid tumor, a neuroendocrine malignant tumor. The case raises the importance of the removal of asymptomatic Meckel's diverticulum.

  11. Carcinoid Tumor in Accidental, Asymptomatic Meckel's Diverticulum

    PubMed Central

    Baranyai, Zsolt; Jósa, Valeria; Merkel, Keresztely; Zolnai, Zsofia

    2013-01-01

    Although Meckel's diverticulum is the most common congenital gastrointestinal disorder, it is controversial whether asymptomatic diverticula in adults should be respected. The authors report the case of a patient who was operated due to ileus caused by adhesions and a Meckel's diverticulum without any sign of inflammation was accidentally noted and removed. As a surprise, the pathological examination of the diverticulum proved carcinoid tumor, a neuroendocrine malignant tumor. The case raises the importance of the removal of asymptomatic Meckel's diverticulum. PMID:24470856

  12. Surgical management of carcinoid heart valve disease.

    PubMed

    Castillo, Javier G; Milla, Federico; Adams, David H

    2012-01-01

    Carcinoid tumors are neuroendocrine tumors with an unpredictable clinical behavior. In the setting of hepatic metastases, the release of bioactive amines from the tumor into the systemic circulation results in carcinoid syndrome: a constellation of clinical symptoms, among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most frequent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe endocardial fibrotic reaction that leads to progressive valve thickening and retraction. Imaging studies commonly reveal severe right-sided valve disease, with fixed leaflets or cusps in a semiopen position. The replacement of the right-sided valves, including the patch enlargement of the right ventricular outflow tract, is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend toward improved surgical outcomes has triggered a more liberal referral for valve replacement during the past decade. Copyright © 2012 Elsevier Inc. All rights reserved.

  13. Esophageal carcinoid tumor treated by endoscopic resection.

    PubMed

    Yagi, Makoto; Abe, Yasuhiko; Sasaki, Yu; Nomura, Eiki; Sato, Takeshi; Iwano, Daisuke; Yoshizawa, Kazuya; Sakuta, Kazuhiro; Kanno, Nana; Nishise, Syouichi; Ueno, Yoshiyuki

    2015-05-01

    The present report describes a rare case of esophageal carcinoid tumor that was treated by endoscopic resection. A 43-year-old woman underwent esophagogastroduodenoscopy at her family clinic for screening of the upper digestive tract and a small lesion resembling a submucosal tumor was detected in the lower esophagus. A biopsy sample from the lesion was diagnosed as esophageal carcinoid tumor and the patient visited our hospital for detailed examination. The tumor was approximately 3 mm in diameter and its surface appeared to be covered with normal squamous epithelium. The tumor had a shiny reddish surface without ulceration or erosion. Magnifying endoscopy with narrow-band imaging showed structures resembling reticular vessels under the epithelium. Endoscopic ultrasonography depicted the tumor as a low-echoic mass within the lamina propria. Computed tomography did not detect the tumor and no metastatic lesions were evident in other organs. With the patient's informed consent, the tumor was resected using endoscopic submucosal dissection, with a sufficient free margin in both the vertical and horizontal directions. Magnifying endoscopic examination showed the resected tumor to have abundant reticular vessels. Finally, the tumor was diagnosed immunopathologically as an esophageal carcinoid tumor (neuroendocrine cell tumor, grade 1), without lymphatic or vascular invasion.

  14. Primary pulmonary carcinoid tumor with metastasis to endometrial polyp

    PubMed Central

    Momeni, Mazdak; Kolev, Valentin; Costin, Dan; Mizrachi, Howard H.; Chuang, Linus; Warner, Richard R.P.; Gretz, Herbert F.

    2012-01-01

    INTRODUCTION A carcinoid tumor occurring in the endometrium has been documented in the literature, but there is no report in regard to carcinoid tumor metastasis to endometrium. PRESENTATION OF CASE We report a case of a malignant carcinoid metastasis to an endometrial polyp. Patient underwent hysteroscopy, and polypectomy. The pathology demonstrated an endometrial polyp containing a 4 mm x 5 mm nodule of metastatic carcinoid tumor, consistent with metastasis from patient's known pulmonary carcinoid. The tumor was morphologically similar to the tumors of the right lung, with similar immune-profile. DISCUSSION This patient presented with a suspicious pelvic ultrasound. Due to her age, the first priority was to exclude uterine cancer. The endometrial polyp, which was found, had a small focus of metastatic carcinoid tumor. To the best of our knowledge, this finding has not been previously recorded in the literature. Our patient also had a history of metastatic carcinoid tumor to breast. This finding is also very uncommon. CONCLUSION This is the first case in the literature described a malignant carcinoid metastasis to an endometrial polyp. PMID:23127865

  15. In vitro chemoresistance testing in well differentiated carcinoid tumors.

    USDA-ARS?s Scientific Manuscript database

    Well-differentiated, “typical” carcinoid tumors traditionally have a very poor response to chemotherapy. We hypothesized that tumor specimens from well differentiated carcinoid tumors would be highly resistant to the effects of chemotherapy when tested against a variety of antineoplastic agents in v...

  16. Primary carcinoid tumor of the frontal sinus: A case report.

    PubMed

    Chu, Michael W; Karakla, Daniel W; Silverberg, Marc; Han, Joseph K

    2010-10-01

    Carcinoid tumors are rare, indolent, neuroendocrine tumors that are most commonly found in the gastrointestinal tract and lungs. They occasionally develop in the head and neck, either as a primary tumor or, more commonly, as a metastasis. The most common sites of head and neck carcinoids are the larynx and middle ear. Only a few cases of carcinoid in the nasal cavity and paranasal sinuses have been reported. We describe a case of primary typical carcinoid of the frontal sinus in a 61-year-old man, and we review the clinical, imaging, surgical, and histochemical findings in this case. The patient was treated with endoscopic resection. A subsequent workup for metastatic and occult primary disease was negative, confirming that the frontal sinus was the primary source. At follow-up 12 months postoperatively, the patient remained without disease. To the best of our knowledge, this is the first report of a primary typical carcinoid tumor originating in the frontal sinus.

  17. Diagnosis and management of typical and atypical lung carcinoids.

    PubMed

    Pusceddu, Sara; Lo Russo, Giuseppe; Macerelli, Marianna; Proto, Claudia; Vitali, Milena; Signorelli, Diego; Ganzinelli, Monica; Scanagatta, Paolo; Duranti, Leonardo; Trama, Annalisa; Buzzoni, Roberto; Pelosi, Giuseppe; Pastorino, Ugo; de Braud, Filippo; Garassino, Marina Chiara

    2016-04-01

    An estimated 20% to 30% of all neuroendocrine tumours originate in the bronchial tree and lungs. According to the 2015 World Health Organization categorization, these tumours are separated into four subtypes characterized by increasing biological aggressiveness: typical carcinoid, atypical carcinoid, large-cell neuroendocrine carcinoma and small-cell carcinoma. Although typical and atypical lung carcinoids account for less than 1-5% of all pulmonary malignancies, the incidence of these neoplasms has risen significantly in recent decades. Surgery is the treatment of choice for loco-regional disease but for advanced lung carcinoids there is no recognized standard of care and successful management requires a multidisciplinary approach. The aim of this review is to provide a useful guide for the clinical management of lung carcinoids.

  18. Computed tomography of abdominal carcinoid tumors

    SciTech Connect

    Picus, D.; Glazer, H.S.; Levitt, R.G.; Husband, J.E.

    1984-09-01

    Computed tomographic (CT) scans were obtained in 20 patients with primary and/or metastatic abdominal carcinoid tumors. The primary tumors were seen rarely on CT. Mesenteric involvement was seen in eight of the 20 patients, usually as a soft-tissue mass surrounded by fat and radiating soft-tissue strands. Enlarged retroperitoneal lymph nodes were seen in seven patients, but rarely were they the only manifestation of intraabdominal disease. The most common finding was liver metastases (13 of 20 patients). CT is helpful in evaluating the extent of tumor before surgical exploration an in following the progression of disease once the diagnosis has been established.

  19. Synchronous Adenocarcinoma of the Colon and Rectal Carcinoid

    PubMed Central

    Vootla, Vamshidhar; Ahmed, Rafeeq; Niazi, Masooma; Balar, Bhavna; Nayudu, Suresh

    2016-01-01

    Primary colonic adenocarcinoma and synchronous rectal carcinoids are rare tumors. Whenever a synchronous tumor with a nonmetastatic carcinoid component is encountered, its prognosis is determined by the associate malignancy. The discovery of an asymptomatic gastrointestinal carcinoid during the operative treatment of another malignancy will usually only require resection without additional treatment and will have little effect on the prognosis of the individual. This article reports a synchronous rectal carcinoid in a patient with hepatic flexure adenocarcinoma. We present a case of a 46-year-old Hispanic woman with a history of hypothyroidism, uterine fibroids and hypercholesterolemia presenting with a 2-week history of intermittent abdominal pain, mainly in the right upper quadrant. She had no family history of cancers. Physical examination was significant for pallor. Laboratory findings showed microcytic anemia with a hemoglobin of 6.6 g/dl. CT abdomen showed circumferential wall thickening in the ascending colon near the hepatic flexure and pulmonary nodules. Colonoscopy showed hepatic flexure mass and rectal nodule which were biopsied. Pathology showed a moderately differentiated invasive adenocarcinoma of the colon (hepatic flexure mass) and a low-grade neuroendocrine neoplasm (carcinoid of rectum). The patient underwent laparoscopic right hemicolectomy and chemotherapy. In patients diagnosed with adenocarcinoma of the colon and rectum, carcinoids could be missed due to their submucosal location, multicentricity and indolent growth pattern. Studies suggest a closer surveillance of the GI tract for noncarcinoid synchronous malignancy when a carcinoid tumor is detected and vice versa. PMID:27920648

  20. [Somatostatin receptor scintigraphy in pediatric bronchial carcinoid tumor].

    PubMed

    Hervás Benito, I; Bello Arques, P; Loaiza, J L; Vercher, J L; Velasco, R P; Rivas Sánchez, A; Ruiz Llorca, C; Martí Vidal, J F; Mateo Navarro, A

    2010-01-01

    Carcinoid tumor is a rare neuroendocrine neoplasm with different locations, the most frequent ones during the pediatric age being the appendix and lung. Scintigraphy with (111)In-DTPA-d-Phe(1)-octreotide has led to an importance advance in the diagnosis of extension in carcinoid tumor patients. We present three pediatric patients with bronchial carcinoid studied with somatostatin analogue scintigraphy (SSRS). The first patient (9 years) was studied using the SSRS after surgery due to carcinoid tumor in the right lower lobe in which tumor remains was observed (this being clearer in the tomography study). The second patient (10 years) presented due to endobronchial tumor in the left lower lobe together with atelectasis of the LUL and emphysema of the LLL. Radiology imaging techniques suggested the differential diagnosis between the endobronchial carcinoid tumor or plasma cells or foreign body gramuloma. The SSRS showed an abnormal deposit of activity in the left hemithorax consisted with carcinoid tumor. No other areas suggesting metastasis were observed. After the surgery (endobronchial resection), new controls with SSRS showed absence of disease. The third patient (12 years) came after a lobectomy (RUL) due to bronchial carcinoid. The SSRS did not show any abnormal areas of activity. In the subsequent control (3 months), a deposit of activity was observed in the middle third of the right hemithorax, after which a lobectomy was performed (RLL and ML) that showed small remains of neuroendocrine carcinoid. Subsequent controls were negative. The SSRS has demonstrated great utility in the diagnosis, follow-up and staging of pediatric patients, carriers of neuroendocrine carcinoid tumors. Copyright 2008 Elsevier España, S.L. y SEMNIM. All rights reserved.

  1. Imaging features of carcinoid tumors metastatic to the breast

    PubMed Central

    Jones, Katie N.; Dilaveri, Christina A.; Perry, Kyle; Reynolds, Carol

    2011-01-01

    Abstract The objective of this study was to describe the imaging findings of carcinoid tumors metastatic to the breast, with pathologic and clinical correlations. We searched our surgical database for cases of pathologically proven carcinoid tumors metastatic to the breast from October 1, 2000, to May 31, 2010. Of the approximate 10,000 breast biopsies identified, 7000 had malignant findings. Ten cases of metastatic carcinoid (0.1% of all malignancies), all with imaging studies available for review, were included in the study. All patients were women and had their primary carcinoid in the gastrointestinal tract (n=9) or lung (n = 1). One patient presented with a palpable breast mass and no history of carcinoid tumor; an ileal carcinoid was discovered after the pathologic diagnosis of metastatic carcinoid was established. In the breast, tumors presented as solitary lesions in half the cases. Metastases to the breast typically presented as circumscribed masses mammographically and as hypoechoic circumscribed masses ultrasonographically; some showed increased through-transmission and increased vascularity with color Doppler evaluation. Five patients had octreotide scans; of these, 4 had increased focal activity in the region of metastasis within the breast. Six patients underwent computed tomography. Without contrast, nodular masses were observed; with contrast, the masses showed rapid enhancement during arterial phase imaging. Magnetic resonance imaging (n = 4) also showed rapid enhancement and washout kinetics after contrast administration. Recognition of carcinoid metastases to the breast in patients with known or occult primary carcinoid tumors is important to avoid unnecessary treatment for primary breast cancer. PMID:21771708

  2. New cancer or carcinoid progression to small cell lung cancer?

    PubMed

    Ie, Susanti; Boyd, Michael

    2015-04-01

    Carcinoids and small cell lung cancer share neuroendocrine cellular origins. Surgery is the definitive treatment in typical carcinoid with few recurrences. For patients considered to be poor surgical candidates, ablative and cryotherapies have been utilized with good results. The long-term consequences of these alternatives approaches are unclear. We report a case of typical carcinoid treated with various alternative approaches over a period of 6 years with either transformation to small cell lung cancer or the development of a new primary in the same location.

  3. Gastrointestinal carcinoid tumours. Histogenetic, histochemical, immunohistochemical, clinical and therapeutic aspects.

    PubMed

    Wilander, E; Lundqvist, M; Oberg, K

    1989-01-01

    The increased knowledge of the pathobiology of gastrointestinal carcinoid (neuroendocrine) tumours and the improved therapeutic possibilities have brought a demand for more precise diagnosis. Although the carcinoid tumours can often be tentatively recognized in routinely processed microscopic slides, their more accurate identification requires additional diagnostic procedures. General neuroendocrine markers such as the argyrophil reaction of Grimelius and immunohistochemistry with application of antibodies against chromogranin A and of neuron-specific enolase are discriminatory staining methods which are used to reveal the neuroendocrine origin of almost all highly differentiated carcinoid tumours of the gastrointestinal tract. Mid-gut carcinoids, which predominate among these tumours almost unexceptionally contain serotonin. This biogenic amine can be demonstrated by the argentaffin reaction of Masson, serotonin immunoreactively or by formalin-induced fluorescence. The characteristic staining pattern of mid-gut carcinoids is almost invariably preserved in the metastatic deposits and consequently the staining methods for identifying serotonin can also be used on metastases to reveal a primary mid-gut carcinoid. The enterochromaffin-like (ECL) cell carcinoids of the body and fundic area of the stomach often seen in association with pernicious anaemia are argyrophil with the Sevier-Munger silver stain. Other neuroendocrine tumours, viz. antral, duodenal and rectal carcinoids should be studied by a battery of relevant peptide hormone antisera for adequate diagnosis. During the last decade new peptide hormones have been found in circulation in patients with carcinoid tumours, but serotonin and urinary 5-HIAA are still the most important markers for carcinoids of the mid-gut origin. Other clinically useful tumour markers are chromogranin A + B, pancreatic polypeptide, human chorionic gonadotropin alpha and beta subunits. For localizing procedures, angiography is the

  4. [Retrospective evaluation of carcinoid tumors of the appendix in children].

    PubMed

    San Vicente, B; Bardají, C; Rigol, S; Obiols, P; Melo, M; Bella, R

    2009-04-01

    Carcinoids of the appendix are rare in children. Usually diagnosed incidentally on histologic investigation following appendectomy for acute apendicitis. To investigate the significance of the diagnosis of appendiceal carcinoid tumors in children, we conducted a retrospective study in our institution. Between 1990 and 2007 a total of 1158 appendectomy were done. In four patients the diagnosis was appendiceal carcinoid. We studied treatment, follow-up and prognosis of this patients. Indicacion for appendectomy was acute pain in lower right quadrant. The median tumor diameter was lower than 1 cm and the appropriate treatment was appendectomy. The prognosis was excellent in all the patients.

  5. Metastatic carcinoid tumor to the breast: report of two cases and review of the literature.

    PubMed

    Lee, Shimwoo; Levine, Pascale; Heller, Samantha L; Hernandez, Osvaldo; Mercado, Cecilia L; Chhor, Chloe M

    The breast is an unusual site for carcinoid metastasis. Due to increasing survival rates for carcinoid tumors, however, awareness of their rare complications is important. Carcinoid metastasis to the breast typically presents as a palpable breast mass or a mass on screening mammogram. Because imaging findings are nonspecific, the diagnosis is established through histological findings of neuroendocrine features corresponding with the known primary carcinoid pathology. Correctly distinguishing metastatic carcinoid from primary breast carcinoma is crucial to avoid more invasive procedures required for the latter. Two cases of metastatic carcinoid to the breast are presented with review of the literature. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Pre-surgical bronchoscopic treatment for typical endobronchial carcinoids.

    PubMed

    Guarino, Carmine; Mazzarella, Gennaro; De Rosa, Nicolina; Cesaro, Cristiano; La Cerra, Giuseppe; Grella, Edoardo; Perrotta, Fabio; Curcio, Carlo; Guerra, Germano; Bianco, Andrea

    2016-09-01

    Carcinoids are tumors that originate from diffuse neuroendocrine system cells (APUD cells) and represent 1-2% of all pulmonary tumors. Although surgical resection remains the mainstay of treatment, bronchoscopic radical resection of typical carcinoids in selected cases exhibiting endoluminal growth and small implant base has also been explored. Bronchoscopic removal of endobronchial lesions may also reduce the risk of post-obstructive infections and improve pulmonary function, allowing the patient to undergo surgery in better clinical and respiratory state. In this paper we have evaluated the impact on surgical planning and outcome of preoperative bronchoscopic resection in treatment of endobronchial typical carcinoids. Our observations further support the role of bronchoscopic treatment before surgery in endobronchial typical carcinoids.

  7. Frequent mutations in chromatin-remodelling genes in pulmonary carcinoids.

    PubMed

    Fernandez-Cuesta, Lynnette; Peifer, Martin; Lu, Xin; Sun, Ruping; Ozretić, Luka; Seidal, Danila; Zander, Thomas; Leenders, Frauke; George, Julie; Müller, Christian; Dahmen, Ilona; Pinther, Berit; Bosco, Graziella; Konrad, Kathryn; Altmüller, Janine; Nürnberg, Peter; Achter, Viktor; Lang, Ulrich; Schneider, Peter M; Bogus, Magdalena; Soltermann, Alex; Brustugun, Odd Terje; Helland, Åslaug; Solberg, Steinar; Lund-Iversen, Marius; Ansén, Sascha; Stoelben, Erich; Wright, Gavin M; Russell, Prudence; Wainer, Zoe; Solomon, Benjamin; Field, John K; Hyde, Russell; Davies, Michael Pa; Heukamp, Lukas C; Petersen, Iver; Perner, Sven; Lovly, Christine; Cappuzzo, Federico; Travis, William D; Wolf, Jürgen; Vingron, Martin; Brambilla, Elisabeth; Haas, Stefan A; Buettner, Reinhard; Thomas, Roman K

    2014-03-27

    Pulmonary carcinoids are rare neuroendocrine tumours of the lung. The molecular alterations underlying the pathogenesis of these tumours have not been systematically studied so far. Here we perform gene copy number analysis (n=54), genome/exome (n=44) and transcriptome (n=69) sequencing of pulmonary carcinoids and observe frequent mutations in chromatin-remodelling genes. Covalent histone modifiers and subunits of the SWI/SNF complex are mutated in 40 and 22.2% of the cases, respectively, with MEN1, PSIP1 and ARID1A being recurrently affected. In contrast to small-cell lung cancer and large-cell neuroendocrine lung tumours, TP53 and RB1 mutations are rare events, suggesting that pulmonary carcinoids are not early progenitor lesions of the highly aggressive lung neuroendocrine tumours but arise through independent cellular mechanisms. These data also suggest that inactivation of chromatin-remodelling genes is sufficient to drive transformation in pulmonary carcinoids.

  8. The rare malignancy of the hepatobiliary system: ampullary carcinoid tumor.

    PubMed

    Ozsoy, Mustafa; Ozsoy, Yucel; Canda, Aras Emre; Nalbant, Olcay Ak; Haskaraca, Fatih

    2011-01-01

    Introduction. Carcinoid tumors are low-grade tumors originating from endoderm and mostly involving the gastrointestinal system. However; they may be seen in any site within the gastrointestinal system. Case Presentation. A 69-year-old female patient. The results of blood tests were observed to be consistent with obstructive jaundice. A mass appearance was not encountered on tomographic examination. Papilla that was tumor-like macroscopically was seen in the second part of the duodenum in diagnostic endoscopy. Pylorus-preserving pancreaticoduodenectomy surgical procedure was applied. On pathological examination of the mass, a tumoral mass was detected in ampulla vateri localization, 1.5 × 1 × 0.8 cm in size, which, in immunohistochemical staining, was evaluated as a neuroendocrine tumor. Also, Metastasis was observed. Conclusion. The rarest type of carcinoid tumor is ampullary located carcinoid tumor, and tumor size is not a reliable indicator for tumor aggressivity in ampullary carcinoid tumors.

  9. What Are the Risk Factors for Gastrointestinal Carcinoid Tumors?

    MedlinePlus

    ... Testing: What You Need to Know . Race and gender Carcinoid tumors are more common among African Americans ... would like to unsubscribe/opt out from our communications, please follow this link: http://www.cancer.org/ ...

  10. Invasive atypical thymic carcinoid: three case reports and literature review

    PubMed Central

    Zhu, Shan; Wang, Zhong-Tang; Liu, Wen-Zhi; Zong, Shi-Xiang; Li, Bao-Sheng

    2016-01-01

    Atypical thymic carcinoid is an extremely rare thymic neuroendocrine tumor derived from the neuroendocrine system. The aims of this paper were to investigate the clinical features of atypical thymic carcinoid and collate information and experience to improve the diagnosis and treatment of this disease. We describe three cases of atypical carcinoid of the thymus; clinical features, pathological data, treatment modalities, and short-term patient outcomes were summarized and analyzed. The initial clinical symptoms and signs of all three patients were nonspecific and an anterior mediastinal mass was found in each patient on chest computed tomography scan. All three patients underwent surgical resection (total thymectomy and complete excision of the tumor), followed by postoperative radiotherapy, with or without chemotherapy. The diagnoses of three patients were confirmed by pathological and immunohistochemical evaluation. We also present a review of the literature to collate as much information as possible and provide a reference for proper diagnosis and treatment of atypical thyroid carcinoid. PMID:27785065

  11. Long-standing malignant pancreatic carcinoid treated with octreotide.

    PubMed

    Varas-Lorenzo, M J

    2010-11-01

    A male presented with a metastatic, plasma serotonin-secreting (high 5-HIAA in urine), malignant pancreatic carcinoid with a carcinoid-like syndrome, and was assessed using ultrasounds (US), computerized tomography (CT), magnetic resonance imaging (MRI), endoscopic ultrasonography (EUS) and Octreoscan; he sequentially received chemotherapy, interferon and octreotide, with long-term, 12-year survival after diagnosis. Given this unusual case, the second reported in our country, the overall literature is reviewed.

  12. A case of carotid body tumor concomitant with carcinoid tumor.

    PubMed

    Mun, Mi Jin; Lee, Jin Choon; Lee, Byung Joo

    2015-02-01

    Neuroendocrine tumors typically fall into two broad categories: those of epithelial origin and those of neural derivation. The former group includes carcinoid tumors and the latter includes paraganglioma. Although paraganglioma and carcinoid tumor have different biologic behaviors, their overlapping histological appearance can pose diagnostic challenges. Carcinoid tumors are rare, slow-growing neuroendocrine tumors arising from the enterochromaffin cells disseminated throughout the gastrointestinal and bronchopulmonary systems. Carotid body tumor is the most common type of extra-adrenal paraganglioma. Paraganglioma tends to grow slowly but can compress adjacent vessel and nerve. A 63-year-old woman showed huge mass extending from carotid body to skull base, encircling internal and external carotid arteries on magnetic resonance image. Surgical removal of carotid body tumor was done after embolization procedure. Postoperative histopathologic examination and immunohistochemical analysis were consistent with paraganglioma concomitant with carcinoid tumor in carotid body. Primary cervical carcinoid tumor is extremely rare, and to the best of our knowledge, this is the first case of concomitant existence of paraganglioma and carcinoid tumor in carotid body.

  13. Generation and sequencing of pulmonary carcinoid tumor cell lines.

    PubMed

    Asiedu, Michael K; Thomas, Charles F; Tomaszek, Sandra C; Peikert, Tobias; Sanyal, Bharati; Sutor, Shari L; Aubry, Marie-Christine; Li, Peter; Wigle, Dennis A

    2014-12-01

    Pulmonary carcinoid tumors account for approximately 5% of all lung malignancies in adults, and comprise 30% of all carcinoid tumors. There are limited reagents available to study these rare tumors, and consequently no major advances have been made for patient treatment. We report the generation and characterization of human pulmonary carcinoid tumor cell lines to study underlying biology, and to provide models for testing novel chemotherapeutic agents. Tissue was harvested from three patients with primary pulmonary typical carcinoid tumors undergoing surgical resection. The tumor was dissociated and plated onto dishes in culture media. The established cell lines were characterized by immunohistochemistry, Western blotting, and cell proliferation assays. Tumorigenicity was confirmed by soft agar growth and the ability to form tumors in a mouse xenograft model. Exome and RNA sequencing of patient tumor samples and cell lines was performed using standard protocols. Three typical carcinoid tumor lines grew as adherent monolayers in vitro, expressed neuroendocrine markers consistent with the primary tumor, and formed colonies in soft agar. A single cell line produced lung tumors in nude mice after intravenous injection. Exome and RNA sequencing of this cell line showed lineage relationship with the primary tumor, and demonstrated mutations in a number of genes related to neuronal differentiation. Three human pulmonary typical carcinoid tumor cell lines have been generated and characterized as a tool for studying the biology and novel treatment approaches for these rare tumors.

  14. FDG-PET imaging in patients with pulmonary carcinoid tumor.

    PubMed

    Moore, William; Freiberg, Evan; Bishawi, Muath; Halbreiner, Micheal S; Matthews, Robert; Baram, Daniel; Bilfinger, Thomas V

    2013-07-01

    This study aimed to assess the imaging findings in patients with pathologically proven carcinoid tumors and determine if SUV can help to differentiate typical from atypical (more aggressive) pulmonary carcinoid tumors. A retrospective review of patients with a biopsy-proven diagnosis of a pulmonary carcinoid tumor at our institution from 2002 to 2010 that had a preoperative PET scan was performed after institutional review board approval was obtained. PET results, including SUV uptake and location, were recorded as well as all data from pathology reports. Carcinoids were considered to be more aggressive if they showed pathological diagnosis consistent with atypical carcinoid, lymph node invasion, poor histological grade (poorly differentiated), or evidence of systemic metastases. Atypical carcinoid pathology consisted of focal necrosis or a higher mitotic index (2-10 per square millimeter) with features of nests, trabeculae, pleomorphic cells, or dense hyperchromasia. SUV uptake was then evaluated and compared between the typical and atypical carcinoid groups using nonparametric statistical methods. We identified 29 patients from 2002 to 2010 at our institution with a pathological diagnosis of pulmonary carcinoid. Twenty-three were histopathologically typical, and the other 6 showed atypia. Mean (SD) nodule size was 2.4 (1.3) cm in the typical group versus 5.0 (3.2) cm in the atypical group (P = 0.065). Mean (SD) SUV uptake in the typical carcinoid group was 2.7 (1.6) and in the atypical group the SUV was 8.1 (4.1) (P < 0.01). A cutoff SUV of 6 or greater is predictive of malignancy (odds ratio, 23.6; P < 0.01), as well as a nodule size of 3.5 cm or greater (odds ratio, 5.1; P = 0.024). Preoperative PET imaging result is frequently positive in carcinoid tumors, and the biological behavior correlates well with SUV; however, size is not as strong of a predictor of malignancy. Size of 3.5 cm or greater and SUV of 6 or greater have a predictive value of greater than 95

  15. Carcinoid tumor of the ileoanal pouch in a patient with ulcerative colitis.

    PubMed

    Resnick, Murray; Pricolo, Victor; Chen, Sonja

    2013-01-04

    Carcinoid tumors have been reported to occur in various locations, particularly in the gastrointestinal tract. The relationship between the development of carcinoids and ulcerative colitis has been an unclear and controversial one. The association of ulcerative colitis and the development of ileal-pouch carcinoids has not, however, been well documented. We report a case of carcinoid tumor arising in an ileoanal pouch and discuss its unique diagnostic and therapeutic considerations.

  16. Increased incidence of second primary malignancy in patients with carcinoid tumors: case report and literature review.

    PubMed Central

    Rivadeneira, D. E.; Tuckson, W. B.; Naab, T.

    1996-01-01

    There is an increased incidence of second noncarcinoid neoplasms in patients with carcinoid tumors. This article reports a case of a synchronous malignant ileal carcinoid tumor in a patient with an adenocarcinoma of the sigmoid colon. This report illustrates the increased association of carcinoid tumors with other gastrointestinal malignancies. Images Figure 1 Figure 2 Figure 3 PMID:8667441

  17. Clear cell carcinoid tumor of the distal common bile duct

    PubMed Central

    Todoroki, Takeshi; Sano, Takaaki; Yamada, Shuji; Hirahara, Nobutsune; Toda, Naotaka; Tsukada, Katsuhiko; Motojima, Ryuji; Motojima, Teiji

    2007-01-01

    Background Carcinoid tumors rarely arise in the extrahepatic bile duct and can be difficult to distinguish from carcinoma. There are no reports of clear cell carcinoid (CCC) tumors in the distal bile duct (DBD) to the best of our knowledge. Herein, we report a CCC tumor in the DBD and review the literature concerning extrahepatic bile duct carcinoid tumors. Case presentation A 73-old man presented with fever and occult obstructive jaundice. Ultrasonography, computed tomography (CT) and magnetic resonance cholangiopancreaticography (MRCP) demonstrated a nodular tumor projection in the DBD without regional lymph node swelling. Under suspicion of carcinoma, we resected the head of the pancreas along with 2nd portion duodenectomy and a lymph node dissection. The surgical specimen showed a golden yellow polypoid tumor in the DBD (0.8 × 0.6 × 0.5 cm in size). The lesion was composed of clear polygonal cells arranged in nests and a trabecular pattern. The tumor invaded through the wall into the fibromuscular layer. Immunohistochemical stains showed that neoplastic cells were positive for neuron-specific enolase (NSE), chromogranin A, synaptophysin, and pancreatic polypeptide and negative for inhibin, keratin, CD56, serotonin, gastrin and somatostatin. The postoperative course was uneventful and he is living well without relapse 12 months after surgery. Conclusion Given the preoperative difficulty in differentiating carcinoid from carcinoma, the pancreaticoduodenectomy is an appropriate treatment choice for carcinoid tumors located within the intra-pancreatic bile duct. PMID:17227590

  18. Bronchial carcinoid tumors: clinical and radiological correlation.

    PubMed

    Squerzanti, Antonella; Basteri, Vittoria; Antinolfi, Gabriele; D'agostino, Fulvio; Scutellari, Pier Nuccio; Ravenna, Franco; Ghirardi, Redenta; Cavallesco, Giorgio

    2002-10-01

    To evaluate the incidence of bronchial carcinoid tumor (BCT) in population affected by various lung tumors, retrospectively reviewed between 1986-2001, and to emphasize the radiographic patterns if they are characteristic. In the above mentioned period, chest x-ray and CT were performed in 1110 patients, both male and female, affected by lung neoplasms. 20 patients were affected by BCT (16 males, and 13 females), aged between 26 and 75 years (mean age 57.5), with histopathologic diagnosis of typical (9 cases, 31%) and atypical (20 cases, 69%) bronchial carcinoid tumors. Localized right lung lesion were as follows: 7 cases in superior, 9 in middle and 4 in inferior lobes; in the left lung, 5 cases in superior, and 4 in inferior lobes. Size of the lesions was as average 2.4 cm in diameter (with a range of 0.5-12 cm). On x-ray and CT images, BCT appeared as a well marginated nodule, of which 9 were peripheral and 20 central. BCT are classified as neuroendocrine carcinomas,and are divided in typical and atypical forms, with variable grade of malignancy. Central neoplasms are symptomatic due to bronchial obstruction (i.e., pneumonia, atelectasis, bronchiectasis, emphysema and/or lung abscess); if airway obstruction is partial, then cough, wheezing and recurrent pulmonary infections occur. Peripheral tumors are generally asymptomatic and they are discovered occasionally, when chest x-ray is made for other reasons. Radiographic features are similar in typical and atypical BCT. In central tumors a rounded well circumscribed hilar mass is noted, with lobulated or bumpy margins. Central cavitation is not referred to. Peripheral BCT appear as a solitary nodule, inferioer then 3 cm in size, marginated, surrounded by normal pulmonary tissue. Signs and symptoms of BCT are evasive and vague. No current clinical or laboratory procedures are useful in confirming the diagnosis; particularly, no imaging modalities are able to differentiate between BCT and other pulmonary tumors. For

  19. A Case of Multicentric Carcinoid in a Patient with Psoriatic Spondyloarthropathy

    PubMed Central

    George, Nabil; Depala, Archana; Al Sweedan, Laith; Chakravarty, Kuntal

    2015-01-01

    We describe the first case of a patient presenting with multicentric carcinoid occurring in the lung and subsequently in the rectum, with chronic psoriatic arthritis. Although reports have been published regarding carcinoid syndrome occurring alongside rheumatoid arthritis, no reports have been made on such a case. Initial presentation of carcinoid syndrome in this patient was insidious and atypical with few symptoms, including shortness of breath and long standing abdominal bloating. Several years later a sudden change in bowel habit prompted a colonoscopy with biopsy that revealed a carcinoid rectal polyp. The case we report describes a rare presentation of carcinoid syndrome in chronic psoriatic arthropathy. PMID:25802789

  20. A case of multicentric carcinoid in a patient with psoriatic spondyloarthropathy.

    PubMed

    George, Nabil; Depala, Archana; Al Sweedan, Laith; Chakravarty, Kuntal

    2015-01-01

    We describe the first case of a patient presenting with multicentric carcinoid occurring in the lung and subsequently in the rectum, with chronic psoriatic arthritis. Although reports have been published regarding carcinoid syndrome occurring alongside rheumatoid arthritis, no reports have been made on such a case. Initial presentation of carcinoid syndrome in this patient was insidious and atypical with few symptoms, including shortness of breath and long standing abdominal bloating. Several years later a sudden change in bowel habit prompted a colonoscopy with biopsy that revealed a carcinoid rectal polyp. The case we report describes a rare presentation of carcinoid syndrome in chronic psoriatic arthropathy.

  1. Atypical carcinoid presenting as dumb-bell-shaped tumour in the normal kidney.

    PubMed

    Verma, Ritu; Gupta, Pallav

    2013-09-24

    Carcinoid tumours are low-grade malignant neoplasms with neuroendocrine differentiation and occur frequently in the gastrointestinal and respiratory tracts. Primary carcinoid tumours of the kidney are rare and a majority of these tumours occur in anomalous kidney and exhibit typical renal carcinoid morphology. We reported a middle-aged man with primary atypical carcinoid tumour occurring in a normal kidney. The patient was diagnosed as having renal cell carcinoma owing to a lack of neuroendocrinal clinical features. Immunohistochemical staining of the nephrectomy specimen helped in the diagnosis of atypical renal carcinoid.

  2. Appendiceal carcinoid tumor in children: implications for less radical surgery?

    PubMed

    Malkan, A D; Wahid, F N; Fernandez-Pineda, I; Sandoval, J A

    2015-03-01

    The discovery of an appendiceal carcinoid tumor found incidentally or during the course of diagnostic or therapeutic procedures is a burden to both the patient and clinician. The role of the correct surgical operation is paramount for lesions suspected to be malignant. In the pediatric population, appendiceal carcinoids continue to challenge the clinician in choosing the optimal treatment when lesions are larger than 2 cm or involve the appendical base. While the criteria used to define these distinct lesions are available in most cases, the management and treatment are still debated and controversial when considering more radical surgical intervention. The purpose of this article is to give an overview regarding the history, diagnosis, histopathology, management, and controversies associated with appendiceal carcinoid in the pediatric population.

  3. Atypical intermediate-grade mediastinal carcinoid. Case presentation.

    PubMed

    Carrillo-Muñoz, Araceli; Onofre-Borja, Melissa; Borrego-Borrego, Rafael; Chávez-Mercado, Leonora; Navarro-Reynoso, Francisco Pascual; Ibarra-Pérez, Carlos

    2011-01-01

    Approximately 25% of carcinoid tumors develop in the respiratory system. Neuroendocrine carcinoids represent ~5% of all mediastinal tumors and 1-5% of all intrathoracic neoplasms. They contain numerous neurosecretory granules that synthesize, store and release neurohumoral substances that can induce the carcinoid syndrome. A 21-year-old male presented with a rapidly progressive paraneoplastic syndrome unleashed by an acute urethritis. Two left mediastinal masses were identified and resected. Postoperative evolution has been uneventful during the first year. We emphasize the importance of early detection of primary and satellite lesions of these tumors including neurohumoral markers and PET/CT scans as in this case, as well as the participation of a multidisciplinary team.

  4. An Analysis of Trends and Growth Factor Receptor Expression of GI Carcinoid Tumors

    PubMed Central

    Bowen, Kanika A.; Silva, Scott R.; Johnson, Jessica N.; Doan, Hung Q.; Jackson, Lindsey N.; Gulhati, Pat; Qiu, Suimin; Riall, Taylor S.; Evers, B. Mark

    2009-01-01

    The purpose of our study was twofold: 1) to determine the incidence, patient and tumor characteristics, and outcome of patients with GI carcinoid tumors using the Surveillance, Epidemiology and End Results (SEER) database, and 2) to delineate the expression pattern of growth factor receptors (GFRs) in carcinoid tumors. The SEER database search provided information on patients diagnosed with carcinoid tumors from 1990–2002. Carcinoid tumor sections (n = 46) were stained for the GFRs: epidermal growth factor receptor (EGFR), insulin-like growth factor receptor (IGFR), vascular endothelial growth factor receptor (VEGFR), and HER-2/neu. Over the 12 year analysis period, 18,180 patients were identified with carcinoid tumors of the foregut, midgut, and hindgut; the incidence of carcinoid tumors increased ~2-fold during this time period. Of the patients with carcinoid tumors, there was a trend of increased expression of VEGF-R and IGF-R, particularly in the foregut and midgut carcinoids. Analysis of the SEER database confirms that the incidence of carcinoid tumors is increasing with an approximate doubling in the number of carcinoid cases from 1990–2002. Furthermore, an increase in VEGF-R and IGF-R expression suggests that GFR inhibitors may be effective adjuvant therapy for carcinoid cancer. PMID:19582519

  5. Carcinoid tumours of the bronchus: a 33 year experience.

    PubMed Central

    Hurt, R; Bates, M

    1984-01-01

    The term adenoma of the bronchus is discussed, and 79 cases of bronchial carcinoid seen from 1951 to 1983 are reviewed. The symptoms, radiological findings, and bronchoscopic appearances are described. There was no case of the carcinoid syndrome. In no case did haemorrhage cause any serious problem after biopsy at rigid bronchoscopy. In three patients the tumour was reported to be an oat cell carcinoma-in two on the basis of material obtained at fibreoptic bronchoscopy. Resection was by pneumonectomy in 10 cases, lobectomy in 52, segmentectomy in six, a bronchoplastic procedure without resection of lung in seven cases, enucleation in two, and a wedge resection in one case. There was one case of atypical carcinoid which was found at operation to be unresectable. A 5-30 year follow up in 57 cases revealed a recurrence of tumour in two cases, nine and 16 years after lung resection. No recurrence occurred in the nine cases treated by conservative bronchial resection with conservation of lung tissue. An actuarially assessed life table analysis shows survival rates of 94% after 10 years, 80% after 15 years, and 64% after 25 years without recurrence. The similarity of carcinoid to oat cell carcinoma is noted and the serious clinical implications of this are analysed, especially in view of the increasing use of fibreoptic bronchoscopy. The malignant potential of carcinoid and the extent of pulmonary resection is discussed. It is concluded that a carcinoid tumour of the lung has only slight malignant potential and that it may be treated by bronchotomy or sleeve resection of the bronchus in suitable cases. If serious infective changes have occurred in the lung distal to the tumour or if the tumour has extended into the lung parenchyma (88% of cases in this series) lung resection will be necessary. The follow up period should be for at least 25 years, in view of the incidence of late recurrence. PMID:6089366

  6. An incidental finding of carcinoid tumor of the cystic duct.

    PubMed

    Lim, Hoang U; Chan, Cyrus C; Knotts, F Barry

    2013-04-04

    Cystic duct carcinoids are extremely rare tumors. We present a 58-year-old female with carcinoid tumor found within the cystic duct margin following laparoscopic cholecystectomy. She subsequently underwent surgical resection with a Roux-en-Y hepaticojejunostomy. No standard guidelines currently exist regarding surgical excision of these rare tumors. Therefore, we conducted a thorough review of the literature to recommend complete oncologic surgical resection with re-establishment of biliary continuity as the mainstay of definitive treatment; adjuvant therapy currently remains investigational. Long-term prognosis is good with this approach.

  7. An incidental finding of carcinoid tumor of the cystic duct

    PubMed Central

    Lim, Hoang U.; Chan, Cyrus C.; Knotts, F. Barry

    2013-01-01

    Cystic duct carcinoids are extremely rare tumors. We present a 58-year-old female with carcinoid tumor found within the cystic duct margin following laparoscopic cholecystectomy. She subsequently underwent surgical resection with a Roux-en-Y hepaticojejunostomy. No standard guidelines currently exist regarding surgical excision of these rare tumors. Therefore, we conducted a thorough review of the literature to recommend complete oncologic surgical resection with re-establishment of biliary continuity as the mainstay of definitive treatment; adjuvant therapy currently remains investigational. Long-term prognosis is good with this approach. PMID:24964431

  8. A Case of Carcinoid Tumor-Associated Hypercalcemia

    PubMed Central

    Siyam, Fadi; Abdullah, Obai; Gardner, Michael; Brietzke, Stephen; Sowers, James

    2012-01-01

    Hypercalcemia as a complication of carcinoid tumors is extremely rare. Accordingly, we report the case of a 55-year-old male with metastatic carcinoid tumor and hypercalcemia, which corrected when the patient was treated with octreotide for symptomatic relief of watery diarrhea. The etiology of the hypercalcemia is presumed to be a neoplastic expression of fibroblast growth factor-23, which was found to be inappropriately high-to-normal when other factors such as parathyroid hormone, calcitonin and vitamin D were appropriately low or low-to-normal. PMID:22493603

  9. [Carcinoid tumor of gastrointestinal tract: about two clinical cases].

    PubMed

    Blasco, María Del Carmen; Boselli O, F Giuliano; Blasco, Carmelo

    2016-01-01

    Carcinoid tumors belong to the families of neuroendocrine tumors. The major sites are the gastrointestinal tract 65% and lungs 25%. The small intestine, specifically the ileum, is the most common. These tumors although rare, are more common in tumors of neuroendocrine origin gastro-entero-pancreatic. In both cases we observe the different clinical presentations that may have carcinoid tumor; in case 1 ulceration of the tumor mass causing the elimination of melena, and severe diarrhea caused by neuroendocrine secretion. Case 2 typical course, totally asymptomatic incidental finding.

  10. Lung parenchymal invasion in pulmonary carcinoid tumor: an important histologic feature suggesting the diagnosis of atypical carcinoid and poor prognosis.

    PubMed

    Ha, Sang Yun; Lee, Jae Jun; Cho, Junhun; Hyeon, Jiyeon; Han, Joungho; Kim, Hong Kwan

    2013-05-01

    The majority of previous studies on pulmonary carcinoid tumor have usually focused on clinical behavior or outcome, seldom considering histopathologic features. We retrospectively collected 63 cases of resected pulmonary carcinoid tumors from 1995 to 2011 at Samsung Medical Center, Seoul, Korea. The clinical and pathological features were correlated and survival analyses were performed. Forty cases (63.5%) were classified as typical carcinoid (TC) and 23 cases (36.5%) were classified as atypical carcinoid (AC) according to WHO classification criteria. AC patients showed a higher frequency of current smoking status and a higher stage of the tumor by the American Joint Committee on Cancer than TC patients. The disease was associated with death and recurrence in five and seven patients, respectively, with almost all of the associations found in AC patients. The five-year survival rate of TC and AC were 100% and 83.5%, respectively, with AC showing poorer prognosis than TC in overall survival (OS) and disease free survival (DFS) (p=0.005 and p=0.002). Lung parenchymal invasion was observed more commonly in AC than in TC (39.1% vs 12.5%, p=0.01) and was a poor prognostic factor in OS and DFS. Rosette-like arrangements were found only in six cases of AC, while abundant basophilic cytoplasm mimicking paraganglioma and ossification were found only in TC. Through the comprehensive study of pulmonary carcinoid tumor in Korea, we suggest that lung parenchymal invasion could be a useful histologic feature to suspect the diagnosis of AC in daily practice as well as to predict the prognosis of carcinoid tumor.

  11. Hypergastrinemia and recurrent type 1 gastric carcinoid in a young Indian male: necessity for antrectomy?

    PubMed

    Senadhi, Viplove; Jani, Niraj

    2011-09-21

    Carcinoid tumors are the most common neuroendocrine tumors. Gastric carcinoids represent 2% of all carcinoids and 1% of all gastric masses. Due to the widespread use of Esophagogastroduodenoscopy for evaluating a variety of upper gastrointestinal symptoms, the detection of early gastric carcinoids has increased. We highlight an alternative management of a young patient with recurrent type 1 gastric carcinoids with greater than 5 lesions, as well as lesions intermittently greater than 1 cm. Gastric carcinoids have a variable presentation and clinical course that is highly dependent on type. Type 1 gastric carcinoids are usually indolent and have a metastasis rate of less than 2%, even with tumors larger than 2 cm. There are a number of experts as well as organizations that recommend endoscopic resection for all type 1 gastric carcinoid lesions less than 1 cm, with a follow-up every 6-12 mo. They also recommend antrectomy for type 1 gastric carcinoids with greater than 5 lesions, lesions 1 cm or greater, or refractory anemia. However, the American Society of Gastrointestinal Endoscopy guidelines state that type 1 gastric carcinoid surveillance is controversial based on the evidence and could not make an evidence-based position statement on the best treatment modality. Our report illustrates a rare cause of iron deficiency anemia in a young male (without any medical history) due to multiple recurrent gastric carcinoid type 1 lesions in the setting of atrophic gastritis causing hypergastrinemia, and in the absence of a vitamin B12 deficiency. Gastric carcinoid type 1 can present in young males without an autoimmune history, despite the known predilection for women aged 50 to 70 years. Type 1 gastric carcinoids can be managed by endoscopic resection in patients with greater than 5 lesions, even with lesions larger than 1 cm. This course of treatment enabled the avoidance of early antrectomy in our patient, who expressed a preference against more invasive measures at his

  12. A thymic carcinoid in a Bengal tiger (Panthera tigris).

    PubMed

    Powe, Joshua; Castleman, William; Fiorello, Christine

    2005-09-01

    An 18-yr-old Bengal tiger (Panthera tigris) presented with acute onset hind limb paresis. Radiographic and ultrasonographic imaging revealed a caudal abdominal aortic thrombus and a cranial mediastinal mass. Necropsy confirmed aortic thrombosis. Necrotizing enteritis and multifocal renal thrombosis were also noted. The cranial mediastinum contained a bilobed mass that histologically and ultrastructurally was consistent with a carcinoid.

  13. Association of carcinoid tumor and low grade glioma

    PubMed Central

    2012-01-01

    Background Lung carcinoid tumor and low grade glioma are two uncommon malignancies. Patients and methods We report the case of 24-year-old man who presented with respiratory disease. Imaging investigations showed a right lung tumor and histological analysis confirmed a typical carcinoid tumor. As part of initial staging, brain MRI revealed an asymptomatic right frontal lesion. First, a right pulmonary lobectomy was performed without adjuvant treatment. In second time, brain tumorectomy was performed. Histological examination confirmed the diagnosis of low grade glioma (LGG). The patient remained in complete remission 2.5 years after the initial diagnosis. Results This is the first case reporting the association between LGG and lung carcinoid tumor, while no association between LGG and a systemic tumor have been published to date. Association of lung carcinoid tumor with other malignant diseases has been reported but remained uncommon. Only minimal data support a potential molecular common origin. Conclusion This exceptional association may be fortuitous. However, their concomitant diagnoses suggest a potential association between both rare diseases. A genetic susceptibility remains possible. PMID:23137305

  14. Association of carcinoid tumor and low grade glioma.

    PubMed

    Tabouret, Emeline; Barrié, Maryline; Vicier, Cecile; Gonçalves, Anthony; Chinot, Olivier; Viens, Patrice; Madroszyk, Anne

    2012-11-08

    Lung carcinoid tumor and low grade glioma are two uncommon malignancies. We report the case of 24-year-old man who presented with respiratory disease. Imaging investigations showed a right lung tumor and histological analysis confirmed a typical carcinoid tumor. As part of initial staging, brain MRI revealed an asymptomatic right frontal lesion. First, a right pulmonary lobectomy was performed without adjuvant treatment. In second time, brain tumorectomy was performed. Histological examination confirmed the diagnosis of low grade glioma (LGG). The patient remained in complete remission 2.5 years after the initial diagnosis. This is the first case reporting the association between LGG and lung carcinoid tumor, while no association between LGG and a systemic tumor have been published to date. Association of lung carcinoid tumor with other malignant diseases has been reported but remained uncommon. Only minimal data support a potential molecular common origin. This exceptional association may be fortuitous. However, their concomitant diagnoses suggest a potential association between both rare diseases. A genetic susceptibility remains possible.

  15. Imaging of carcinoid tumors with iodine-123 metaiodobenzylguanidine

    SciTech Connect

    Bomanji, J.; Levison, D.A.; Zuzarte, J.; Britton, K.E.

    1987-12-01

    Scintigraphy with /sup 123/I-radiolabeled metaiodobenzylguanidine (MIBG) was performed successfully in two patients with metastatic carcinoid tumor. The scintigraphic findings show diversity of the tumor deposits in the uptake of (/sup 123/I)MIBG in the same patient and between patients.

  16. Unusual presentation of carcinoid tumor as acute cholecystitis

    SciTech Connect

    Saxton, C.R.

    1983-07-01

    The patient described had signs, symptoms, and laboratory data consistent with acute cholecystitis. A sonogram also suggested cholecystitis, and the gallbladder was not displayed by nuclear imaging. Cholecystectomy revealed the absence of stones but showed carcinoid tumor metastatic to the gallbladder.

  17. Thymic carcinoids in multiple endocrine neoplasia type 1.

    PubMed Central

    Teh, B T; Zedenius, J; Kytölä, S; Skogseid, B; Trotter, J; Choplin, H; Twigg, S; Farnebo, F; Giraud, S; Cameron, D; Robinson, B; Calender, A; Larsson, C; Salmela, P

    1998-01-01

    OBJECTIVE: To study the clinical, pathologic, and genetic features of thymic carcinoids in the setting of multiple endocrine neoplasia type 1 (MEN1) and to study means for detection and prevention of this tumor in patients with MEN1. SUMMARY BACKGROUND DATA: Thymic carcinoid is a rare malignancy, with approximately 150 cases reported to date. It may be associated with MEN1 and carries a poor prognosis, with no effective treatment. Its underlying etiology is unknown. METHODS: Ten patients with MEN1 from eight families with anterior mediastinal tumors were included in a case series study at tertiary referring hospitals. Clinicopathologic studies were done on these patients, with a review of the literature. Mutation analysis was performed on the MEN1 gene in families with clusterings of the tumor to look for genotype-phenotype correlation. Loss of heterozygosity was studied in seven cases to look for genetic abnormalities. RESULTS: Histologic studies of all tumors were consistent with the diagnosis of thymic carcinoid. Clustering of this tumor was found in some of the families-three pairs of brothers and three families with first- or second-degree relatives who had thymic carcinoid. All patients described here were men, with a mean age at detection of 44 years (range 31 to 66). Most of the patients had chest pain or were asymptomatic; none had Cushing's or carcinoid syndrome. All tumors were detected by computed tomography (CT) or magnetic resonance imaging (MRI) of the chest. The results of octreoscans performed in three patients were all positive. Histopathologic studies were consistent with the diagnosis of thymic carcinoid and did not stain for ACTH. Mutation analysis of the families with clustering revealed mutations in different exons/introns of the MEN1 gene. Loss of heterozygosity (LOH) studies of seven tumors did not show LOH in the MEN1 region, but two tumors showed LOH in the 1p region. CONCLUSIONS: MEN1-related thymic carcinoids constitute approximately 25

  18. Cutaneous and Subcutaneous Metastases From Atypical Laryngeal Carcinoids

    PubMed Central

    Wang, Kui-Rong; Jia, Yuan-Jing; Zhou, Shui-Hong; Wang, Qin-Ying; Bao, Yang-Yang; Feng, Zhi-Ying; Yao, Hong-Tian; Fan, Jun

    2016-01-01

    Abstract The incidence of cutaneous and subcutaneous metastases from atypical laryngeal carcinoids is approximately 20%. However, the pathogenesis and natural history of, and prognostic factors for, the condition remain poorly understood. We reported a 54-year-old female presented with cutaneous and subcutaneous metastases from atypical laryngeal carcinoid. Laryngoscopy revealed a 0.5 × 1.5-cm reddish mass on the laryngeal surface of the epiglottis. Under general anesthesia, a biopsy sample was obtained via suspension laryngoscopy. Routine pathology revealed atypical laryngeal carcinoid. Immunohistochemical staining of the sections of primary tumor was positive for cytokeratin, chromogranin A, synaptophysin, hypoxia-inducible factor-1α, P53, and CD56. GLUT-1, p-Akt, and PI3K were negative. The Ki-67 index was 15%. Supraglottic laryngectomy and selective right-neck dissection were performed. After 6 months, the patient complained of pain in the right wall of the chest; multiple cutaneous and subcutaneous nodules were evident at that site and in the abdomen. An abdominal nodule was biopsied and pathology revealed that the atypical metastatic carcinoid had metastasized to both cutaneous and subcutaneous areas of the abdomen. Chemotherapy was then prescribed. Currently, the intrathecal drug delivery system remains in place. No local recurrence has been detected. Furthermore, we systematically reviewed clinical manifestations of the disease, pathogenesis, prognostic factors, and treatment. The metastasis rate (cutaneous and subcutaneous) was approximately 12.2%. Thirty patients (62.5%) with cutaneous and subcutaneous metastases exhibited contemporaneous lymph node invasion. The 3-, 5-, and 10-year survival rates were 44.0%, 22.0%, and 13.0%, respectively. The prognosis of patients with atypical laryngeal carcinoids was poor. Relevant prognostic factors included the level of p53, human papilloma virus status, certain hypoxic markers, and distant metastasis. No

  19. Prognosis of patients with carcinoid heart disease after valvular surgery.

    PubMed

    Manoly, Imthiaz; McAnelly, Sarah-Louise; Sriskandarajah, Sanjeevan; McLaughlin, Kenneth Edward

    2014-08-01

    A best evidence topic in cardiac surgery was written according to a structured protocol. We addressed the following question: in patients who are diagnosed with carcinoid heart disease (CHD), do valvular surgeries improve their prognosis? Fifty percent of the patients with clinically diagnosed carcinoid syndrome had cardiac involvement which was present either as valvular dysfunction or as cardiac metastases. These patients often require surgery due to their heightened risk of cardiac disease. Altogether 217 relevant papers were identified as a result of the below-mentioned search, of which 10 papers represented the best evidence to answer the question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses were tabulated. Of the patients who were identified to have carcinoid heart disease in different studies, 193 patients had valve procedure, mainly replacements at tricuspid, mitral and aortic valve positions and either valvuloplasty or replacement at pulmonary valve. Tricuspid and pulmonary valves represented the majority of the excised valves among patients undergoing valvular surgery for CHD. The pathology of carcinoid valve was attributed to the presence of plaque, causing thickening and retraction. Pure regurgitation was the most common finding in all the valves except pulmonary valve which had both stenosis and insufficiency. Thirty-day mortality was 17% (range 1-63%) and long-term survivors were reported to be alive at an average of 58 months (28-80 months) after the valve surgery. The evidence demonstrates that surgical intervention can lead to improved prognosis and reduce the symptoms of heart failure. Postoperative mortality was mainly due to the carcinoid disease itself and not as a complication of the surgery. Therefore, surgery could be considered for symptomatic palliation in carefully selected individuals.

  20. Review of the pathogenesis, diagnosis, and management of type I gastric carcinoid tumor.

    PubMed

    Zhang, Linda; Ozao, Junko; Warner, Richard; Divino, Celia

    2011-08-01

    Gastric carcinoid tumors comprise 7% of all gastrointestinal carcinoids and have significantly increased in incidence over the past few decades. Seventy to 80% of gastric carcinoids are type I, which usually are clinically asymptomatic and found incidentally at endoscopic evaluation for abdominal pain or anemia. In this review, advances in understanding the pathophysiology of type I gastric carcinoid are highlighted. In addition, various current diagnostic and treatment options are discussed. Although type I carcinoids generally hold a benign course, rigorous investigation is needed to ensure accurate diagnosis and optimal treatment. This includes appropriate diagnostic procedures and imaging and accurate staging of tumor. Tumor size, depth of invasion, presence of metastasis, and the tumor's gastrin dependency dictate treatment options. Appropriate treatments can consist of endoscopic resection, antrectomy, medical management, or frequent follow-up. This article provides a systematic method of evaluating and treating type I gastric carcinoid.

  1. Synchronous Gastrointestinal Carcinoid Tumor and Colon Adenocarcinoma: Case Reports and Literature Review

    PubMed Central

    Winn, Jessica N.; Sathyamurthy, Anjana; Kneib, Jessica L.; Ibdah, Jamal A.; Tahan, Veysel

    2017-01-01

    Case series Patient: Male, 40; Male, 70 Final Diagnosis: Synchronous gastrointestinal carcinoid tumor and colon adenocarcinoma Symptoms: Weakness Medication: — Clinical Procedure: Colonoscopy Specialty: Gastroenterology and Hepatology Objective: Rare co-existance of disease or pathology Background: Coexistence of carcinoid tumor and colorectal adenocarcinoma is rare. In this report, we present two cases and review the current literature for synchronous carcinoid tumor and colorectal carcinoma occurrence. Case Reports: In both cases, the rectal carcinoid tumors and sigmoid colon adenocarcinomas were detected by colonoscopy. The colon adenocarcinomas were effectively treated with a laparoscopic sigmoidectomy and the carcinoids were successfully removed endoscopically. Our 40-year-old patient was the youngest among 17 reported patient cases. Conclusions: These two cases demonstrate that the diagnosis of gastrointestinal carcinoid requires a complete assessment of the remainder of the colon for another primary cancer to achieve a timely treatment management strategy. PMID:28579603

  2. Ligation-assisted endoscopic submucosal resection with circumferential mucosal incision for duodenal carcinoid tumor.

    PubMed

    Harada, Hideaki; Suehiro, Satoshi; Shimizu, Takanori; Katsuyama, Yasushi; Hayasaka, Kenji; Ito, Hideto

    2015-09-14

    Here we present the case of a 64-year-old female with a duodenal carcinoid tumor treated by ligation-assisted endoscopic submucosal resection (ESMR-L) with circumferential mucosal incision (CMI). Band ligation was effective in resecting the duodenal carcinoid tumor after CMI, with an uneventful post-procedural course. Histopathological examination showed clear tumor margins at deeper tissue levels. Thus, in the present case, ESMR-L with CMI was useful for the treatment of duodenal carcinoid tumor.

  3. Ligation-assisted endoscopic submucosal resection with circumferential mucosal incision for duodenal carcinoid tumor

    PubMed Central

    Harada, Hideaki; Suehiro, Satoshi; Shimizu, Takanori; Katsuyama, Yasushi; Hayasaka, Kenji; Ito, Hideto

    2015-01-01

    Here we present the case of a 64-year-old female with a duodenal carcinoid tumor treated by ligation-assisted endoscopic submucosal resection (ESMR-L) with circumferential mucosal incision (CMI). Band ligation was effective in resecting the duodenal carcinoid tumor after CMI, with an uneventful post-procedural course. Histopathological examination showed clear tumor margins at deeper tissue levels. Thus, in the present case, ESMR-L with CMI was useful for the treatment of duodenal carcinoid tumor. PMID:26379409

  4. Carcinoid heart disease from ovarian primary presenting with acute pericarditis and biventricular failure

    PubMed Central

    Vergani, D; Massironi, L; Lombardi, F; Fiorentini, C

    1998-01-01

    A case is described of a 54 year old woman who had acute pericarditis with large exudative effusion accompanied by severe right and left ventricular failure. The patient was finally diagnosed with carcinoid heart disease from an ovarian carcinoid teratoma. She was treated with octreotide—a somatostatin analogue—followed by radical surgical resection of the neoplasm. At one year follow up only mild carcinoid tricuspid regurgitation remained. Only 16 cases of carcinoid heart disease from an ovarian primary have been described in literature. Moreover clinically manifest acute, non-metastatic pericarditis and left heart failure are not considered as possible presentations of carcinoid heart disease, whatever the origin. In a recent series a small pericardial effusion was considered an infrequent and unexpected echocardiographic finding in carcinoid heart patients. One case of "carcinoid pericarditis" has previously been described as a consequence of pericardial metastasis. Left sided heart involvement is usually caused by bronchial carcinoids or patency of foramen ovale; both were excluded in the case presented.

 Keywords: carcinoid heart disease;  ovarian tumour;  acute pericarditis;  heart failure PMID:10065036

  5. Resolution of right-sided heart failure symptoms after resection of a primary ovarian carcinoid tumor.

    PubMed

    Goldman, Todd; Adamson, Kathi; Yang, Eugene

    2014-10-01

    Carcinoid tumors are rare neuroendocrine malignancies that typically originate from the gastrointestinal tract. Patients who are diagnosed with carcinoid heart disease generally have poor prognoses because of advanced metastases during staging and few therapeutic options. We present the case of a 61-year-old woman with right-sided heart failure, secondary to carcinoid heart disease caused by a primary ovarian carcinoid tumor. After undergoing surgical resection of the left ovary and fallopian tube, the patient experienced complete resolution of her heart failure symptoms. In addition to the patient's case, we discuss the diagnosis, nature, and treatment of this rare condition.

  6. An Unusual Case of Synchronous Carcinoid of Ovary and Gall Bladder

    PubMed Central

    Bassi, Rupali; Arora, Raksha; Bhasin, Sangeeta; Khurana, Nita

    2013-01-01

    Multifocal carcinoid is a known phenomenon. We present a rare combination of an ovarian carcinoid synchronous with gallbladder carcinoid. This rare combination has not been reported so far. An asymptomatic 45-years-old perimenopausal lady was diagnosed to have a metastatic ovarian cancer, but on laparotomy she was found to have a primary synchronous metastatic gall bladder as well. On histopathological evaluation she was found to have two separate primary carcinoids. Subsequently the patient received chemotherapy and is completely asymptomatic on follow up. Further research needs to be undertaken and guidelines need to be formulated for management of these cases. PMID:24371533

  7. Primary carcinoid tumor of the skin: a literature review.

    PubMed

    Jedrych, Jaroslaw; Pulitzer, Melissa

    2014-04-01

    Primary carcinoid tumor of the skin (PCTS) is an uncommon indolent neoplasm, with 10 cases described in the literature. The tumors affect patients in the sixth to ninth decades of life (mean = 66.3 years) with an equal gender distribution and predilection for the head and trunk. They present as slowly enlarging nodules of variable duration, ranging from 1 to 60 years (mean = 11.4 years). PCTS is characterized by architectural, cytomorphologic, ultrastructural, and immunohistochemical features typical of a low-grade neoplasm with neuroendocrine differentiation. PCTS typically follows a benign clinical course and therefore has to be distinguished from cutaneous metastases of visceral carcinoid tumors, which herald dissemination of malignancy and poor prognosis. While the distinction from other histologically similar entities can be achieved by histopathological examination, PCTS can be distinguished from a visceral metastasis only by a judicious clinicopathologic correlation. Herein we describe the clinical presentation, histological appearance, and management of these tumors.

  8. Primary renal carcinoid tumor: A rare cystic renal neoplasm

    PubMed Central

    Yoon, Jung-Hee

    2013-01-01

    We present the case of a 21-year-old man with an incidentally detected cystic renal mass. A well-defined, solid mass measuring approximately 8 cm x 6 cm with a cystic component was identified in the left kidney by abdominal multidetector computed tomography (CT) and ultrasonography. The mass was well-enhanced on the corticomedullary CT phase and washout of enhancement occurred on the nephrographic phase. The mass contained peripheral wall and septal calcifications in the cystic component. The lesion was resected and diagnosed as a primary renal carcinoid tumor. Primary carcinoid tumors of the kidney are extremely rare. This case is notable because of the rarity of this neoplasm and its unique radiologic and pathologic findings. A review of previously reported cases in the literature is also presented. PMID:24003360

  9. Postoperative radiotherapy for residual tumor of primary mediastinal carcinoid teratoma

    PubMed Central

    Tu, Lingli; Sun, Lan; Zhou, Yu; Gong, Youling; Xue, Jianxin; Gao, Jun; Lu, You

    2013-01-01

    A 36-year-old woman had presented with dry cough for 2 months. Thoracic computed tomography (CT) scan showed a 12 cm × 8 cm ×5 cm mass in the anterior mediastinum. Due to intimately involving the aortic arch, tumor was removed incompletely. Residual tumor remained approximate 2 cm × 3 cm × 4 cm. Histologic diagnosis was a mature cystic teratoma containing a carcinoid. Subsequently, radiotherapy (RT) was administrated on residual tumor for a total dose of 50 Gy at 2 Gy/d fraction in 25 fractions. At 2-year follow-up, the patient had stable disease. In conclusion, adjuvant radiotherapy with 50 Gy is an effective approach for residual tumor of mediastinal carcinoid teratoma. PMID:23585961

  10. Appendix carcinoid associated with the Peutz-Jeghers syndrome.

    PubMed

    Hofmann, Sabine; Barth, Thomas F E; Kornmann, Marko; Henne-Bruns, Doris

    2014-01-01

    The Peutz-Jeghers syndrome (PJS) is a rare hereditary, autosomal-dominant disorder. It is characterized by a gastrointestinal polyposis and mucocutaneous melanic spots. It has also been reported as a precondition for malignancies with a life-time-hazard for cancer up to 93%, caused by a germline mutation in the STK11 gene. A 21-year-old man presented with nausea and abdominal pain. He had a known history of PJS since the age of 13 when he was treated for intussusception due to a hamartomatous polyp. Preoperative diagnostics revealed a second intussusception and an extensive intestinal polyposis. Intraoperative findings confirmed the suspected diagnoses and desvagination was performed. Nearly 50 polyps were removed from the small intestinum over several longitudinal sections. As the appendix appeared thickened an appendectomy was performed simultaneously. Histology showed hamartomatous polyps and the incidental finding of a pT1 carcinoid of the appendix. The patient recovered well and needed no further treatment for his carcinoid tumor. The mechanism of carcinogenesis in PJS still remains debatable, although the genetic disorder underlying the syndrome is known. A predisposition for carcinoid tumors also stays questionable. To our knowledge there is no description of an association between carcinoid tumors of the appendix and PJS to date. Life-expectancy in patients with PJS is reduced. Causes are the development of malignancies and complications from the polyps such as intussusception. Since there is no treatment possible main focus must be aimed at early recognition of malignancies and the prevention of complications. Copyright © 2014. Published by Elsevier Ltd.

  11. Primary hepatic carcinoid tumor: case report and literature review

    PubMed Central

    Camargo, Éden Sartor; Viveiros, Marcelo de Melo; Corrêa, Isaac José Felippe; Robles, Laercio; Rezende, Marcelo Bruno

    2014-01-01

    Primary hepatic carcinoid tumors are extremely rare neoplasms derived from hormone-producing neuroendocrine cells. It is difficult to make their diagnosis before biopsy, surgical resection or necropsy. A recent publication described only 94 cases of these tumors. There is no sex predilection and apparently it has no association with cirrhosis or preexisting hepatic disease. The most effective treatment is hepatectomy, and resection is determined by size and location of the lesions. PMID:25628206

  12. 90Y-Edotreotide for Metastatic Carcinoid Refractory to Octreotide

    PubMed Central

    Bushnell, David L.; O'Dorisio, Thomas M.; O'Dorisio, M. Sue; Menda, Yusuf; Hicks, Rodney J.; Van Cutsem, Eric; Baulieu, Jean-Louis; Borson-Chazot, Francoise; Anthony, Lowell; Benson, Al B.; Oberg, Kjell; Grossman, Ashley B.; Connolly, Mary; Bouterfa, Hakim; Li, Yong; Kacena, Katherine A.; LaFrance, Norman; Pauwels, Stanislas A.

    2010-01-01

    Purpose Metastatic carcinoid is an incurable malignancy whose symptoms, such as diarrhea and flushing, can be debilitating and occasionally life-threatening. Although symptom relief is available with octreotide, the disease eventually becomes refractory to octreotide, leaving no proven treatment options. The goal of this study was to evaluate the clinical effect of using 90Y-edotreotide to treat symptomatic patients with carcinoid tumors. Patients and Methods Patients enrolled had metastatic carcinoid, at least one sign/symptom refractory to octreotide, and at least one measurable lesion. Study treatment consisted of three cycles of 4.4 GBq (120 mCi) 90Y-edotreotide each, once every 6 weeks. Results Ninety patients were enrolled in the study. Using Southwest Oncology Group tumor response criteria, 67 (74.%) of 90 patients (95% CI, 65.4% to 83.4%) were objectively stable or responded. A statistically significant linear trend toward improvement was demonstrated across all 12 symptoms assessed. Median progression-free survival was significantly greater (P = .03) for the 38 patients who had durable diarrhea improvement than the 18 patients who did not (18.2 v 7.9 months, respectively). Adverse events (AEs) were reported in 96.7% (87 of 90) of patients. These AEs consisted primarily of reversible GI events (76 of 90), which could be caused in part by concomitant administration of amino acid solution given to reduce radiation exposure to the kidneys. There was one case each of grade 3 oliguria and grade 4 renal failure, each lasting 6 days. Conclusion 90Y-edotreotide treatment improved symptoms associated with malignant carcinoid among subjects with no treatment alternatives. Treatment was well-tolerated and had an acceptable expected AE profile. PMID:20194865

  13. Carcinoid Tumors of Digestive Organs: a Clinicopathologic Study of 13 Cases

    PubMed Central

    Terada, Tadashi

    2009-01-01

    The author investigated clinicopathologic features of 13 cases of carcinoid tumor in the digestive organs. The 13 cases of carcinoid were identified from 18,267 pathological specimens of digestive organs in the last 10 years in our pathology laboratory. The tumor locations were rectum in 9 cases, duodenum in 2 cases, liver in 1 case, and stomach in 1 case. The age of the patients ranged from 52 to 82 years with a mean of 63 years. Male to female ratio was 7 : 6. The presenting symptoms were abnormal pain in 3 cases and asymptomatic in 10 cases. None of the cases showed carcinoid syndrome. The diameter ranged from 5 mm to 25 mm in gastrointestinal carcinoids, and 60 mm in the hepatic carcinoid. The treatment was endoscopic mucosal resection in 10 cases and surgical resection in 3 cases. The outcome is good except for hepatic atypical carcinoid which showed metastases and died of systemic metastasis. Histologically, 12 carcinoid tumors were typical carcinoids, and one (liver) was atypical carcinoid. Organoid pattern was present in 12 cases. Trabecular arrangement, ribbon arrangement, rosette formation, and pseudoglandular arrangement were recognized in 12 cases, in 8 cases, in 7 cases, and in 5 cases, respectively. Immunohistochemically, tumor cells were positive for at least one of pan-neuroendocrine markers including chromogranin, synaptophysin, neuron-specific enolase, CD56, and glucagon. Of these, synaptophysin was positive in 11/13 (85%), neuron-specific enolase 10/13 (80%), chromogranin 8/13 (62%), CD56 6/13 (46%), and glucagon 4/13 (31%). In summary, the author reported the incidence of digestive organ carcinoid tumors, and the clinicopathologic features of the 13 cases with carcinoid. PMID:27956948

  14. Bilateral Choroidal Metastases from Endobronchial Carcinoid Treated with Somatostatin Analogues

    PubMed Central

    De Bruyn, Deborah; Lamont, Jan; Vanderstraeten, Erik; Van Belle, Simon; Platteau, Elise; De Zaeytijd, Julie; Hoornaert, Kristien P.

    2016-01-01

    Objective: To describe a patient with bilateral multifocal choroidal metastases from an endobronchial carcinoid treated with a somatostatin analogue. Method: A 60-year-old woman presenting with photopsia in the left eye underwent an extensive ophthalmic examination, including fluorescein angiography, OCT and ultrasound. Results: Fundoscopy revealed a small retinal tear in the left eye, for which she received laser treatment. In addition, choroidal masses were detected in both eyes. Her medical history of a pneumectomy for a bronchial carcinoid six years earlier together with recent elevated chromogranin A blood levels prompted a diagnosis of choroidal metastases. Subsequently, a Gallium-68 DOTANOC positron emitting tomography/computer tomography scan revealed a spinal cord metastasis and mediastinal as well as mesenterial lymph node invasion. Systemic treatment with Sandostatin®, a somatostatin analogue was started. Up until two years after the initial presentation and treatment, these choroidal lesions remained stable without any signs of growth. Conclusion: Endobronchial carcinoid tumors have an indolent nature and long-term follow-up is recommended for early detection of metastases. Although treatment with somatostatin analogues rarely induces complete tumor regression, tumor stabilization and prevention of symptoms related to hormone secretion is achieved. This well-tolerated systemic treatment provides a worthy alternative treatment for choroidal metastasis compared to classic radiotherapy without any risk of radiation or laser-related visual loss. PMID:27843513

  15. Paraneoplastic neuromyelitis optica spectrum disorder associated with stomach carcinoid tumor.

    PubMed

    Al-Harbi, Talal; Al-Sarawi, Adnan; Binfalah, Mohamed; Dermime, Said

    2014-09-01

    Neuromyelitis optica (NMO), or Devic's syndrome, is an autoimmune central nervous system demyelinating disorder primarily affecting the spinal cord and the optic nerves. It is characterized by the presence of NMO antibodies, alongside clinical and radiological findings. NMO and NMO-spectrum disorders (NMO-SD) have been reported in autoimmune disorders, and are infrequently described as a paraneoplastic syndrome with cancers of lung, breast, and carcinoid tumors of the thyroid. We report a patient who presented with severe vomiting, blurring of vision, vertigo, diplopia, left hemiparesis and hemisensory loss and ataxia. She was found to have a longitudinally-extensive demyelinating lesion extending from the medulla to the upper cervical spinal cord on MRI. Her gastric endoscopy revealed carcinoid tumor of the stomach, and classic paraneoplastic antibodies in the serum were negative. She had extremely high serum gastrin level and high titer of NMO IgG autoantibody. The patient made an excellent recovery with tumor resection and immunotherapy, with both clinical and radiological improvement. On rare instances, NMO or NMO-SD may present as a paraneoplastic neurological syndrome associated with carcinoid tumor of the stomach. Copyright © 2014 King Faisal Specialist Hospital & Research Centre. Published by Elsevier B.V. All rights reserved.

  16. Carcinoid tumors: CT and I-131 meta-iodo-benzylguanidine scintigraphy

    SciTech Connect

    Adolph, J.M.; Kimmig, B.N.; Georgi, P.; zum Winkel, K.

    1987-07-01

    The diagnostic value of computed tomography (CT) and iodine-131 meta-iodo-benzylguanidine (MIBG) scintiscanning was studied in nine patients with histologically proved carcinoid tumors of intestinal (n = 4), bronchial (n = 3), or thymic (n = 2) origin. CT scans clearly depicted the tumors and metastases in relation to surrounding vital structures but did not provide findings specific for carcinoids. The appearance on CT of an abdominal soft-tissue mass with a radiating pattern of linear densities was found to be highly suggestive of intestinal carcinoid tumors. I-131 MIBG scintiscans disclosed intense tracer uptake in the tumors and metastases in five patients. MIBG studies correctly depicted nine of nine tumor manifestations in intestinal carcinoids and four of six tumor manifestations in bronchus carcinoids. No MIBG concentration was found in thymus carcinoids. Because of its selective uptake mechanism, I-131 MIBG scintigraphy can allow specific detection and localization of neuroendocrine tumor tissue in patients with suspected carcinoid tumors. MIBG scintigraphy has diagnostic potential as a screening procedure in carcinoid tumors, especially those of intestinal origin.

  17. Late recurrent pulmonary typical carcinoid tumor: case report and review of the literature.

    PubMed

    Ciment, Ari; Gil, Joan; Teirstein, Alvin

    2006-10-01

    Carcinoid tumors are uncommon pulmonary neoplasms. They are classified histologically as either atypical or typical. Atypical carcinoids are aggressive malignancies that require radical surgical resection and have a guarded prognosis with a propensity to metastasize and recur. Typical carcinoids are low-grade malignancies with relatively less metastatic or recurring potential and are usually treated with simple excision. Recurrence of a typical pulmonary carcinoid tumor more than a decade after initial resection is very rare. A patient with recurrence of a typical carcinoid tumor 11 years after resection of the primary lesion with one involved lymph node is reported here. Late recurrences are rare in both atypical and typical varieties, but are much more common in atypical carcinoids. The patient reported here represents the fifth case of recurrence of a typical carcinoid tumor more than ten years after resection. This suggests that, after resection of a typical carcinoid neoplasm, patients should be monitored carefully, especially if lymph node metastases are present at the time of surgery.

  18. Laser excision of a typical carcinoid tumor of the larynx: a case report.

    PubMed

    Cevizci, Raşit; Karakullukçu, Barış; van den Brekel, Michiel W M; Balm, Alfons J

    2010-01-01

    Neuroendocrine carcinomas of the larynx include a range of rare tumors which have variable biologic behavior, affecting treatment and prognosis. Among these, typical carcinoid tumors are the least common type. Prognosis of typical carcinoid tumor is better than atypical carcinoid tumor and small cell carcinoma of the larynx. Conservation surgery is the preferred treatment modality. Transoral CO2 laser surgery can be a good alternative for appropriate cases because of the functional results and less morbidity. In this article, a 71-year-old female presented with complaints of feeling a mass during swallowing. Fiberoptic examination of the larynx revealed a mass located on the right aryepiglottic fold and biopsy revealed the tumor as a typical carcinoid tumor. We describe CO2 laser excision of a typical carcinoid tumor of the larynx in this case report.

  19. Intestinal ischaemia associated with carcinoid tumor: a case report with review of the pathogenesis.

    PubMed

    Yener, O

    2013-01-01

    Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that are often indolent and may not become clinically apparent until there is a metastatic spread or evidence of carcinoid syndrome. A 44-year-old man presented to our clinic department with a history of previous left colon cancer operation, chronic crampy left lower quadrant pain, mass and severe anemia. A MR scan was obtained which demonstrated a calcified mesenteric mass 12×8×10 cm diameter with surrounding left colon mesenteric infiltration. The liver was normal. A case of ischaemic ileal necrosis is reported. It was associated with elastic vascular sclerosis produced by mesenteric metastases of an ileal carcinoid tumor. It is postulated that intestinal ischaemia may be of more importance in the production of abdominal pain by carcinoid tumors than has been generally accepted, and that it is the result of functional and structural changes in and around the mesenteric blood vessels, caused by substances secreted by the carcinoid tumor.

  20. A case of carcinoid tumor of the terminal ileum and simultaneous ovarian dermoid cyst.

    PubMed

    Hamada, Hosam

    2011-04-01

    Carcinoid tumors are slow-growing malignant neoplasms associated with an indolent clinical course. About 60% of such tumors are located within the gastrointestinal tract. Here, we describe the first case, to our knowledge, of a carcinoid tumor of the terminal ileum and a simultaneous ovarian dermoid cyst. A 57-year-old woman was presented with abdominal pain, vomiting, and clinical signs of mechanical bowel obstruction. Radiograph and computed tomography scan of the abdomen showed hydroaeric levels. Laparotomy revealed a mass in the terminal ileum and a right ovarian cystic mass. Right hemicolectomy and right oopherectomy were performed. The histopathological workup showed a carcinoid tumor of the terminal ileum and ovarian dermoid cyst. Small intestinal carcinoid tumor, an uncommon disease, has been reported earlier to coincide with various neoplasms. No association between small intestinal carcinoid tumor and ovarian dermoid cyst has been reported earlier in the English literature.

  1. Renal carcinoid tumor with liver metastasis followed up postoperatively for 9 years.

    PubMed

    Ouyang, BinShen; Ma, XiaoMei; Yan, HongZhu; He, Jin; Xia, ChunYan; Yu, HongYu

    2015-10-06

    We describe a case of renal carcinoid tumor with liver metastasis followed up postoperatively for 9 years. A 33-year-old man presented with left flank dull ache. On the abdominal computed tomography, a solid renal mass in the upper portion of the left kidney was detected. The patient had no other abnormal findings, such as suspected distant metastasis or lymph node metastasis. Radical nephrectomy was performed on 14/9/2005. Histological examination and immunohistochemical staining confirm primary renal carcinoid tumor. 9 years after radical nephrectomy, computed tomography of the abdomen demonstrated a 2 cm × 1.8 cm cyst mass in the right liver. Similar pathologic characteristics were found between the renal carcinoid tumor and liver tumor. We present a primary renal carcinoid tumor with liver metastasis 9 years after radical nephrectomy. With literature review, renal carcinoid tumors exhibit heterogenous behavior.

  2. Primary Carcinoid Tumor of the Ileal Efferent Limb of an Ileovesicostomy: A Case Report

    PubMed Central

    Mellis, Adamantios M.; Parker, Daniel C.; Buethe, David D.; Slobodov, Gennady

    2011-01-01

    We report on the evaluation and management of a 47-year-old white male found to have primary carcinoid tumor of the ileal segment of his diverting ileovesicostomy thirty-five months after initial creation. Subsequent to presentation with intermittent gross hematuria, CT urogram highlights an 8 mm enhancing lesion near the enterovesical junction of urinary diversion. Office cystoscopy confirms presence of a lesion that was later endoscopically resected and found to be a well-differentiated carcinoid tumor. Evaluation with serum markers, direct visualization utilizing endoscopy, and imaging was without finding of alternate primary or metastatic lesions. The patient ultimately had the proximal ileal portion of his ileovesicostomy excised and the distal portion converted into an ileal conduit. After briefly discussing the carcinoid tumor and the carcinoid syndrome it may cause, we review the literature on the incidence of carcinoid tumors in a population requiring the use of intestine in the urinary tract. PMID:22606607

  3. Pediatric neuroendocrine carcinoid tumors: Management, pathology, and imaging findings in a pediatric referral center.

    PubMed

    Degnan, Andrew J; Tocchio, Shannon; Kurtom, Waleed; Tadros, Sameh S

    2017-09-01

    While neuroendocrine (carcinoid) tumors are increasingly recognized in the adult population, they are often not suspected in children. Retrospective review of all well-differentiated neuroendocrine (carcinoid) tumors was performed based on pathology reports from a quaternary pediatric medical center between January 2003 and June 2016. Clinical presentations, treatment approaches, imaging findings, and outcomes were reviewed and analyzed. A total of 45 cases of pathology-proven carcinoid tumor were reported with an average age of 14.1 years (range: 7-21 years, SD: 2.8 years). Of these cases, 80% (36) were appendiceal, 11% (5) bronchial, 2% (1) colonic, 2% (1) gastric, 2% (1) enteric, and 2% (1) testicular. Metastases were observed in one (3%) appendiceal, one (100%) enteric, and two (40%) bronchial cases. No recurrence was demonstrated in any appendiceal carcinoid cases. Recurrence was seen in one of three extra-appendiceal gastrointestinal tumors. Tumor site and size significantly correlated with metastases and recurrence. Contrary to recent epidemiological investigations in adults, appendiceal carcinoid tumors remain the most common site for pediatric carcinoid tumors. Appendiceal carcinoid tumors exhibited benign clinical courses without recurrence during short-term follow-up. Extra-appendiceal gastrointestinal carcinoid tumors exhibited much more aggressive behavior with greater metastases and recurrence. Bronchial carcinoid tumors demonstrated good clinical response to resection even in cases with mediastinal lymph node involvement. While increased use of urine 5-HIAA levels and somatostatin receptor-specific imaging might improve detection and guide management of extra-appendiceal carcinoid tumors, longer-term follow-up is needed. © 2017 Wiley Periodicals, Inc.

  4. Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

    PubMed Central

    Wang, Horng-Yuan; Chen, Ming-Jen; Yang, Tsen-Long; Chang, Ming-Chih; Chan, Yu-Jan

    2005-01-01

    Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501000/µL, and leukocytosis of 20410/µL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally. PMID:15968742

  5. [Severe tricuspid insufficiency and primary carcinoid tumor of the ovary. Long term success after valve replacement. Apropos of a case].

    PubMed

    Herreman, F; Vernant, P; Cachera, J P; Monier, P

    1978-01-01

    The case is reported of carcinoid heart disease in a lady of 70 with intractable congestive cardiac failure 5 years after the removal of a primary carcinoid tumour of the ovary. The special features of primary carcinoid tumours of the ovary are recalled, with emphasis on their rarity and of the absence of liver metastases. The various features of carcinoid syndrome are recalled in the light of current knowledge of the pathogenesis. A review of the literature on cardiac involvement in primary carcinoid tumours of the ovary, amounting to 10 cases, is included. The possibility of surgical cure of the heart lesions in carcinoid tumour by a prosthetic tricuspid valve are discussed, in the light of the 6 reported cases and the present one. Our report is the first one of replacement of the valve after removal of a primary ovarien carcinoid tumour, and the excellent result has been maintained after three years.

  6. Development of effective prophylaxis against intraoperative carcinoid crisis.

    PubMed

    Woltering, Eugene A; Wright, Anne E; Stevens, Melissa A; Wang, Yi-Zarn; Boudreaux, John P; Mamikunian, Gregg; Riopelle, James M; Kaye, Alan D

    2016-08-01

    The prophylactic use of a preoperative, intraoperative, and postoperative high-dose continuous octreotide acetate infusion was evaluated for its ability to minimize the incidence of carcinoid crises during neuroendocrine tumor (NET) cytoreductive surgeries. A retrospective study was approved by the institutional review boards at Ochsner Medical Center-Kenner and Louisiana State University Health Sciences Center. Ochsner Medical Center-Kenner operating room and multispecialty NET clinic. One hundred fifty consecutive patients who underwent a total of 179 cytoreductive surgeries for stage IV, small bowel NETs. All patients received a 500-μg/h infusion of octreotide acetate preoperatively, intraoperatively, and postoperatively. Anesthesia and surgical records were reviewed. Carcinoid crisis was defined as a systolic blood pressure of less than 80mm Hg for greater than 10minutes. Patients who experienced intraoperative hypertension or hypotension, profound tachycardia, or a "crisis" according to the operative note were also reviewed. One hundred sixty-nine (169/179; 94%) patients had normal anesthesia courses. The medical records of 10 patients were further investigated for a potential intraoperative crisis using the aforementioned criteria. Upon review, 6 patients were determined to have had a crisis. The final incidence of intraoperative crisis was 3.4% (6/179). A continuous high-dose infusion of octreotide acetate intraoperatively minimizes the incidence of carcinoid crisis. We believe that the low cost and excellent safety profile of octreotide warrant the use of this therapy during extensive surgical procedures for midgut and foregut NETs. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Expanding indications for ESD: submucosal disease (SMT/carcinoid tumors).

    PubMed

    Goto, Osamu; Uraoka, Toshio; Horii, Joichiro; Yahagi, Naohisa

    2014-04-01

    Endoscopic submucosal dissection (ESD) is useful for submucosal tumors (SMTs) within the superficial submucosal layer, but perforation frequently occurs during ESD for SMTs located at the deeper layer. Endoscopic resection for small esophageal SMTs is acceptable, although candidates for endoscopic removal are rare. Laparoscopic assistance will be effective for minimally invasive endoscopic local resection for certain types of gastric SMT. Endoscopic mucosal resection with a ligation device would be better than ESD for rectal carcinoid in terms of simplicity and effectiveness. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Pulmonary and tricuspid valvuloplasty in carcinoid heart disease.

    PubMed

    Karimi, Ashkan; Pourafshar, Negiin; Fudge, James C

    2016-12-28

    A 26-year-old female with carcinoid heart disease consisting of severe pulmonary and tricuspid valve stenosis was admitted with line associated sepsis. She recovered from sepsis with antibiotics and aggressive fluid resuscitation but became grossly volume overloaded with evidence of tense ascites and lower extremity edema. She developed worsening renal and hepatic function due to congestive nephropathy and hepatopathy, which did not respond to intravenous diuretics, and she was deemed too sick for surgical pulmonary and tricuspid valve replacement. Pulmonary and tricuspid valvuloplasty was performed as a rescue measure to alleviate her congestive symptoms and improve her candidacy for valve replacement. © 2016 Wiley Periodicals, Inc.

  9. Familial risk of small intestinal carcinoid and adenocarcinoma.

    PubMed

    Kharazmi, Elham; Pukkala, Eero; Sundquist, Kristina; Hemminki, Kari

    2013-08-01

    Small intestinal cancer (SIC) is rare, and its etiology is poorly understood. We compared clusters of families with SICs of different histologic subtypes. By using the nationwide family cancer data sets of Sweden and Finland, we identified a cohort of 9964 first-degree relatives of 1799 patients with SIC, diagnosed from 1961 through 2009. Data were collected from time periods as long as 47 years (mean, 35.4 y), and cancer incidence was determined. Standardized incidence ratios (SIRs) were calculated and stratified by sex, age, time period, and cancer type, using the incidence rates for the entire national population as the reference. Among the 1799 SIC cases, 1.1% had a sibling with SIC, so the SIR was 11.8 (95% confidence interval [CI], 7.2-18.2); 1.1% had a parent or child with SIC (SIR, 3.5; 95% CI, 2.0-5.6). The SIR of concordant carcinoid histology of SIC among siblings was 28.4 (95% CI, 14.7-49.6; n = 12) and in parent-child pairs was 9.9 (95% CI, 5.4-16.6; n = 14). The familial risk of concordant histologic subtypes increased for siblings diagnosed with adenocarcinoma, but only 2 familial cases were identified. In family members of patients with SIC of the adenocarcinoma subtype, risks of colorectal and bladder cancer were modestly but significantly increased compared with the general population. Family members of patients with SIC of the carcinoid subtype had an increased risk for kidney cancer and polycythemia vera. Based on data from our population-based study, first-degree relatives of patients with small intestinal carcinoid tumors have developed these tumors with high incidence. Because of the rareness of this tumor, the absolute risk remains moderate even within families. Gastroenterologists could inform patients with small intestinal carcinoids about the familial risk and encourage counseling for their first-degree relatives. Studies are needed to identify genetic factors that affect susceptibility to SIC. Copyright © 2013 AGA Institute. Published

  10. Refractory carcinoid syndrome: a review of treatment options

    PubMed Central

    Riechelmann, Rachel P.; Pereira, Allan A.; Rego, Juliana F. M.; Costa, Frederico P.

    2016-01-01

    Carcinoid syndrome (CSy) is a constellation of symptoms that may commonly present in patients with well differentiated neuroendocrine tumors (NETs), with somatostatin analogs (SSAs) being the first-line option for symptom management. However, symptomatic progression eventually occurs and in this scenario of a refractory CSy; several treatment options have been studied such as dose escalation of SSA, interferon and liver-directed therapies. Nevertheless, recent phase III trials have contributed to the understanding and management of this condition. We performed a comprehensive review of interventional studies examining refractory CSy to provide the evidence for current treatment options and propose a treatment sequence. PMID:28203303

  11. The effect of preceding biopsy on complete endoscopic resection in rectal carcinoid tumor.

    PubMed

    Lee, Sang Pyo; Sung, In-Kyung; Kim, Jeong Hwan; Lee, Sun-Young; Park, Hyung Seok; Shim, Chan Sup

    2014-04-01

    Biopsy of rectal carcinoid tumor is commonly taken before endoscopic resection. However the preceding biopsy can inhibit complete resection by causing blurred tumor border and fibrosis of the tissue. The objective of the study was to investigate the effect of preceding biopsy on complete endoscopic resection in rectal carcinoid tumor. It was also determined if rectal carcinoid tumors can be macroscopically distinguished by endoscopy. We reviewed retrospectively the records of patients with rectal carcinoid tumor who had undergone an endoscopic treatment at our hospital, during a 7-yr period. The resection margin was clear in 57 of 98 cases. The preceding biopsy was taken in 57 cases and the biopsy was significantly associated with the risk of incomplete tumor resection (OR, 3.696; 95% CI, 1.528-8.938, P = 0.004). In 95.9% of the cases, it was possible to suspect a carcinoid tumor by macroscopic appearance during initial endoscopy. The preceding biopsy may disturb complete resection of rectal carcinoid tumor. In most cases, the carcinoid tumor could be suspected by macroscopic appearance. Therefore the preceding biopsy is not essential, and it may be avoided for the complete resection.

  12. [Ovarian carcinoid tumor responsible of permanent facial erythrosis and flushings during sexual intercourse].

    PubMed

    Talvande, B; Dorange, A; Lecouflet, M; Le Nezet, M; Kianifard, B; Maillard, H; Duquenne, M

    2016-11-01

    Ovarian neuroendocrine tumors are extremely rare. In the ovary, the term of neuroendocrine tumor is usually related to carcinoid tumors although it may be sometimes applied to neuroendocrine carcinomas of non-small cells or small cells cancers usually occurring in the lungs. These carcinoid tumors may develop de novo or from other tumors including teratomas. We report a patient who presented with an ovarian carcinoid tumor developed, near or from a dermoid cyst, which was original by its mode of discovery in the dermatology department. Indeed, the patient consulted because of permanent facial erythrosis, with flushes but also facial telangiectasias. During medical examination, classic symptoms of carcinoid syndrome including heart disease were obvious. The occurrence of flushes during coitus should evoke pelvic tumor location. A carcinoid syndrome is naturally evoked in the presence of flushes but it must also be part of the differential diagnosis in a patient with facial erythrosis or telangiectasias, especially if they are associated with diarrhea or right heart failure. The prognosis of carcinoid heart disease is considerably better in case of ovarian location than when it is a primitive carcinoid tumor of lung or from gastrointestinal tract. Copyright © 2016 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  13. Minute ampullary carcinoid tumor with lymph node metastases: a case report and review of literature

    PubMed Central

    Senda, Eri; Fujimoto, Koji; Ohnishi, Katsuhiro; Higashida, Akihiro; Ashida, Cho; Okutani, Toshio; Sakano, Shigeru; Yamamoto, Masayuki; Ito, Rieko; Yamada, Hajime

    2009-01-01

    Background Carcinoid tumors are usually considered to have a low degree of malignancy and show slow progression. One of the factors indicating the malignancy of these tumors is their size, and small ampullary carcinoid tumors have been sometimes treated by endoscopic resection. Case presentation We report a case of a 63-year-old woman with a minute ampullary carcinoid tumor that was 7 mm in diameter, but was associated with 2 peripancreatic lymph node metastases. Mild elevation of liver enzymes was found at her regular medical check-up. Computed tomography (CT) revealed a markedly dilated common bile duct (CBD) and two enlarged peripancreatic lymph nodes. Endoscopy showed that the ampulla was slightly enlarged by a submucosal tumor. The biopsy specimen revealed tumor cells that showed monotonous proliferation suggestive of a carcinoid tumor. She underwent a pylorus-preserving whipple resection with lymph node dissection. The resected lesion was a small submucosal tumor (7 mm in diameter) at the ampulla, with metastasis to 2 peripancreatic lymph nodes, and it was diagnosed as a malignant carcinoid tumor. Conclusion Recently there have been some reports of endoscopic ampullectomy for small carcinoid tumors. However, this case suggests that attention should be paid to the possibility of lymph node metastases as well as that of regional infiltration of the tumor even for minute ampullary carcinoid tumors to provide the best chance for cure. PMID:19159493

  14. Rectal carcinoids are on the rise: early detection by screening endoscopy.

    PubMed

    Scherübl, H

    2009-02-01

    Rectal carcinoids are on the rise; in the United States the age-adjusted incidence has increased by 800% -1000% in the last 35 years. The incidence of carcinoids of the stomach, pancreas, or small bowels has also multiplied. The reasons for these epidemiological changes are not yet understood. Both screening sigmoidoscopy and screening colonoscopy lead to a shift to smaller-sized (< or =13 mm) rectal carcinoids and earlier tumor stages at diagnosis. During the last 35 years the overall 5-year survival of patients with rectal carcinoid disease has increased by almost 20% (in the US). Thus, endoscopic screening of the colorectum is effective in the early diagnosis not only of colorectal adenomas and adenocarcinomas but also of carcinoids. Rectal carcinoids that are 10.0 mm or less and do not infiltrate the muscularis propria can be removed endoscopically. If histological angioinvasion or lymph node metastases are found, surgical lymph node dissection has to be considered. Before deciding on definitive therapy, rectal carcinoids should be staged by means of endoscopic ultrasonography, CT, or MRI and somatostatin receptor scintigraphy.

  15. Primary ovarian carcinoid tumor showing unusual histology and nuclear accumulation of β-catenin.

    PubMed

    Kim, Hyun-Soo; Yoon, Gun; Jang, Hye-In; Song, Sang Yong; Kim, Byoung-Gie

    2015-01-01

    Carcinoid tumor of the ovary is uncommon. We herein report a very rare case of primary ovarian carcinoid tumor with aggressive histology and an unusual immunophenotype. A 21-year-old woman presented with a palpable abdominal mass. Computed tomographic scan revealed a large, extensively necrotic solid mass in the left ovary. The patient underwent a left salpingo-oophorectomy. Grossly, the left adnexa showed a large, vaguely lobulated ovarian tumor measuring 22×15×13 cm. Histologically, the tumor had a readily identifiable neuroendocrine growth pattern, but some areas showed solid growth pattern associated with mild nuclear pleomorphism and multiple foci of punctate necrosis. Furthermore, mitotic figures were recognized in 8 per 10 high-power fields, and a few foci of large coagulative tumor necrosis were also noted. In addition, the tumor tissue exhibited uniform, strong nuclear β-catenin immunoreactivity, indicating the nuclear accumulation of β-catenin in the individual tumor cells. In summary, we described the first case of primary ovarian carcinoid tumor with loss of neuroendocrine growth pattern, increased mitotic activity and large areas of coagulative tumor necrosis. According to the WHO classification of pulmonary carcinoid tumor, this case may be classified as "atypical" carcinoid. However, currently, no primary ovarian atypical carcinoid exists in the classification system. Due to its rarity, there are no established diagnostic criteria and clinical data on patient outcomes for ovarian carcinoid tumors with aggressive histology. Additional reports are clearly necessary. We also showed for the first time the nuclear accumulation of β-catenin in carcinoid tumor cells, suggestive of a role for β-catenin in the tumorigenesis of ovarian atypical carcinoid tumor or its aggressive histology.

  16. Primary ovarian carcinoid tumor showing unusual histology and nuclear accumulation of β-catenin

    PubMed Central

    Kim, Hyun-Soo; Yoon, Gun; Jang, Hye-In; Song, Sang Yong; Kim, Byoung-Gie

    2015-01-01

    Carcinoid tumor of the ovary is uncommon. We herein report a very rare case of primary ovarian carcinoid tumor with aggressive histology and an unusual immunophenotype. A 21-year-old woman presented with a palpable abdominal mass. Computed tomographic scan revealed a large, extensively necrotic solid mass in the left ovary. The patient underwent a left salpingo-oophorectomy. Grossly, the left adnexa showed a large, vaguely lobulated ovarian tumor measuring 22×15×13 cm. Histologically, the tumor had a readily identifiable neuroendocrine growth pattern, but some areas showed solid growth pattern associated with mild nuclear pleomorphism and multiple foci of punctate necrosis. Furthermore, mitotic figures were recognized in 8 per 10 high-power fields, and a few foci of large coagulative tumor necrosis were also noted. In addition, the tumor tissue exhibited uniform, strong nuclear β-catenin immunoreactivity, indicating the nuclear accumulation of β-catenin in the individual tumor cells. In summary, we described the first case of primary ovarian carcinoid tumor with loss of neuroendocrine growth pattern, increased mitotic activity and large areas of coagulative tumor necrosis. According to the WHO classification of pulmonary carcinoid tumor, this case may be classified as “atypical” carcinoid. However, currently, no primary ovarian atypical carcinoid exists in the classification system. Due to its rarity, there are no established diagnostic criteria and clinical data on patient outcomes for ovarian carcinoid tumors with aggressive histology. Additional reports are clearly necessary. We also showed for the first time the nuclear accumulation of β-catenin in carcinoid tumor cells, suggestive of a role for β-catenin in the tumorigenesis of ovarian atypical carcinoid tumor or its aggressive histology. PMID:26191292

  17. Chemotherapy and irradiation for locally advanced and metastatic pulmonary carcinoid tumors

    PubMed Central

    Chong, Curtis R.; Wirth, Lori J.; Nishino, Mizuki; Chen, Aileen B.; Sholl, Lynette M.; Kulke, Matthew H.; McNamee, Ciaran J.; Jänne, Pasi A.; Johnson, Bruce E.

    2015-01-01

    Objectives The optimal management of locally advanced and metastatic pulmonary carcinoid tumors remains to be determined. Materials and methods A retrospective review was conducted on patients with typical and atypical pulmonary carcinoid tumors treated at our institutions between 1990 and 2012. Results 300 patients were identified with pulmonary carcinoid, (80 patients with atypical carcinoid), of whom 29 presented with metastatic disease (16 atypical). Of evaluable patients, 26 (41%) with stages I–III atypical carcinoid tumors recurred at a median time of 3.7 years (range, 0.4–32), compared to 3 (1%) patients with typical carcinoid (range, 8–12.3). 39 patients were treated with chemotherapy, including 30 patients with metastatic disease (27 atypical), and 7 patients were treated with adjuvant platinum–etoposide chemoradiation (6 atypical, 1 typical, 6 stage IIIA, 1 stage IIB). At a median follow-up of 2 years there were 2 recurrences in the 7 patients receiving adjuvant treatment. Median survival after diagnosis of metastatic disease for patients with atypical pulmonary carcinoid was 3.3 years with a 5-year survival of 24%. Treatment regimens showing efficacy in pulmonary carcinoid include 15 patients treated with octreotide-based therapies (10% response rate (RR), 70% disease control rate (DCR), 15 month median progression-free survival (PFS)), 13 patients treated with etoposide + platinum (23% RR, 69% DCR, 7 month median PFS), and 14 patients treated with temozolomide-based therapies (14% RR, 57% DCR, 10 month median PFS). 8 of 10 patients with octreotide-avid disease treated with an octreotide-based regimen experienced disease control (1 partial response, 7 stable disease) for a median of 18 months (range 6–72 months). Conclusions These results support our previous finding that a subset of pulmonary carcinoid tumors are responsive to chemotherapy. PMID:25218177

  18. [Somatotropic pituitary tumor in a patient with a previous ovarian strumal carcinoid. Report of one case].

    PubMed

    Riedemann, Erick; Rojas, Claudio; Quevedo, Iván

    2004-07-01

    Strumal carcinoid is an unusual form of monodermal ovarian teratoma with thyroid-like follicles mixed with typical carcinoid tumor patterns. We report a 49 years old woman presenting with an acromegaly. At the age of 45, an ovarian strumal carcinoid was excised and at three years of follow up, she complained of finger and feet growth. Laboratory showed an elevated serum IGF-1 and a sella turcica magnetic resonance imaging showed an 8 mm microadenoma. She was subjected to a transphenoidal excision and the pathological study disclosed a solid acidophilic pituitary adenoma. Two months after surgery, there was a significant decrease in serum IGT-I levels.

  19. Diode Laser in Management of Tracheobronchial Typical Carcinoid: A Case Report with Review of Literature

    PubMed Central

    Bhambhani, Navin; Masurkar, Vishwanath; Thombre, Sonal

    2015-01-01

    Tracheobronchial carcinoids are relatively uncommon neoplasms with malignant (though indolent) potential and variable behavior. Clinical presentation is usually similar to any obstructive tracheobronchial lesions or rarely with presentation of carcinoid syndrome. Management varies from Pneumonectomy to use of lung sparing bronchoscoplastic techniques. We report the case of a 32-year-old male patient diagnosed with carcinoid of left main bronchus. Patient was successfully treated with trans-bronchoscopic diode laser. At 24 months of follow up patient remains asymptomatic and recurrence-free. PMID:26500999

  20. Atypical Carcinoid Tumor with Anaplastic Lymphoma Kinase (ALK) Rearrangement Successfully Treated by an ALK Inhibitor.

    PubMed

    Nakajima, Masayuki; Uchiyama, Naoki; Shigemasa, Rie; Matsumura, Takeshi; Matsuoka, Ryota; Nomura, Akihiro

    This is the first report in which crizotinib, an anaplastic lymphoma kinase (ALK) inhibitor, reduced an atypical carcinoid tumor with ALK rearrangement. A 70-year-old man developed a tumor in the left lung and multiple metastases to the lung and brain. The pathology of transbronchial biopsied specimens demonstrated an atypical carcinoid pattern. Combined with immunohistochemical findings, we diagnosed the tumor as atypical carcinoid. ALK gene rearrangement was observed by both immunohistochemical (IHC) and fluorescence in situ hybridization. He was treated with chemotherapy as first-line therapy, however, the tumor did not respond to chemotherapy. Thereafter, he was treated with crizotinib, which successfully reduced the tumors.

  1. A Rare Primary Neuroendocrine Tumor (Typical Carcinoid) of the Sublingual Gland

    PubMed Central

    Ohki, Kousuke; Uchida, Fumihiko; Kanno, Naomi; Hasegawa, Shogo; Yanagawa, Toru; Bukawa, Hiroki

    2016-01-01

    A typical carcinoid is extremely rare in the oral cavity. We here present a case of a typical carcinoid arising in the sublingual gland of a 62-year-old woman. The tumor was removed by primary excision with 10 mm surgical margins and submandibular dissection. Examination of the tumor showed medium-sized tumor cells that were positive for CD56 and chromogranin A, with no necrosis, and with a mitotic count less than 1/10 HPF. A pathological diagnosis of typical carcinoid was made from both morphological and immunological examinations. One year after excision surgery, there was no tumor recurrence or neck metastasis. PMID:27840746

  2. Whole abdominal radiation in the management of metastatic gastrointestinal carcinoid tumor

    SciTech Connect

    Keane, T.J.; Rider, W.D.; Harwood, A.R.; Thomas, G.M.; Cummings, B.J.

    1981-11-01

    Twenty-eight patients with metastatic gastrointestinal carcinoid tumor were treated with whole abdominal radiation. Twenty-one of these patients had the carcinoid syndrome. The prescribed mid-line tumor dose was 2000 to 2500 rad in 20 to 25 fractions in 4 to 5 weeks. The overall 5 year survival was 35% (median survival 2.5 years). A marked survival difference was noted in those patients with carcinoid syndrome compared to the non-functioning group. None of the syndrome patients survived five years (median survival 1.7 years) whereas six of the seven non-syndrome patients are five year survivors.

  3. Calcitonin-producing well-differentiated neuroendocrine carcinoma (carcinoid tumor) of the urinary bladder: case report

    PubMed Central

    Mascolo, Massimo; Altieri, Vincenzo; Mignogna, Chiara; Napodano, Giorgio; De Rosa, Gaetano; Insabato, Luigi

    2005-01-01

    Background The occurrence of calcitonin-secreting primary carcinoid tumor of the urinary bladder is extremely rare. Case presentation The case of a 68-year-old male with carcinoid tumor arising in the urinary bladder is presented. Transurethral resection of a polypoid small tumor 0.4 cm in diameter was performed. Immunohistochemical study using neuroendocrine markers allowed a straightforward diagnosis of a low-grade neuroendocrine carcinoma (carcinoid tumor) of the urinary bladder. Immunohistochemistry demonstrated calcitonin immunoreactivity in the most of the tumor cells. Conclusion This tumor shows specific clinical, macroscopical and histological features and must be considered in the differential diagnosis of bladder neoplasms. PMID:16048646

  4. Association synchrone d'un cancer du sein bilatéral et d'une tumeur stromale gastro-intestinale: à propos d'un rare cas

    PubMed Central

    Afif, Mohammed; Ouziane, Imane; Kouhen, Fadila; Khalil, Jihane; Elomrani, Fedwa; Elkacemi, Hanan; Kebdani, Tayeb; Errihani, Hassan; Benjaafar, Noureddine

    2015-01-01

    Les tumeurs stromales gastro-intestinales sont les tumeurs mésenchymateuses les plus fréquentes, pourtant, leur association avec les tumeurs du sein sont rares, seulement quelques cas cliniques sont rapportés dans la littérature. Nous rapportons l'observation d'une jeune femme de 41 ans, suivie à l'institut national d'oncologie de Rabat, pour un carcinome canalaire du sein, bilatéral, chez qui le bilan d'extension a objectivé une tumeur stromale de type gastro-intestinale aux dépens de l'estomac. Nous décrivons à travers cette observation les aspects épidémiologiques, cliniques, et les particularités de la prise en charge de cette association rare. PMID:26090003

  5. Serum alpha-fetoprotein-positive gastric carcinoid with liver metastasis.

    PubMed

    Mori, H; Onji, M; Yoshida, A; Fukunishi, R

    1980-01-01

    The patient was a 60-year-old Japanese male. He complained of epigastralgia and right chest pain of 4 month's duration, and general malaise, nausea and vomiting of 2 month's duration. Physical examination revealed on the right third rib a tender mass with a diameter of 2 cm and hepatomegaly with a multi-nodular surface and red palms. There were no signs of carcinoid syndrome, such as cutaneous flushing. Laboratory examinations disclosed certain biochemical alterations; alkaline phosphatase 810 IU/l, gamma-glutamyl transpeptidase (gamma-GTP) 2090 IU/l, carcinoembryonic antigen (CEA) 23.5 ng/ml and alpha-fetoprotein (AFP) 6,800 ng/ml. Both HBs-Ag and HBs-Ab were negative. The patient died in a uremic state, with rapid increases of jaundice and ascites. Autopsy revealed gastric carcinoid with extensive metastases to the liver and the bone marrow. Tumor cells showed argyrophilia but not argentaffinity. Immunofluorescence specific for AFP was positive in the hepatocytes, particularly those adjacent to the metastatic tumor cells but not in the tumor cells, either primary or secondary. 79 cases reported in Japan of serum AFP-positive malignant tumor other than hepatocellular carcinoma and certain other malignancies of germ cell origin are reviewed and discussed.

  6. Duodenal and Ampullary Carcinoid Tumors: Size Predicts Necessity for Lymphadenectomy.

    PubMed

    Dogeas, Epameinondas; Cameron, John L; Wolfgang, Cristopher L; Hirose, Kenzo; Hruban, Ralph H; Makary, Martin A; Pawlik, Timothy A; Choti, Michael A

    2017-08-01

    Unlike other neuroendocrine tumors of the gastrointestinal tract, management of duodenal and periampullary carcinoids remains controversial. We aimed to determine the metastatic potential and optimal choice of therapy for these neoplasms. A retrospective review of all patients treated at the Johns Hopkins Hospital between 1996 and 2012 was conducted. Clinicopathologic factors associated with lymph nodal involvement and clinical outcomes were evaluated. A total of 101 patients were identified. Eighty (79.2%) tumors arose from the duodenum and 21 (20.8%) from the periampullary area. Thirty-five (34.7%) patients underwent pancreaticoduodenectomy (PD), 12 (11.9%) local resection, 38 (37.6%) endoscopic excision, and 16 (15.8%) patients harbored incidental tumors identified in the specimen after PD for another indication. Lymph node (LN) pathologic evaluation was done in 56 patients, among which 27 (48%) had positive LN. Specifically, LN positivity (LN+) for tumors <1 cm in size was 4.5% (1/22), for tumors 1-2 cm 72% (13/18), and for tumors >2 cm 81% (13/16). Tumor size was the only factor associated with LN+ (p = 0.029). Lymph nodal involvement is common for duodenal and periampullary carcinoid tumors, particularly among those >1 cm in size; therefore, resection with lymphadenectomyfor these larger tumors is recommended.

  7. Combined adenocarcinoma-carcinoid tumor of transverse colon.

    PubMed

    Bhattacharjee, Prosanta Kumar; Halder, Shyamal

    2013-01-01

    A 65-year-old male presented with painless hematochezia associated with episodic cramps in upper abdomen, watery diarrhea, and a slowly growing mass in upper abdomen. Examination revealed a firm 6 x 5 cm, intra-abdominal, epigastric mass. Colonoscopy up to 90 cm showed a stenosing, ulcero-proliferative lesion in the transverse colon. No synchronous lesion was detected. Biopsy revealed mucin secreting adenocarcinoma. Exploration showed the growth involving the transverse colon proximal to the splenic flexure with a part of ileum, approximately three feet proximal to ileo-caecal junction, adherent to it. No significant mesenteric lymph node enlargement was evident. The patient underwent resection of the growth along with the segment of adherent ileum. Continuity was re-established by a colo-colic and ileo-ileal anastomosis respectively. Patient received adjuvant chemotherapy. Post-operative histopathology demonstrated a composite histological pattern with an admixture of carcinoid tumor and adenocarcinoma, invasion of ileal serosa and adenocarcinomatous deposits in mesocolic lymph nodes, the tumor staging being (T4, N0, M0/Stage II) for carcinoid and (T4, N1, M0/Stage III) for adenocarcinoma. Patient was followed-up for a year and was doing well without any evidence of recurrence.

  8. Complications from carcinoid syndrome: review of the current evidence

    PubMed Central

    Mota, José Mauricio; Sousa, Luana Guimarães; Riechelmann, Rachel P

    2016-01-01

    Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. In this scenario, long-term secretion of vasoactive substances—serotonin, tachynins, and others, may induce fibrogenic responses in local or distant tissues, leading to complications such as carcinoid heart disease (CHD), mesenteric and/or retroperitoneal fibrosis. Rare cases of lung/pleural fibrosis and scleroderma have also been described. Despite it not being well described yet, current evidence suggests the pathogenesis of such fibrogenic complications relies on signalling through 5-HT2B and TGF-β1. Medical management is still very limited and lacks prospective and randomised studies for definitive recommendations. Surgical procedures remain the best definitive treatment option for CHD and abdominal fibrosis. Recently, cognitive impairment has also been described as a potential consequence of CS. This review critically discusses the literature concerning the epidemiology, pathogenesis, clinical features, diagnosis, and treatment options for CS-related long-term complications. PMID:27594907

  9. Ultrastructural cytochemistry of platelets and megakaryocytes in the carcinoid syndrome.

    PubMed

    Lewis, J C; Maldonado, J E; Mann, K G; Moertel, C G

    1976-09-01

    Platelets and megakaryocytes from 11 patients with the carcinoid syndrome have been studied by transmission electron microscopy. Cells fixed in phosphate-buffered glutaraldehyde are oval to discoid, with pseudopods, a dilated open-channel system, and a prominent dense tubular system as defined by peroxidase activity and alkaline bismuth stain. Atypical with hexagonal lattices and treaded substructures and large (diameter greater than 0.5 mum), phosphatase-positive, debris-containing vacuoles are four times more numerous than in normal platelets. Incubation of platelets in a 0.05% suspension of latex results in particle incorporation into phagosomes and the debris-containing vacuoles. Molybdate-dichromate stain reveals two classes of dense bodies, one of which (with a reticular core) is 20 times more numerous than in normal platelets. Bone marrow megakaryocytes lack both dense bodies and debris vacuoles analogous to those found in circulating platelets. These results suggest autophagy or endocytosis abnormalities and provide evidence for multiple types of dense bodies in carcinoid platelets.

  10. Rectal carcinoid tumor metastasis to a skull base meningioma

    PubMed Central

    Huang, Jennifer; Gupta, Amit; Badve, Chaitra; Cohen, Mark L; Wolansky, Leo J

    2016-01-01

    Carcinoid tumors are rare, slow-growing neuroendocrine tumors that most frequently develop in the gastrointestinal tract or lungs and have high potential for metastasis. Metastasis to the brain is rare, but to another intracranial tumor is extremely rare. Of the intracranial tumors, meningiomas are the most common to host metastases, which may be related to its rich vascularity and E-cadherin expression. We describe the case of a 65-year-old female with active chemotherapy-treated neuroendocrine carcinoma who presented with left-sided facial numbness, headaches, and blurry vision. Initial imaging revealed a 1 cm irregular dural-based left petrous apex mass suggestive of a meningioma that was re-imaged four months later as a rapidly enlarging, extra-axial, mass extending into the cavernous sinus, effacing Meckel’s cave that resembled a trigeminal schwannoma. Pathology revealed a carcinoid tumor metastatic to meningioma. While the mass displayed characteristic imaging findings of a schwannoma, rapid growth in the setting of known active malignancy should prompt the clinician to consider mixed pathology from metastatic disease or a more aggressive meningioma. PMID:26825133

  11. A 12-mm carcinoid tumor of the minor duodenal papilla with lymph node metastases.

    PubMed

    Fukami, Yasuyuki; Kurumiya, Yasuhiro; Mizuno, Keisuke; Sekoguchi, Ei; Kobayashi, Satoshi; Ito, Akira; Tomida, Akihiro; Onishi, Sakura; Shirotsuki, Ryo; Okubo, Kenji; Narita, Michihiko

    2013-01-01

    Carcinoid tumors located in the minor duodenal papilla are extremely rare, with only a few cases reported in the literature. Herein, we report the case of a 71-year-old man with a 12-mm carcinoid tumor at the minor duodenal papilla with lymph node metastases. Multidetector-row computed tomography with contrast enhancement revealed a 12-mm well-enhanced tumor in the duodenum. Upper gastrointestinal endoscopy showed a 12-mm submucosal tumor at the minor papilla of the duodenum. Biopsy specimens revealed a carcinoid tumor, and a subtotal stomach-preserving pancreatoduodenectomy was performed. Carcinoid tumors at the minor duodenal papilla have a high prevalence of nodal disease, even for tumors <2 cm in diameter. Therefore, we believe that radical resection with tumor-free margins (i.e. pancreatoduodenectomy) is the treatment of choice.

  12. Primary carcinoid tumor of the urinary bladder with prominent subnuclear eosinophilic granules.

    PubMed

    Zozumi, Masataka; Nakai, Mayumi; Matsuda, Ikuo; Hao, Hiroyuki; Ueda, Yasuo; Nojima, Michio; Yamamoto, Shingo; Hirota, Seiichi

    2012-02-15

    Primary carcinoid tumor of the urinary bladder is a very rare neoplasm. We report here a case of primary carcinoid tumor of the urinary bladder with an unusual cytological feature in a 72-year-old Japanese man. A bladder polypoid mass was incidentally found by ultrasonography during the follow-up of a benign prostate hyperplasia. Histological examination of the transurethrally resected tissue revealed that the upper part of the mass was a tumor showing tubuloglandular anastomosing structures. Most of the tumor cells had peculiar subnuclear eosinophilic granules. The features of the granules were reminiscent of those observed in neuroendocrine cells of the intestine. The tumor cells were immunohistochemically positive for chromogranin A and synaptophysin. The tumor was diagnosed as carcinoid tumor of pure form of the urinary bladder. The lower part of the mass showed the findings of glandular cystitis, as its coexistence with carcinoid tumors of the bladder has often been described in previous reports.

  13. Concurrent small bowel adenocarcinoma and carcinoid tumor in Crohn's disease--case report and literature review.

    PubMed

    Boltin, Doron; Levi, Zohar; Halpern, Marisa; Fraser, Gerald M

    2011-10-01

    Adenocarcinomas of the large and small bowel, as well as intestinal carcinoid tumors have been reported at increased rates in Crohn's disease. We herein report a rare case of concurrent adenocarcinoma and carcinoid tumor of the small bowel presenting as intestinal obstruction and found incidentally at laparotomy in a 55 year old male with longstanding ileal Crohn's disease. We performed a Medline Pubmed search for cases of synchronous or composite adenocarcinoma and carcinoid tumor in the setting of Crohn's disease and identified four similar cases. Concurrent adenocarcinoma and carcinoid tumor occurred both in newly diagnosed and longstanding Crohn's disease, most commonly involved the terminal ileum and presented with symptoms mimicking Crohn's disease. Diagnosis was made incidentally at laparotomy in all cases. Lymph node involvement was variable. Clinicians should be aware of this rare entity for expeditious surgical intervention.

  14. Cytohistology of papillary carcinoid and emerging concept of pulmonary neuroendocrine neoplasms.

    PubMed

    Yang, Grace C H

    2016-01-01

    This timely review starts by reporting the clinical, cytologic and histologic features of a morphologic variant of pulmonary carcinoid tumor forming exclusively of papillae. This growth pattern is so rare that it was not included in 2014 WHO classification of pulmonary neuroendocrine neoplasms. The current concept is reviewed, and example of spindle cell carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma, and small cell carcinoma are illustrated with fine needle aspiration cytology, surgical and clinical follow-up. Finally, the new findings in cell biology and molecular biology that led to the emerging concept that carcinoids and high-grade neuroendocrine lung carcinomas are separate biological entities are reviewed and summarized in a tumorigenic module.

  15. [Renal carcinoid tumor presenting as bladder tamponade: a case report and review of the Japanese cases].

    PubMed

    Kajita, Yoichiro; Megumi, Yuzuru; Okabe, Tatsushiro

    2005-07-01

    A 65-year-old man presented with sudden onset of gross hematuria and urinary retention. Computed tomographic scan (CT) showed a cystic multilocular enhancing lesion (9 cm in diameter) at the left renal hilum causing thinning and lateral displacement of the left renal parenchyma. Left hydronephrosis and a renal calculi were observed. We performed radical nephrectomy suspecting a cystic renal cell carcinoma. Microscopic examination and immunohistochemical studies confirmed the diagnosis of the carcinoid tumor. The tumor cells were fully positive for neuron-specific enolase and keratin, and partially positive for chromogranin-A. One of the resected lymph nodes was positive for metastasis. Additional gastrointestinal tract examinations for carcinoid tumor were negative. However, he was concurrently diagnosed with poorly differentiated prostate cancer and hormonal therapy was started. He is free of recurrent carcinoid tumor nine months postoperatively. This case is the 31st report of renal carcinoid tumors in Japan.

  16. A carcinoid tumor of the ampulla of Vater treated by endoscopic snare papillectomy.

    PubMed

    Pyun, Dae-Keun; Moon, Gyoo; Han, Jimin; Kim, Myung-Hwan; Lee, Sang Soo; Seo, Dong-Wan; Lee, Sung-Koo

    2004-12-01

    Here, a case of a patient with incidental finding of a carcinoid tumor of the ampulla of Vater, who was treated with endoscopic snare papillectomy, is reported. A 62-year-old male was admitted to our hospital due to a carcinoid tumor of the ampulla of Vater, which was found during follow-up endoscopy after an endoscopic mucosal resection of early gastric cancer. No lymphadenopathy or visceral metastasis was found on an abdominal CT scan, In-111 octerotide scan and EUS. The ampulla was then en bloc removed by endoscopic snare papillectomy. The resected specimen revealed a 0.7 x 0.5 x 0.1 cm sized carcinoid tumor. All margins of resection were negative for tumor. After six months of follow-up, there was no evidence of recurrence and metastasis, either endoscopically or radiologically. To our knowledge, this case is the first report of an ampullary carcinoid tumor treated by endoscopic snare papillectomy in Korea.

  17. Primary carcinoid tumor of medulla spinalis: case report and review of the literature.

    PubMed

    Zhang, Xian-feng; Zhang, Yan; Yan, Xu; Bie, Li

    2014-12-19

    Carcinoid tumors are slow growing neuroendocrine tumors which can originate from various sites within the body. A carcinoid tumor originating in the medulla spinalis has not previously been reported in the literature. We report a case of a 33-year-old man, presenting with a five-month history of bilateral lower extremity pain, as well as paresthesia, and mild weakness in one lateral lower extremity. A lumbar laminectomy of L3 to L5 and en bloc resection of the tumor was performed. Postoperative histopathology and immunohistochemical analysis of the tumor were consistent with that of a carcinoid tumor. There were no clinical or radiological signs of tumor recurrence or metastasis at the patient's two year postoperative follow-up. During the differential diagnosis of medulla spinalis tumors, the possibility of a primary carcinoid tumor originating within the medulla spinalis should be considered. An accurate tumor classification is imperative to ensure that the most effective course of treatment is pursued.

  18. Treatment of solitary gastric carcinoid tumor by endoscopic polypectomy in a patient with pernicious anemia

    PubMed Central

    Kadikoylu, Gurhan; Yavasoglu, Irfan; Yukselen, Vahit; Ozkara, Esra; Bolaman, Zahit

    2006-01-01

    Type I gastric carcinoid tumors result from hypergastrinemia in 1%-7% of patients with pernicious anemia. We diagnosed pernicious anemia in a 48-year-old female patient with complaint of fatigue for three months. She had no gastrointestinal symptoms. Endoscopic examination ot the upper gastrointestinal tract revealed atrophic gastritis and a polypoid lesion in the corpus of 3-4 mm in size. Endoscopic polypectomy was performed. Histopathological examination of the specimen revealed positive chromogranin A and synaptophysin stainings compatible with the diagnosis of a carcinoid tumor. Serum gastrin level was increased, urinary 5-hydroxyindoleacetic acid was within the normal range. There was no other symptom, sign, or laboratory finding of a carcinoid syndrome in the patient. No metastasis was found with indium-111 octreotide scan, computed tomographies of abdomen and thorax. Type I gastric carcinoid tumors are only rarely solitary and patients with tumors < 1 cm in size may benefit from endoscopic polypectomy. PMID:16830392

  19. Medial hypertrophy of the ovarian vein: a novel type of vascular pathology associated with a primary ovarian carcinoid tumor.

    PubMed

    Dessauvagie, Benjamin F; Lai, Patrick H; Oost, Ebo; Thomas, Anitha; Stewart, Colin J R

    2015-01-01

    Primary carcinoid tumors of the ovary are rare accounting for only 1% of neoplasms that are associated with the carcinoid syndrome. However, the carcinoid syndrome can occur in the absence of hepatic metastases due to the release of vasoactive peptides directly into the systemic circulation via the ovarian vein. We present a 69-yr-old woman presenting with carcinoid valvular disease and congestive cardiac failure who was found to have a primary left ovarian carcinoid tumor. At operation it was noted that the left ovarian vein had an unusually firm and thickened appearance, and histologic examination revealed marked fibromuscular medial hypertrophy with luminal compression. There was no associated vascular elastosis. This ovarian venous alteration appears to represent a novel addition to the spectrum of cardiovascular injuries associated with carcinoid tumors.

  20. Skeletal metastasis of carcinoid tumors: Two case reports and review of the literature.

    PubMed

    Hori, Takeshi; Yasuda, Taketoshi; Suzuki, Kayo; Kanamori, Masahiko; Kimura, Tomoatsu

    2012-05-01

    Carcinoid tumors are neuroendocrine neoplasms derived from enterochromaffin cells. Skeletal metastases from carcinoid tumors are considered to be extremely rare. In this study, we present two cases of carcinoid tumors that metastasized to the bone. Furthermore, we review 50 published case reports and reveal the features of skeletal metastasis of carcinoid tumors. The first case involved a 59-year-old man with a history of multiple metastases of a lung carcinoid tumor. The patient complained of back pain and numbness in the lower limbs. Magnetic resonance imaging revealed metastases in the thoracic spine. A spinal decompression was performed and the symptoms were resolved. The second case involved a 74-year-old man had been diagnosed with a lung carcinoid tumor 15 years previously and complained of left thigh pain. A radiograph showed osteolytic lesions in the shaft of the left femur. We repaired the femur using an intramedullary nail following curettage of the tumor. A radiograph of the femur revealed a callus on the pathological fracture. The patient was able to walk using a crutch 3 months after surgery. We reviewed 50 cases that described skeletal metastasis from carcinoid tumors. The average age of the patients was 54.9 years and 33 patients (66%) were male. The most common site of skeletal metastasis was the spine. We also investigated the survival rate of patients who developed skeletal metastasis from carcinoid tumors. The findings showed that survival of patients who developed osteolytic skeletal metastasis was worse than that of patients who developed osteosclerotic skeletal metastasis.

  1. Resection of Carcinoid Tumor Metastatic to the Right Ventricle and Tricuspid Valve.

    PubMed

    Williams, Adam R; Wang, Andrew; Kim, Han; Milano, Carmelo A

    2015-12-01

    Carcinoid tumors rarely metastasize to the heart, and previous reports involved the myocardium only. We present a case of carcinoid tumor metastatic to the right ventricle (RV) and tricuspid valve. Preoperative cardiac magnetic resonance imaging (CMR) showed the tumor arising from the right ventricular free wall and involving the tricuspid valve apparatus. The tumor was resected through a right atriotomy and required tricuspid valve replacement.

  2. Increased accumulation of N-isopropyl-(I-123)p-iodoamphetamine in bronchial carcinoid tumor

    SciTech Connect

    Nishizawa, S.; Higa, T.; Kuroda, Y.; Sano, A.; Murakami, M.; Takahashi, Y. )

    1990-02-01

    Radionuclide imaging with N-isopropyl-(I-123)p-iodoamphetamine (({sup 123}I)IMP) was performed in a patient with recurrent bronchial carcinoid tumor. Increased accumulation of ({sup 123}I)IMP was observed in the known lesions of the brain and neck on both tomographic and planar images. Scintigraphy also revealed unknown metastatic lesions of the paraaortic lymph nodes, which were later confirmed on x-ray CT. Iodine-123-IMP may have a potential role in evaluating carcinoid tumors.

  3. Scintigraphic detection of carcinoid tumors with a cost effectiveness analysis

    PubMed Central

    Dimitroulopoulos, Dimitris; Xynopoulos, Dimitris; Tsamakidis, Klisthenis; Paraskevas, Emmanouel; Zisimopoulos, Athanassios; Andriotis, Efthymios; Fotopoulou, Ekaterini; Kontis, Marios; Paraskevas, Ioannis

    2004-01-01

    AIM: To evaluate the diagnostic sensitivity and accuracy and the cost-effectiveness of this technique in the detection of gastroenteropancreatic carcinoid tumors and their metastases in comparison with conventional imaging methods. METHODS: Somatostatin receptor scintigraphy (SRS) was performed in 24 patients with confirmed carcinoids and 7 under investigation. The results were compared with those of conventional imaging methods (chest X-ray, upper abdominal ultrasound, chest CT, upper and lower abdominal CT). Also a cost-effectiveness analysis was performed comparing the cost in Euro of several combinations of SRS with conventional imaging modalities. RESULTS: SRS visualized primary or metastatic sites in 71.0% of cases and 61.3% of conventional imagings. The diagnostic sensitivity of the method was higher in patients with suspected lesions (85.7% vs 57.1%). SRS was less sensitive in the detection of metastatic sites (78.9% vs 84.2%). The undetectable lesions by SRS metastatic sites were all in the liver. Between several imaging combinations, the combinations of chest X-ray/upper abdominal CT/SRS and chest CT/upper abdominal CT/SRS showed the highest sensitivity (88.75%) in terms of the number of detected lesions. The combinations of chest X-ray/upper abdominal US/SRS and chest CT/upper abdominal ultrasound/SRS yielded also a quite similar sensitivity (82%). Compared to the cost of the four sensitive combinations the combination of chest X-ray/upper abdominal ultrasound/SRS presented the lower cost, 1183.99 Euro vs 1251.75 Euro for chest CT/upper abdominal ultrasound/SRS, 1294.93 Euro for chest X/ray/upper abdominal CT/SRS and 1362.75 Euro for chest CT/upper abdominal CT/SRS. CONCLUSION: SRS imaging is a very sensitive method for the detection of gastroenteropancreatic carcinoids but is less sensitive than ultrasound and CT in the detection of liver metastases. Between several imaging combinations, the combination of chest X-ray/upper abdominal CT/SRS shows the

  4. [Multidetector computed tomography assessment of the degree of differentiation of bronchial carcinoid tumors].

    PubMed

    Bueno Palomino, A; Zurera Tendero, L; Espejo Herrero, J J; Espejo Pérez, S; Villar Pastor, C

    2013-01-01

    To define the CT findings for bronchial carcinoid tumors and to determine whether these findings enable these tumors to be classified as typical or atypical. We reviewed the chest CT studies performed between 1 January 2001 and 31 December 2009 in patients at our hospital diagnosed with bronchial carcinoid tumors. The sample consisted of 52 patients (23 women and 29 men) with a mean age of 47 years (range 11-77 years). The 52 cases were classified as typical or atypical on the basis of the following radiological findings: size, location, focality, type of growth, calcifications, signs secondary to bronchial obstruction, and the presence of significant lymph node enlargement or metastases. These findings were then compared with the histological findings. Typical carcinoid tumors were the most prevalent (46 cases). The variables associated with atypical tumors were: male sex, advanced age at onset, and size >3cm. The accuracy of CT in classifying atypical tumors correctly increased with the number of variables indicative of atypical carcinoid tumors. A negative result for atypical nature made it possible to rule out an atypical carcinoid tumor in 95% of the cases. CT is useful for defining and characterizing carcinoid tumors into typical or atypical, although a precise formula for differentiating between the two types remains to be defined. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  5. Aprepitant for the management of refractory emesis in a patient with a small bowel carcinoid tumor.

    PubMed

    Kyeremateng, Sam; Boland, Jason W

    2014-06-01

    Nausea and vomiting are common distressing symptoms with multiple etiologies. Serotonin and substance P can induce nausea and vomiting by binding to specific receptors (5-hydroxytryptamine3 [5HT3] and neurokinin-1 [NK-1] receptors respectively). Carcinoid tumors, which originate from enterochromaffin cells of the neuroendocrine system, secrete several biologically active amines and peptides, including serotonin and substance P, that are responsible for the distant effects of this tumor. The authors present an 88-year-old lady with metastatic carcinoid tumor, with evidence of carcinoid syndrome. She had nausea and vomiting that became unresponsive to 5HT3 receptor antagonists and other antiemetics. As substance P is released from carcinoid tumors and has a role in the pathogenesis of emesis, the NK-1 receptor antagonist aprepitant was trialed. This provided complete and sustained improvement of the nausea and vomiting until her death 2 months later. This case demonstrates the potential role and rationale of NK-1 receptor antagonists in the management of resistant emesis in patients with carcinoid tumors. Clinical trials are needed to evaluate the efficacy and toxicity of these drugs in the management of emesis in patients with carcinoid syndrome.

  6. Primary neuroendocrine tumor (carcinoid tumor) of the testis: a case report with review of literature.

    PubMed

    Lubana, Sandeep Singh; Singh, Navdeep; Chan, Hon Cheung; Heimann, David

    2015-05-31

    The term carcinoid (Karzinoide) was coined by German pathologist Oberndorfer in 1907. Primary testicular carcinoid tumors (TCT) are rare, constituting 0.23% of all testicular tumors. In this report we describe a case of primary TCT of the testis and present the results of an extensive literature review to cover all the aspects of carcinoid tumor, including the definition, classification, origin, presentation, diagnostic evaluation, management, prognosis, and follow-up. A 34-year-old male presented with chronic right scrotal swelling with recent onset of pain. Radical orchiectomy revealed a solid intratesticular tumor confined to the testis and epididymis, without lymphovascular invasion. Histology was consistent with neuroendocrine carcinoma. The tumor was staged as pT1 N0 M0 S2. Immunohistochemistry was positive for neuroendocrine markers. An extratesticular carcinoid tumor was ruled out. Urinary excretion of 5-hydroxyindoleacetic acid and Chromogranin A were within normal range. It is important to follow serotonin levels since the elevated levels of serotonin can cause carcinoid heart disease. If metastatic lesions are not accessible for resection, a trial of octreotide therapy can be given. This case also adds to the rare reports in the literature of primary carcinoid tumors of the testis having low malignant potential. The literature review highlights new diagnostic and therapeutic interventions and stresses the importance of long-term follow-up due to evidence of delayed metastasis or recurrences and also due to emergence of new complications as a result of improved prognosis and prolonged survival.

  7. Partial abdominal evisceration and intestinal autotransplantation to resect a mesenteric carcinoid tumor

    PubMed Central

    2011-01-01

    Background Midgut carcinoids are neuroendocrine tumors that commonly metastasize to the intestinal mesentery, where they predispose to intestinal obstruction, ischemia and/or congestion. Because of their location, many mesenteric carcinoid tumors are deemed unresectable due to the risk of uncontrollable bleeding and prolonged intestinal ischemia. Case Presentation We report the case of a 60-year-old male with a mesenteric carcinoid tumor obstructing his superior mesenteric vein, resulting in intestinal varices and severe recurrent GI bleeds. While his tumor was thought to be unresectable by conventional techniques, it was successfully resected using intestinal autotransplantation to safely gain access to the tumor. This case is the first described application of this technique to carcinoid tumors. Conclusions Intestinal autotransplantation can be utilized to safely resect mesenteric carcinoid tumors from patients who were not previously thought to be surgical candidates. We review the literature concerning both carcinoid metastases to the intestinal mesentery and the use of intestinal autotransplantation to treat lesions involving the mesenteric root. PMID:21281518

  8. VEGFR-2 expression in carcinoid cancer cells and its role in tumor growth and metastasis

    PubMed Central

    Silva, Scott R.; Bowen, Kanika A.; Rychahou, Piotr G.; Jackson, Lindsey N.; Weiss, Heidi L.; Lee, Eun Y.; Townsend, Courtney M.; Evers, B. Mark

    2014-01-01

    Carcinoid tumors are slow growing and highly vascular neuroendocrine neoplasms that are increasing in incidence. Previously, we showed that carcinoid tumors express vascular endothelial growth factor receptor 2 (VEGFR-2) in the epithelial compartment of carcinoid tumor sections; yet, its role is not completely understood. The purpose of our study was to: (i) assess the expression of VEGFR-2 in the novel human carcinoid cell line BON, (ii) to determine the role of PI3K/Akt signaling on VEGFR-2 expression and (iii) to assess the effect of VEGFR-2 on BON cell invasion, migration and proliferation. We found that, although VEGFR-2 is expressed in BON cells, reduction in VEGFR-2 expression actually enhanced proliferation, invasion, and migration of the BON cell line. Also, expression of VEGFR-2 was inversely related to PI3K signaling. Carcinoid liver metastases in mice demonstrated decreased VEGFR-2 expression. Furthermore, the expression of a truncated, soluble form of VEGFR-2 (sVEGFR-2), a protein demonstrated to inhibit cell growth, was detected in BON cells. The presence of VEGFR-2 in the epithelial component of carcinoid tumors and in the BON cell line suggests an alternate role for VEGFR-2, in addition to its well-defined role in angiogenesis. The expression of sVEGFR-2 may explain the inverse relationship between VEGFR-2 expression and PI3K/Akt signaling and the inhibitory effect VEGFR-2 has on BON cell proliferation, migration and invasion. PMID:20473929

  9. Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

    PubMed Central

    2010-01-01

    Background Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature. Case presentation A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes. Conclusion Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause. PMID:20149263

  10. Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain.

    PubMed

    Kim, Yong Gil; Kim, Tae Nyeun; Kim, Kyeong Ok

    2010-02-12

    Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature. A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes. Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.

  11. A pilot randomized control study to evaluate endoscopic resection using a ligation device for rectal carcinoid tumors

    PubMed Central

    Sakata, Hiroyuki; Iwakiri, Ryuichi; Ootani, Akifumi; Tsunada, Seiji; Ogata, Shinichi; Ootani, Hibiki; Shimoda, Ryo; Yamaguchi, Kanako; Sakata, Yasuhisa; Amemori, Sadahiro; Mannen, Kotaro; Mizuguchi, Masanobu; Fujimoto, Kazuma

    2006-01-01

    AIM: Rectal carcinoid tumors smaller than 10 mm can be resected with local excision using endoscopy. In order to remove rectal carcinoid tumors completely, we evaluated endoscopic mucosal resection with a ligation device in this pilot control randomized study. METHODS: Fifteen patients were diagnosed with rectal carcinoid tumor (less than 10 mm) in our hospital from 1993 to 2002. There were 9 males and 6 females, with a mean age 61.5 years (range, 34-77 years). The patients had no complaints of carcinoid syndrome symptoms. Fifteen patients were randomly divided into 2 groups: 7 carcinoid tumors were treated by conventional endoscopic resection, and 8 carcinoid tumors were treated by endoscopic resection using a ligation device. RESULTS: All rectal carcinoid tumors were located at the middle to distal rectum. The size of the tumors varied from 3 mm to 10 mm and background characteristics of the patients were not different in the two groups. The rate of complete removal of carcinoid tumors using a ligation device (100%, 8/8) was significantly higher than that of conventional endoscopic resection (57.1%, 4/7). The three patients had tumor involvement of deep margin, for which additional treatment was performed. No complications occurred during or after endoscopic resection using a ligation device. All patients in the both groups were alive during the 3-year observation period. CONCLUSION: Endoscopic resection using a ligation device is a useful and safe method for resection of small rectal carcinoid tumors. PMID:16810752

  12. Lanreotide Depot: An Antineoplastic Treatment of Carcinoid or Neuroendocrine Tumors.

    PubMed

    Wolin, Edward M; Manon, Amandine; Chassaing, Christophe; Lewis, Andy; Bertocchi, Laurent; Richard, Joel; Phan, Alexandria T

    2016-12-01

    Peptide drugs for antineoplastic therapies usually have low oral bioavailability and short in vivo half-lives, requiring less preferred delivery methods. Lanreotide depot is a sustained-release somatostatin analog (SSA) formulation produced via an innovative peptide self-assembly method. Lanreotide is approved in the USA and Europe to improve progression-free survival (PFS) in patients with unresectable gastroenteropancreatic neuroendocrine tumors (GEP-NETs) and also approved in Europe for symptom control in carcinoid syndrome associated with GEP-NETs. This review discusses how the distinct molecule and formulation of lanreotide depot provide advantages to patients and health care providers, as well as the most recent clinical evidence demonstrating the safety and efficacy of lanreotide depot in inhibiting tumor growth and controlling hormonal symptoms in GEP-NETs. The lanreotide depot formulation confers a remarkable pharmacokinetic profile with no excipients, comprised only of lanreotide acetate and water. Of note, lanreotide depot constitutes an example for peptide self-assembly based formulations, providing insights that could help future development of sustained-release formulations of other antineoplastic peptides. Most patients with GEP-NETs will present with inoperable or incurable disease; thus, medical management for symptoms and tumor control plays a crucial role. Recent long-term clinical studies have demonstrated that lanreotide depot is well tolerated, prolongs PFS in GEP-NET patients, and significantly reduces symptoms related to carcinoid syndrome. The unique depot formulation and delivery method of lanreotide confer advantages in the treatment of metastatic GEP-NETs, contributing to improvements in NET-related symptoms and PFS without reducing quality of life in this patient population.

  13. Small duodenal carcinoids: a case series comparing endoscopic resection and autoamputation with band ligation.

    PubMed

    Scherer, John R; Holinga, Julie; Sanders, Michael; Chennat, Jennifer; Khalid, Asif; Fasanella, Kenneth; Singhi, Aatur D; McGrath, Kevin

    2015-04-01

    We sought to compare the efficacy and safety of endoscopic ultrasound-guided endoscopic resection (ER) and endoscopic band ligation (EBL) for autoamputation of small duodenal carcinoids. The ideal management of small duodenal carcinoid tumors remains unclear. A retrospective review of duodenal carcinoids over a 10-year period (2002 to 2012) was performed at our tertiary-care teaching hospital. All patients with duodenal carcinoids ≤10 mm in size treated with either ER or EBL were included. The main outcome measurements were the efficacy and safety of endotherapy. A total of 37 patients with 39 subcentimeter duodenal carcinoids were identified. In the EBL group, the mean (SD) tumor size was 6.7±2.1 mm compared with 6.7±1.7 mm in the ER group (P=0.943). The mean Ki-67 index was ≤2% in specimens available for histologic analysis in both groups (16/23 EBL and 15/16 ER). The positive deep margin rate in the ER group was 68.8%. Residual carcinoid tumor cells were detected on follow-up biopsies in 1 patient after EBL, and 2 patients after ER. All underwent subsequent successful endotherapy. No adverse events occurred in the EBL group compared with an 18.8% adverse event rate in the ER group (P=0.066). Endoscopic ultrasound-guided EBL is a safe, effective method for removal of small superficial duodenal carcinoids and seems to be a lower risk alternative to conventional ER with cautery.

  14. Conservation of the Notch1 signaling pathway in gastrointestinal carcinoid cells.

    PubMed

    Kunnimalaiyaan, Muthusamy; Traeger, Kelly; Chen, Herbert

    2005-10-01

    Gastrointestinal (GI) carcinoid cells secrete multiple neuroendocrine (NE) markers and hormones including 5-hydroxytryptamine and chromogranin A. We were interested in determining whether activation of the Notch1 signal transduction pathway in carcinoid cells could modulate production of NE markers and hormones. Human pancreatic carcinoid cells (BON cells) were stably transduced with an estrogen-inducible Notch1 construct, creating BON-NIER cells. In the present study, we found that Notch1 is not detectable in human GI carcinoid tumor cells. The induction of Notch1 in human BON carcinoid cells led to high levels of functional Notch1, as measured by CBF-1 binding studies, resulting in activation of the Notch1 pathway. Similar to its developmental role in the GI tract, Notch1 pathway activation led to an increase in hairy enhancer of split 1 (HES-1) protein and a concomitant silencing of human Notch1/HES-1/achaete-scute homolog 1. Furthermore, Notch1 activation led to a significant reduction in NE markers. Most interestingly, activation of the Notch1 pathway caused a significant reduction in 5-hydroxytryptamine, an important bioactive hormone in carcinoid syndrome. In addition, persistent activation of the Notch1 pathway in BON cells led to a notable reduction in cellular proliferation. These results demonstrate that the Notch1 pathway, which plays a critical role in the differentiation of enteroendocrine cells, is highly conserved in the gut. Therefore, manipulation of the Notch1 signaling pathway may be useful for expanding the targets for therapeutic and palliative treatment of patients with carcinoid tumors.

  15. The PARP inhibitor ABT-888 potentiates darbazine-induced cell death in carcinoids

    PubMed Central

    Somnay, Yash; Lubner, Sam; Gill, Harpreet; Matsumura, Jon Blake; Chen, Herbert

    2016-01-01

    Monoagent DNA-alkylating chemotherapies like dacarbazine are among a paucity of medical treatments for advanced carcinoid tumors, but are limited by host toxicity and intrinsic chemoresistance through the base excision repair (BER) pathway via poly (ADP-ribose) polymerase (PARP). Hence, inhibitors of PARP may potentiate DNA-damaging agents by blocking BER and DNA restoration. We show that the PARP inhibitor ABT-888 (Veliparib) enhances the cytotoxic effects of dacarbazine in carcinoids. Two human carcinoid cell lines (BON and H727) treated with a combination of ABT-888 and dacarbazine resulted in synergistic growth inhibition signified by combination indices <1 on the Chou-Talalay scale. ABT-888 administered prior to varying dacarbazine doses promoted the suppression of neuroendocrine biomarkers of malignancy ASCL1 and CgA, shown by Western analysis. ATM phosphorylation and p21Waf1/Cip1 activation, indicative of DNA damage, were increased by ABT-888 when combined with dacarbazine treatment, suggesting BER pathway attenuation by ABT-888. PE Annexin V/7-AAD staining and sorting revealed a profound induction of apoptosis following combination treatment, which was further confirmed by increased PARP cleavage. These results demonstrate that ABT-888 synergizes dacarbazine treatment in carcinoids. Therefore, ABT-888 may help treat carcinoids unresponsive or refractory to mainstay therapies. PMID:27632933

  16. Distinct patterns of chronic gastritis associated with carcinoid and cancer and their role in tumorigenesis.

    PubMed Central

    Solcia, E.; Rindi, G.; Fiocca, R.; Villani, L.; Buffa, R.; Ambrosiani, L.; Capella, C.

    1992-01-01

    A series of 60 gastric endocrine tumors comprised 44 body-fundus argyrophil carcinoids, of which 23 arose in a background of hypergastrinemia and type A chronic atrophic gastritis (A-CAG), mainly with histologic patterns suggestive of an autoimmune process. Only 22 percent (compared with 19 percent of 58 tumor-free A-CAG cases) of 36 carcinoids and 21 percent of 19 A-CAG carcinoids investigated had Helicobacter pylori (HP) colonization, against 50 percent of 14 CAG-associated neuroendocrine carcinomas or mixed endocrine-exocrine tumors, 84 percent of 150 cases with early gastric cancer (p < 0.001 versus carcinoids), mostly with B- or AB-type CAG, 76 percent of 97 tumor-free AB-CAG, and 95 percent of 151 tumor-free B-CAG cases. Secondary hypergastrinemia and local mechanisms activated by chronic autoimmune gastritis are among factors involved in the pathogenesis of relatively indolent CAG-associated carcinoids, whereas active HP gastritis in cooperation with environmental carcinogens may likely cause more severe epithelial transformation, leading to ordinary cancer and, possibly, to neuroendocrine carcinomas or mixed endocrine-exocrine tumors. Images FIG. 1 FIG. 2 FIG. 3 PMID:1341079

  17. Intradural Carcinoid Tumor Found in a Patient with No History of Cancer.

    PubMed

    Audlin, Jason R; Khullar, Gaurav; Deshaies, Eric M; Kurra, Swamy; Lavelle, William F

    2016-12-01

    Carcinoid tumors are rare neoplasms that often arise from the gastrointestinal or respiratory tracts. They often metastasize to bone tissue and pancreatic and hepatic sites. The central nervous system and most specifically the spinal cord are rarely involved. Primary carcinoid tumors of the central nervous system are even rarer. A 58-year-old man presented with progressive bilateral lower extremity weakness and a negative history of cancer. Imaging revealed an intradural mass at the L1 and L2 spinal levels. Surgical resection of the tumor via laminectomy was performed. The tumor was observed to have eroded through the dura and was compressing the spinal nerves. Histopathologically, the mass was observed to be of carcinoid origin. A subsequent octreoscan revealed no primary sites of carcinoid tumor. Postoperatively, the patient was followed for 1.5 years. The patient reported improvement in sensation to his lower extremities with no change in motor findings. Carcinoid tumors of the central nervous system are extremely rare, but they should remain in the differential diagnosis for patients experiencing extremity weakness and back or neck pain with an intradural mass and no primary source of the tumor identified or other manifestations of a primary tumor. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Comparison of Endoscopic Mucosal Resection With Circumferential Incision and Endoscopic Submucosal Dissection for Rectal Carcinoid Tumor.

    PubMed

    Chen, Ru; Liu, Xiang; Sun, Siyu; Wang, Sheng; Ge, Nan; Wang, Guoxin; Guo, Jintao

    2016-06-01

    Endoscopic submucosal dissection (ESD) has been a valuable treatment of choice for rectal carcinoid tumors that are endoscopically treatable because of its satisfactory resection rate and low recurrence rate. For treatment of small rectal carcinoids, endoscopic mucosal resection (EMR) is more often chosen by endoscopists because of its safety and time efficiency. We applied circumferential incision and endoscopic mucosal resection (CI-EMR), which is a modification of EMR, to treat rectal carcinoid tumors and compared their efficacy and safety. Between January 2008 and December 2013, we enrolled 66 patients (30 in the ESD group and 36 in the CI-EMR group) at Shengjing Hospital who were pathologically diagnosed with rectal carcinoid tumors <15 mm in diameter. We retrospectively analyzed en bloc resection rates, pathologic complete resection rates, incidences of complications, follow-up outcomes, and procedure times. The en bloc resection rate and the histologic complete resection rate were similar, but the procedure time was longer in the ESD group than in the CI-EMR group. In cases that were incompletely resected, neither local recurrence nor distant metastasis was detected during follow-up. Similarly minor bleeding occurred in both groups, and no perforation occurred after either procedure. CI-EMR showed comparable en bloc resection and histologically complete resection rates and is technically simpler and minimally invasive compared with ESD. Because of its easier performance and shorter procedure time, CI-EMR may be preferable to ESD for resection of rectal carcinoid tumors <15 mm in diameter without invasion or distant metastases.

  19. Primary carcinoid tumor of the gallbladder: a case report and brief review of the literature.

    PubMed

    Zou, Yi-Ping; Li, Wei-Min; Liu, Hao-Run; Li, Ning

    2010-02-23

    Primary carcinoid tumor of the gallbladder is rare and comprises less than 1% of all carcinoid tumors. Preoperative diagnosis of carcinoid tumor of the gallbladder is difficult. The imageology findings are similar to those in other gallbladder cancers. A 46-year-old woman was hospitalized with a preoperative diagnosis of gallbladder carcinoma, The patient was referred for surgical opinion and laparotomy was subsequently performed. A 4 x 5 cm mass was found within the gallbladder, located on the free surface of the body and fundus of the gallbladder. Neither metastases nor direct invasion to the liver was found. The entire mass and gallbladder were excised and intact. Histologically, the tumor consisted of small oval cells with round-to-oval neclei and tumor cells formed small nodular, trabeculare and acinar structures. The tumor showed moderate pleomorphism with scattered mitotic figures, but no definite evidence of vascular permeation, perineural invasion or lymphatic permeation was seen. The tumor cells invaded the mucosa extensively, and some penetrated the muscular layer but not through the serosa of the gallbladder into the liver. Immunohistochemical studies revealed strong positive reaction for chromogranin A and NSE. This lesion was proved to be a primary carcinoid tumor of the gallbladder. A brief review of literature, clinical feature, pathology and treatment of this rare disease was discussed. Primary carcinoid tumor of the gallbladder is uncommon. The definite diagnosis is often made on histopathological results after surgery.

  20. Management of sub-5 mm rectal carcinoids with lymph node metastases

    PubMed Central

    Toh, James Wei Tatt; Henderson, Christopher; Yabe, Takako Eva; Ong, Evonne; Chapuis, Pierre; Bokey, Les

    2015-01-01

    Minute (<5 mm) and small (5–10 mm) rectal carcinoids discovered during colonoscopy are generally considered to be non-aggressive, and the management and surveillance of patients with this entity are usually limited. We present the case of a 61-year-old Chinese female with multiple sub-5 mm carcinoid tumours in the rectum without any computed tomography (CT) evidence of lymph node or distant metastases. She underwent an ultra-low anterior resection for a sessile rectal polyp with the histological appearance of a moderately differentiated adenocarcinoma. Seven foci of minute carcinoids in the rectum and perirectal lymph node metastastic spread from the carcinoid tumours were also discovered on histopathology. There were no lymph node metastases originating from adenocarcinoma. This case report and review of the literature suggests that minute rectal carcinoids are at risk of metastasizing and that these patients should be investigated for lymph node and distant metastatic spread with CT and somatostatin receptor scintigraphy or its equivalent, as this would influence prognosis and surgical management of these patients. Findings relating to lymphovascular invasion, perineural invasion, high Ki-67, mitotic rate, depth of tumour invasion, central ulceration, multifocal tumours and size are useful in predicting metastases and may be used in scoring tools. Size alone is not a good predictor of metastastic spread. PMID:25342710

  1. Primary carcinoid tumor of the ovary arising in a mature cystic teratoma: a case report.

    PubMed

    Ting, W H; Hsiao, S M; Lin, H H; Wei, M C

    2014-01-01

    Primary ovarian carcinoid tumors are rare entities, they may appear with other teratomatous components, and can be often being mistaken as part of mature cystic teratomas. Consistent with their rarity and low incidence, imaging clues that could have led to suspicion of this tumor are not well-documented. Herein, the authors present a rare case of primary ovarian carcinoid tumor in a mature cystic teratoma, who initially presented with complaints of abdominal distension for months. Contrast-enhanced computerized tomography (CT) demonstrated a multilobular mass with different density components including fat, soft tissue, and calcification materials, as well as rich vascular supply from the right ovarian vein. Serum tumor markers were within normal limits. Bilateral salpingo-oophorectomy was performed and the pathological diagnosis was mature cystic teratoma with coexisting primary ovarian carcinoid tumor, insular type. The patient has remained well with no residual disease for over one year of follow-up.

  2. Large presacral tailgut cyst with a carcinoid tumor in a male: report of a case.

    PubMed

    Charalampakis, Vasileios; Stamatiou, Dimitrios; Christodoulakis, Manousos; Kafousi, Maria; Chryssou, Evangelia; de Bree, Eelco; Melissas, John

    2014-05-01

    Tailgut cysts are developmental hamartomas found in the presacral space. They are usually detected incidentally during physical examinations or imaging studies. However, they may cause symptoms due to compression of nearby organs. Due to their potential malignant transformation, surgical resection is warranted, while routine biopsy is considered controversial because of the concern about infection of the tailgut cyst and needle-track implantation of malignant cells. The co-existence of a carcinoid in a tailgut cyst is extremely rare. Only 16 cases have been reported previously, the vast majority of which were found in females. We herein present the case of a carcinoid in a tailgut cyst found in a male patient, discuss the potential pathogenesis of tailgut carcinoids, and underline the fact that their previous consideration of the condition as a female-restricted entity should be rejected.

  3. A case of very well-differentiated adenocarcinoma with carcinoid tumor in the ascending colon.

    PubMed

    Yamauchi, Hayato; Sakurai, Shinji; Tsukagoshi, Ritsuko; Suzuki, Masaki; Tabe, Yuichi; Fukasawa, Takaharu; Kiriyama, Shinsuke; Fukuchi, Minoru; Naitoh, Hiroshi; Kuwano, Hiroyuki

    2014-01-01

    Malignant tumors with mixed glandular and neuroendocrine characteristics with at least 30% of each component are classified as mixed adenoneuroendocrine carcinoma (MANEC) by the World Health Organization 2010 classification. We report here a case of very well-differentiated adenocarcinoma accompanied by carcinoid tumor, categorized as MANEC. A 41-year-old Japanese man was clinically diagnosed with ascending colon cancer and underwent right hemicolectomy. Using an immunohistologic technique, the pathologic diagnosis was very well-differentiated adenocarcinoma accompanied by carcinoid tumor and marked eosinophil infiltration, which was categorized as MANEC. By immunohistochemical analysis, tumor cells of the carcinoid component exhibited very low proliferation activity. Our case was thought to be MANEC without high malignant potential. MANEC as per the World Health Organization 2010 classification seems to include tumors with diverse grades of malignancy, and it might need to have subclassifications according to the malignancy potential of the tumor cells.

  4. [A case of laparoscopic surgery for a rectal carcinoid after ALTA therapy for an internal hemorrhoid].

    PubMed

    Aomatsu, Naoki; Nakamura, Masanori; Hasegawa, Tsuyoshi; Nakao, Shigetomi; Uchima, Yasutake; Aomatsu, Keiho

    2014-11-01

    We report a case of laparoscopic surgery for a rectal carcinoid after aluminum potassium and tannic acid (ALTA) therapy for an internal hemorrhoid. A 66-year-old man was admitted to our hospital because of bleeding during defecation. He was diagnosed via anoscopy with Goligher grade II internal hemorrhoids. Examination via colonoscopy revealed 2 yellowish submucosal tumors in the lower rectum that were 5mm and 10mm in diameter. A rectal carcinoid tumor was diagnosed based on histopathology. Abdominal computed tomography demonstrated no metastases to the liver or lymph nodes. First, we performed ALTA therapy for the internal hemorrhoids. Two weeks later, we performed laparoscopic-assisted low anterior resection (D2) for the rectal carcinoid. The patient was discharged without complications and has not experienced recurrence during the 2 years of follow-up care.

  5. An unusual presentation of a carcinoid tumor of the common bile duct.

    PubMed

    Jethava, Ashif; Muralidharan, Visvanathan; Mesologites, Thalia; Stoica-Mustafa, Elena; Dasanu, Constantin A

    2013-01-10

    Carcinoid tumors arising from the bile ducts account for only a small fraction of biliary tract cancers. We report herein a 42-year-old man with a carcinoid tumor of the common bile duct. He presented with abdominal pain, bloating and dyspepsia. Clinicolaboratory and imaging studies suggested a probable obstructive common bile duct lesion. The patient underwent an endoscopic retrograde cholangiopancreatography with a stent placement in view of common bile duct decompression. Persistence of symptoms prompted a laparotomy and pancreaticoduodenectomy that revealed a well-differentiated carcinoid tumor originating in the common bile duct. Clinician's familiarity with the unusual sites of origin of neuroendocrine tumors and/or atypical presentation of such tumors may facilitate their early recognition and allow for a timely intervention.

  6. Small bowel carcinoid: Location isn't everything!

    PubMed

    Hari, Danielle M; Goff, Stephanie L; Reich, Heidi J; Leung, Anna M; Sim, Myung-Shin; Lee, Ji Hey; Wolin, Edward; Amersi, Farin

    2013-08-27

    To investigate the prognostic significance of the primary site of disease for small bowel carcinoid (SBC) using a population-based analysis. The Surveillance, Epidemiology and End Results (SEER) database was queried for histologically confirmed SBC between the years 1988 and 2009. Overall survival (OS) and disease-specific survival (DSS) were analyzed using the Kaplan-Meier method and compared using Log rank testing. Log rank and multivariate Cox regression analyses were used to identify predictors of survival using age, year of diagnosis, race, gender, tumor histology/size/location, tumor-node-metastasis stage, number of lymph nodes (LNs) examined and percent of LNs with metastases. Of the 3763 patients, 51.2% were male with a mean age of 62.13 years. Median follow-up was 50 mo. The 10-year OS and DSS for duodenal primaries were significantly better when compared to jejunal and ileal primaries (P = 0.02 and < 0.0001, respectively). On multivariate Cox regression analysis, after adjusting for multiple factors, primary site location was not a significant predictor of survival (P = 0.752 for OS and P = 0.966 DSS) while age, number of primaries, number of LNs examined, T-stage and M-stage were independent predictors of survival. This 21-year, population-based study of SBC challenges the concept that location of the primary lesion alone is a significant predictor of survival.

  7. Primary Neuroendocrine Tumor (Carcinoid Tumor) of the Testis: A Case Report with Review of Literature

    PubMed Central

    Lubana, Sandeep Singh; Singh, Navdeep; Chan, Hon Cheung; Heimann, David

    2015-01-01

    Patient: Male, 34 Final Diagnosis: Primary neuroendocrine tumor of the testis Symptoms: Pain • swelling Medication: None Clinical Procedure: Radical orchiectomy Specialty: Oncology Objective: Rare disease Background: The term carcinoid (Karzinoide) was coined by German pathologist Oberndorfer in 1907. Primary testicular carcinoid tumors (TCT) are rare, constituting 0.23% of all testicular tumors. In this report we describe a case of primary TCT of the testis and present the results of an extensive literature review to cover all the aspects of carcinoid tumor, including the definition, classification, origin, presentation, diagnostic evaluation, management, prognosis, and follow-up. Case Report: A 34-year-old male presented with chronic right scrotal swelling with recent onset of pain. Radical orchiectomy revealed a solid intratesticular tumor confined to the testis and epididymis, without lymphovascular invasion. Histology was consistent with neuroendocrine carcinoma. The tumor was staged as pT1 N0 M0 S2. Immunohistochemistry was positive for neuroendocrine markers. An extratesticular carcinoid tumor was ruled out. Urinary excretion of 5-hydroxyindoleacetic acid and Chromogranin A were within normal range. Conclusions: It is important to follow serotonin levels since the elevated levels of serotonin can cause carcinoid heart disease. If metastatic lesions are not accessible for resection, a trial of octreotide therapy can be given. This case also adds to the rare reports in the literature of primary carcinoid tumors of the testis having low malignant potential. The literature review highlights new diagnostic and therapeutic interventions and stresses the importance of long-term follow-up due to evidence of delayed metastasis or recurrences and also due to emergence of new complications as a result of improved prognosis and prolonged survival. PMID:26027014

  8. Incidental Primary Intradural Carcinoid Tumor in a Patient with Lumbar Radiculopathy.

    PubMed

    Koustais, Stavros; O'Halloran, Philip J; Hassan, Alfrazdaq; Brett, Francesca; Young, Steven

    2017-09-01

    Carcinoid tumors are neuroendocrine neoplasms derived from enterochromaffin cells. Central nervous system involvement is rare. The reported cases include metastases to brain or spinal cord, and primary skeletal or extradural disease resulting in compressive myeloradiculopathy. There are 2 previously reported cases of primary intradural extramedullary carcinoid tumor, presenting with compressive symptoms. We report the first case of primary incidental intradural extramedullary carcinoid tumor. A 68-year-old man was complaining of a 2-month history of left-sided back pain and sciatica; however, he presented with acutely worse symptoms of severe left sciatica. There was no sphincter dysfunction nor power deficit on examination. A magnetic resonance imaging (MRI) scan of the lumbar spine revealed an L4-5 central disk protrusion causing left L5 nerve root compression, and a heterogeneous enhancing intradural mass at the L2-3 level displacing the cauda equina. An L4-5 microdiscectomy followed by an L2-3 laminectomy and resection of the intradural mass was performed. There was a postoperative left S1 paresthesia that was managed medically; however, the postoperative period was unremarkable. A postoperative MRI of the lumbar spine showed complete resection of the mass. A staging scan revealed an incidental thyroid nodule; however, there was no other disease burden. Histopathology of the resected specimen revealed a primary spinal carcinoid tumor. To our knowledge, this is the first reported case of incidental primary intradural spinal carcinoid tumor. Even though intradural spinal carcinoid tumors are exceedingly rare, they should be included in the differential diagnosis of enhancing intradural lesions. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Usefulness of endoscopic ultrasonography (EUS) for selecting carcinoid tumors as candidates to endoscopic resection.

    PubMed

    Varas, M J; Gornals, J B; Pons, C; Espinós, J C; Abad, R; Lorente, F J; Bargalló, D

    2010-10-01

    Carcinoid tumors (CTs) represent the most common type of neuroendocrine tumors (NETs). Digestive CTs in the gastroduodenal and colorectal tracts may be assessed using endoscopy and echoendoscopy or endoscopic ultrasonography (EUS) with the goal of attempting local resection with curative intent without having recourse to surgery. Endpoints in this study included:--Assessing the usefulness of EUS for selecting CTs as candidates to endoscopic excision. --Assessing the effectiveness of local resection (complete carcinoid resection) and the safety (complications) of the technique involved. OUr series included 18 patients (12 males and 6 females) with 23 tumors. Sixteen patients (10 males and 6 females) were selected, with age ranging from 40 to 81 years (mean: 57 years), biopsied, endoscopically treated digestive carcinoid tumors, and a previous negative extension study. Twenty-one 2-to-20-mm (mean size 8 mm) tumors were resected in 23 procedures. After endoscopy plus biopsy and echoendoscopy (EUS), excision was carried out with conventional polypectomy snare mucosectomy and submucosal injection with saline and/or adrenaline in most cases (15), and mucosectomy technique following lesion ligation with elastic bands for six cases. Two cases underwent transanal endoscopic surgery (TEM), one of them following non-curative polypectomy. A total of 23 local procedures were performed with the key goal of assessing efficacy (complete resection: CR) and safety (complications). There were no severe complications except for the last gastric mucosectomy for a 6-mm carcinoid, where a miniperforation occurred that was solved by using 3 clips (1/23: 4.3%).EUS sensitivity was 94%. Complete resection was 90.5% (19/21). The endoscopic mucosal resection of selected carcinoid tumors is a safe, effective technique. EUS is the technique of choice to select patients eligible for endoscopic resection (carcinoids smaller than 20 mm in superficial layers, with an unscathed muscularis propria and

  10. Current concepts in diagnosis and perioperative management of carcinoid heart disease.

    PubMed

    Castillo, Javier G; Silvay, George; Solís, Jorge

    2013-09-01

    Carcinoid tumors are neuroendocrine tumors with a very unpredictable clinical behavior. In the setting of hepatic metastases, the tumor's release of bioactive substances into the systemic circulation results in carcinoid syndrome: a constellation of symptoms among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most prominent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe fibrotic reaction which frequently involves the right-sided valves and may extend towards the subvalvular apparatus leading to valve thickening and retraction. Left-sided involvement is rare and mostly observed in the presence of an interatrial shunt, endobronchial tumor localization, and high tumor activity. Echocardiographic techniques often reveal noncoaptation of the valves, which are fixed in a semiopen position. In patients with advanced lesions and severe valvular dysfunction, surgery is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend towards improved surgical outcomes has triggered a more liberal referral for valve replacement. Carcinoid heart disease poses two distinct challenges for the anesthesiologist: carcinoid crisis and low cardiac output syndrome secondary to right ventricular failure. Carcinoid crisis, characterized by flushing, hypotension, and bronchospasm, may be precipitated by catecholamines and histamine releasing drugs used routinely in patients undergoing valve surgery. Although a broader utilization of octreotide have significantly simplified the anesthetic and perioperative management of these patients, a very balanced anesthetic technique is required to identify and manage low cardiac output syndrome.

  11. [Appendiceal carcinoid: report of 3 clinical cases and review of the literature].

    PubMed

    Bronzino, P; Cassinelli, G; Cuneo, A; Rassu, P C; Partipilo, F; Bianchi, M; Casaccia, M

    2003-05-01

    The Authors report three cases of appendiceal carcinoids, found during appendectomy for acute appendicitis during the last 15 years. Appendiceal carcinoid is a rare neoplasm, more frequent in females age ranging between 20 and 29 years. Lack of symptoms explains the occasional diagnosis of this disease. The prognosis is related mostly to the size of the tumor, local and lymphatic invasion, presence of metastases, localization inside the appendix, histological type and age of the patient. Therapy is mainly surgical and consists of appendectomy and right colectomy according to different criteria which are being discussed in this article.

  12. Small cell carcinoma of the colon arising in a carcinoid tumor.

    PubMed

    Saif, M Wasif

    2013-04-01

    Small cell carcinomas of the gastrointestinal tract are rare and clinically aggressive tumors. A case is presented of a 70 year-old woman who presented with small bowel obstruction and was found to have a cecal mass. She underwent right hemicolectomy, and histopathology showed a small cell carcinoma arising in the background of a carcinoid tumor. Although small cell carcinomas of the colon have frequently been found in association with colonic adenomas, this appears to be the first report of a low-grade carcinoid tumor in combination with a small cell carcinoma.

  13. Primary pure carcinoid tumour of the testis: A case report and review of the literature.

    PubMed

    Takada, Hideki; Iwatsuki, Shoichiro; Itoh, Yasunori; Sato, Shinya; Hayase, Masa; Yasui, Takahiro

    2016-10-05

    Primary testicular carcinoid tumours (TCT) are very rare, and a large tumour size and the presence of carcinoid syndrome predict a malignant course. Histologically, it is difficult to differentiate between benign and malignant TCTs. We report a case of a primary pure TCT with an unusual presentation in a 23- year-old man, who had an asymptomatic, enlarged scrotum on the right side for 7 years. On gross examination, the tumour was 9.6 cm in diameter. The Ki-67 labelling index was 19.8%. High inguinal orchidectomy was performed, and 30 months after surgery the patient remains asymptomatic.

  14. Malignant Neuroendocrine Tumour (Carcinoid) of the Spleen in an African Pygmy Hedgehog (Atelerix albiventris).

    PubMed

    Lowden, L R; Davies, J L

    2016-07-01

    A malignant neuroendocrine tumour (carcinoid) of the spleen was diagnosed on post-mortem examination of a 3-year-old, male African pygmy hedgehog (Atelerix albiventris). The animal presented with a history of inappetence, weight loss, lethargy, a wide-based gait and a palpable abdominal mass. The gross pathological, histopathological, histochemical and immunohistochemical findings are described. Primary splenic carcinoids are reported rarely in the human medical literature and this is believed to be the first report in a non-human animal. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. Giant cell arteritis and polymyalgia rheumatica as first manifestation of typical pulmonary carcinoid tumor.

    PubMed

    Aguiar, T; Vincent, M B

    2015-12-23

    Giant cell arteritis (GCA), a systemic vasculitis of unknown origin, may appear rarely as a paraneoplastic syndrome. Cases secondary to pulmonary neuroendocrine tumors have not been reported. A 75-year-old female developed prednisone-responsive GCA/polymyalgia rheumatica (PMR) shortly followed by syndrome of inappropriate antidiuretic hormone secretion. An 8 mm carcinoid lung tumor with positron emission tomography normal uptake was found. After a thoracoscopic tumor resection the patient experienced complete clinical and laboratory remission. This is the first report of GCA with PMR in the context of carcinoid lung tumor. It emphasizes the role of paraneoplastic vasculitis as a possible cause of GCA.

  16. Eltrombopag Use in Thrombocytopenia for Endoscopic Submucosal Dissection of a Gastric Carcinoid

    PubMed Central

    Kaltenbach, Tonya; Martin, Beth; Rouse, Robert V.; Soetikno, Roy

    2014-01-01

    Severe thrombocytopenia is a contraindication for therapeutic endoscopy due to the risk of bleeding. Platelet transfusions can temporarily increase platelet count, but are difficult to administer in the 2 weeks following endoscopic resection, during which the patient is at high risk for delayed bleeding. We present the use of a novel thrombopoietin receptor agonist, eltrombopag, to sustain platelet levels for the safe and complete endoscopic submucosal dissection of a gastric carcinoid in a patient with severe thrombocytopenia due to cirrhosis and idiopathic thrombocytopenic purpura. We performed complete and safe endoscopic removal of a gastric carcinoid after correcting the thrombocytopenia. PMID:26157896

  17. Chrysin suppresses the achaete-scute complex-like1 and alters the neuroendocrine phenotype of carcinoids

    PubMed Central

    Somnay, Yash R.; Dull, Barbara Zarebczan; Eide, Jacob; Jaskula-Sztul, Renata; Chen, Herbert

    2015-01-01

    Carcinoids are neuroendocrine neoplasms that cause significant morbidity and mortality, and for which few effective therapies are available. Given the recent identification of the anti-cancer flavonoid chrysin, we sought to investigate its therapeutic potential in carcinoids. Here, we report chrysin’s ability to modulate the achaete-scute complex-like1 (ASCL1), a neuroendocrine-specific transcription factor highly implicated in the malignant phenotype of carcinoids and other neuroendocrine cancers. Moreover, we elucidate the role of ASCL1 in carcinoid growth and bioactivity. Treatment of two carcinoid cell lines (BON and H727) with varying chrysin concentrations suppressed cell proliferation, while reducing expression of ASCL1 and the neuroendocrine biomarker chromogranin A (CgA), demonstrated by Western blotting. Propidium iodide and PE AnnexinV/7-AAD staining and sorting following chrysin treatment revealed S/G2 phase arrest and apoptosis, respectively. This was corroborated by chrysin-induced cleavage of caspase-3 and PARP and activation of p21Waf1/Cip1. Furthermore, direct ASCL1 knockdown with an ASCL1-specific small interfering RNA inhibited CgA and synaptophysin expression as well as carcinoid proliferation, while also reducing cyclin B1 and D1, and increasing p21Waf1/Cip1 and p27Kip1 expression, suggesting an arrest of the cell-cycle. Collectively, these findings warrant the deliberation of targeted ASCL1 suppression by chrysin or other agents as a therapeutic approach for carcinoid management. PMID:26403073

  18. An Ovarian Carcinoid Tumor With Peptide YY-Positive Insular Component: A Case Report and Review of the Literature.

    PubMed

    Erdenebaatar, Chimeddulam; Yamaguchi, Munekage; Saito, Fumitaka; Motooka, Chisato; Tashiro, Hironori; Katabuchi, Hidetaka

    2016-07-01

    Ovarian carcinoid tumors are uncommon and account for 1% of all carcinoid tumors. The insular type of ovarian carcinoid tumor is common in western countries; in contrast, the strumal and trabecular types seem to be common in Asian countries. Strumal and trabecular types are associated with peptide YY (PYY) production, which may cause constipation. Here, we report the case of a 70-yr-old Japanese woman with chronic constipation who was referred to Kumamoto University Hospital because of a right adnexal mass. Imaging tests suggested that the solid mass might be malignant; therefore, abdominal total hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were performed. A subsequent histopathologic examination confirmed an insular carcinoid tumor with a trabecular component in the right ovary. Both components were positive for PYY but not for serotonin. The patient complained of diarrhea instead of constipation soon after the surgery. Because PYY-positive insular carcinoid tumor in the ovary has not been previously reported, we reviewed 19 reported cases of patients with PYY-positive ovarian carcinoid tumors. The origins, common histologic types and symptoms caused by specific peptides secreted in ovarian carcinoid tumors differ between western and Asian countries.

  19. [Four resections of metachronous liver metastases and lateral lymph node metastases of a rectal carcinoid tumor - a case report].

    PubMed

    Nakamoto, Takayuki; Koyama, Fumikazu; Nakagawa, Tadashi; Nakamura, Shinji; Ueda, Takeshi; Nishigori, Naoto; Inoue, Takashi; Kawasaki, Keijirou; Obara, Shinsaku; Fujii, Hisao; Nakajima, Yoshiyuki

    2014-11-01

    The authors present a case of rectal carcinoid tumor with lateral lymph node metastases and liver metastases that was successfully treated by 4 resections. A 70-year-old man was diagnosed with a rectal carcinoid tumor (20 mm in diameter) with submucosal (SM) invasion. Radical resection was performed at 25 months, 38 months, and 57 months, when abdominal computed tomography (CT) revealed metachronous liver metastases of the rectal carcinoid tumor. At 50 months, metachronous lateral lymph node metastases were also revealed. Three hepatectomies and a laparoscopic lateral lymph node dissection were performed. The patient is currently free of disease at 25 months after the last intervention.

  20. Tubular carcinoids of the appendix: the CK7/CK20 immunophenotype can be a diagnostic pitfall.

    PubMed

    Matsukuma, Karen E; Montgomery, Elizabeth A

    2012-07-01

    Tubular carcinoid is a rare variant of appendiceal well-differentiated neuroendocrine tumour. Although considered benign lesions, the small infiltrating tubules that characterise the tumour may raise concern for metastatic adenocarcinoma. To our knowledge, the cytokeratin 7 (CK7)/cytokeratin 20 (CK20) expression profile of these neoplasms remains unexplored. The authors characterised the CK7/CK20 immunophenotype and Ki-67 expression of the eight available tubular carcinoids seen at their institution from 1991 to 2011. CK7 and CK20 staining was variable, ranging from none to focal staining for either or both CK7 and CK20, to diffuse expression of CK7 or CK20. The CK7/CK20 expression profile is of limited value when the differential diagnosis includes primary tubular carcinoid and well-differentiated metastatic adenocarcinoma. In such cases, careful attention to the location of the neoplasm, mitotic count and presence or absence of an associated classic carcinoid component are more useful for arriving at the correct diagnosis.

  1. Role of computed tomography angiography in detection and staging of small bowel carcinoid tumors

    PubMed Central

    Bonekamp, David; Raman, Siva P; Horton, Karen M; Fishman, Elliot K

    2015-01-01

    Small-bowel carcinoid tumors are the most common form (42%) of gastrointestinal carcinoids, which by themselves comprise 70% of neuroendocrine tumors. Although primary small bowel neoplasms are overall rare (3%-6% of all gastrointestinal neoplasms), carcinoids still represent the second most common (20%-30%) primary small-bowel malignancy after small bowel adenocarcinoma. Their imaging evaluation is often challenging. State-of-the-art high-resolution multiphasic computed tomography together with advanced postprocessing methods provides an excellent tool for their depiction. The manifold interactive parameter choices however require knowledge of when to use which technique. Here, we discuss the imaging appearance and evaluation of duodenal, jejunal and ileal carcinoid tumors, including the imaging features of the primary tumor, locoregional mesenteric nodal metastases, and distant metastatic disease. A protocol for optimal lesion detection is presented, including the use of computed tomography enterography, volume acquisition, computed tomography angiography and three-dimensional mapping. Imaging findings are illustrated with a series of challenging cases which illustrate the spectrum of possible disease in the small bowel and mesentery, the range of possible appearances in the bowel itself on multiphase data and extraluminal findings such as the desmoplastic reaction in mesentery and hypervascular liver metastases. Typical imaging pitfalls and pearls are illustrated. PMID:26435774

  2. Fourteen-year-old girl with endobronchial carcinoid tumour presenting with asthma and lobar emphysema.

    PubMed

    Andersen, Julie Bjerglund; Mortensen, Jann; Damgaard, Karen; Skov, Marianne; Sparup, Jørgen; Petersen, Bodil Laub; Rechnitzer, Catherine; Borgwardt, Lise

    2010-04-01

    Bronchial carcinoid tumours seldom occur in children, and represent a rare cause of pulmonary obstruction. Because of low clinical suspicion and the variable ways of presentation, diagnosis may be delayed. We report on a patient with this tumour. It is hoped that increased awareness of the tumour can lead to earlier diagnosis. Report of a case. This case describes a 14-year-old previously healthy girl, presenting with asthma-like symptoms throughout 2 years, decreased lung function and emphysema in left lower lobe on chest x-ray. Computerized tomography (CT) showed an intraluminal process in the left main bronchus and emphysema in both the upper and lower left lobe and showed no signs of metastasis or spread to lung tissue. Bronchoscopy showed an inflammatory polyp. Surgical resection demonstrated a typical carcinoid tumour. Later control biopsy revealed no persisting malignant tissue. The asthma symptoms returned and a new bronchoscopy showed scarring and narrowing of the left bronchus. Treatment comprised of dilatation by bronchoscopy plus daily combination corticosteroids and beta-2-agonist inhalation and the symptoms improved. No signs of relapse 16 months postdiagnosis. The case clearly shows the delay, which is common in the diagnosis of children with bronchial carcinoid tumours. Symptoms of the obstructive nature of the tumour are variable and might present as emphysema seen on x-ray and CT. Carcinoid tumour should be considered in children with longstanding pulmonary symptoms with no response to conventional treatment. Prognosis is good but long-term follow up is needed.

  3. Regression of liver metastases of occult carcinoid tumor with slow release Lanreotide therapy

    PubMed Central

    Bondanelli, Marta; Ambrosio, Maria Rosaria; Zatelli, Maria Chiara; Cavazzini, Luigi; Al Jandali Rifa’y, Laura; degli Uberti, Ettore C.

    2005-01-01

    Few clinical studies have demonstrated an anti-proliferative activity of somatostatin (SST) analogs in carcinoids. We report the case of a woman with liver metastases of neuroendocrine tumor and no evidence of the primary tumor. The liver metastases were characterized by high proliferation index, immunoreactiviy for somatostatin receptor (SSTR)-1, 2, 3 and 5 and positive octreoscan. Urinary 5-hydroxyindolacetic acid, serum serotonin and chromogranin A were elevated. Slow release lanreotide (SR-LAN) therapy for 3 mo controlled clinical and biochemical signs of carcinoid tumor and caused a clear-cut reduction in the diameter of two liver metastases and disappearance of another lesion, with further reduction after 6 and 18 mo. We demonstrated a clear-cut long-lasting anti-proliferative effect of SR-LAN on liver metastases of occult carcinoid with high proliferation index and immunoreactivity for SSTR-1, 2, 3, and 5. Immuno-histochemistry for SSTRs could be a suitable method for the selection of patients with metastatic carcinoid that may benefit from SST analog therapy. PMID:15801004

  4. Patient-reported outcomes with lanreotide Autogel/Depot for carcinoid syndrome: An international observational study.

    PubMed

    Ruszniewski, Philippe; Valle, Juan W; Lombard-Bohas, Catherine; Cuthbertson, Daniel J; Perros, Petros; Holubec, Luboš; Delle Fave, Gianfranco; Smith, Denis; Niccoli, Patricia; Maisonobe, Pascal; Atlan, Philippe; Caplin, Martyn E

    2016-05-01

    Lanreotide Autogel/Depot effectively controls symptoms in patients with carcinoid syndrome associated with neuroendocrine tumours. Data on patient-reported outcomes are sparse. To evaluate the effect of lanreotide on patient-reported outcomes (PROs) with carcinoid syndrome. This was an international, open-label, observational study of adults with neuroendocrine tumours and history of diarrhoea, receiving lanreotide for >3 months for relief of carcinoid syndrome symptoms. The primary PRO measure was satisfaction with diarrhoea control. Secondary PRO measures included severity, change in symptoms and impact on daily life of diarrhoea; and patient satisfaction with flushing control. Of 273 patients enrolled, 76% were 'completely' or 'rather' satisfied with diarrhoea control; 79% reported improvement in diarrhoea with lanreotide. The proportion of patients with 'mild', 'minimal', or 'no diarrhoea' increased from 33% before treatment to 75% during treatment; 75% were unconcerned about the impact of diarrhoea on daily life. Satisfaction with flushing control amongst patients with significant flushing at treatment initiation was 73%. Lanreotide treatment was associated with improvements in symptoms as well as a range of PROs in patients with neuroendocrine tumours and carcinoid syndrome (ClinicalTrials.gov: NCT01234168). Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

  5. [Typical pulmonary carcinoid tumor: evolution, related prognostic factors and lymphadenectomy indications].

    PubMed

    Bagan, P; Das-Neves-Pereira, J C; Rivera, C; Gibault, L; Badia, A; Mordant, P; Riquet, M; Le Pimpec Barthes, F

    2014-01-01

    The bronchopulmonary typical carcinoid tumors are often considered as non-metastatic neoplasia. The appearance of metastases is observed in 10% of the cases. We detail here studies based on the identification of the risk factors of metastases occurrence to adapt the lung surgery and lymph node dissection to the individual patient risk.

  6. Intranuclear Pseudo-inclusions and Grooves in Fine Needle Aspiration Cytology of Pulmonary Carcinoid Tumor.

    PubMed

    Mokhtari, Maral; Kumar, Perikala Vijayananda

    2016-02-01

    Cytologic findings of pulmonary carcinoid have been well described. We report new cytological findings in a case of carcinoid tumor. The patient is a 36-year-old man presenting with hemoptysis of about six months in duration. Chest CT scans showed a well-defined round polypoid lesion measuring 1 × 1 cm within the right upper lobe of the bronchus with hyperinflation of the right upper lobe. Trans-bronchial fine needle aspiration and biopsy were done. Cytologic smears showed isolated and loose clusters of uniform round to spindle shape cells with round centrally located nuclei, fine granular (salt and pepper) chromatin and pale cytoplasm. Intranuclear pseudo-inclusions and grooves were seen in some tumor cells. No mitotic figures or necrosis were evident. A cytological diagnosis of carcinoid tumor was made and histopathologic examination and subsequent immunohistochemical study confirmed the diagnosis. Carcinoid tumor may be reliably diagnosed on fine needle aspiration cytology smears. Intranuclear pseudo-inclusions and grooves may be evident in tumor cells.

  7. Primary cervical spine carcinoid tumor in a woman with arm paresthesias and weakness: a case report

    PubMed Central

    2013-01-01

    Introduction Carcinoid tumors are neuroendocrine neoplasms derived from the enterochromaffin cells. Central nervous system involvement is rare and has been reported either as metastases to the brain and spine or primary tumors involving the sacrococcygeal spine. We report the first case of a primary carcinoid tumor of the cervical spine. Case presentation A 50-year-old African-American woman presented with a 4-month history of numbness, paresthesias, and mild left-hand weakness. Magnetic resonance imaging of her cervical spine revealed a homogenously enhancing extradural mass, indenting the cervical cord and expanding the left neural foramen at C7–T1. A C7 corpectomy, en bloc resection of the tumor, and anterior C6–T1 fusion were performed to decompress the spinal cord and nerves and provide stability. Postoperative histopathologic examination and immunohistochemical analysis were consistent with carcinoid tumor. There has been no recurrence at the 6-year follow-up visit. Conclusions Primary cervical carcinoid tumor is extremely rare, but should be included in the differential diagnosis of enhancing expansile extradural masses compressing the spinal cord and nerves. Surgical resection may provide a definitive cure. PMID:23972315

  8. Endobronchial Carcinoid Tumor in a Girl with Initial Histologic Diagnosis of Leiomyoma

    PubMed Central

    Arshad, Muhammad; Haq, Mehmood-ul; Ali, Syed Waqas

    2015-01-01

    Endobronchial tumors represent the rarest cause of airway obstruction in pediatric population. Due to rarity of the condition, a high index of suspicion is required for early diagnosis. We report a patient in whom diagnostic bronchoscopic biopsy was reported as leiomyoma while post resection histopathology showed an atypical carcinoid. PMID:26623257

  9. Management of occult adrenocorticotropin-secreting bronchial carcinoids: limits of endocrine testing and imaging techniques.

    PubMed

    Loli, P; Vignati, F; Grossrubatscher, E; Dalino, P; Possa, M; Zurleni, F; Lomuscio, G; Rossetti, O; Ravini, M; Vanzulli, A; Bacchetta, C; Galli, C; Valente, D

    2003-03-01

    The differential diagnosis and the identification of the source of ACTH in occult ectopic Cushing's syndrome due to a bronchial carcinoid still represents a challenge for the endocrinologist. We report our experience in six patients with occult bronchial carcinoid in whom extensive hormonal, imaging, and scintigraphic evaluation was performed. All patients presented with hypercortisolism associated with high plasma ACTH values. The CRH test and high dose dexamethasone suppression test suggested an ectopic source of ACTH in three of six patients. During bilateral inferior petrosal sinus sampling, none of the patients showed a central to peripheral ACTH gradient. At the time of diagnosis, none of the patients had radiological evidence of the ectopic source of ACTH, whereas pentetreotide scintigraphy identified the lesion in two of four patients. Finally, a chest computed tomography scan revealed the presence of a bronchial lesion in all patients, and pentetreotide scintigraphy identified four of six lesions. In all patients a bronchial carcinoid was found and removed. In one patient with scintigraphic evidence of residual disease after two operations, radioguided surgery, using a hand-held gamma probe after iv administration of radiolabeled pentetreotide, was performed; this allowed detection and removal of residual multiple mediastinal lymph node metastases. In conclusion, our data show that there is not a single endocrine test or imaging procedure accurate enough to diagnose and localize occult ectopic ACTH-secreting bronchial carcinoids. Radioguided surgery appears to be promising in the presence of multiple tumor foci and previous incomplete removal of the tumor.

  10. Carcinoid tumor of the appendix: A consecutive series from 1237 appendectomies

    PubMed Central

    Tchana-Sato, Vincent; Detry, Olivier; Polus, Marc; Thiry, Albert; Detroz, Bernard; Maweja, Sylvie; Hamoir, Etienne; Defechereux, Thierry; Coimbra, Carla; Roover, Arnaud De; Meurisse, Michel; Honoré, Pierre

    2006-01-01

    AIM: To report the experience of the CHU Sart Tilman, University of Liège, Belgium, in the management of appendiceal carcinoid tumor. METHODS: A retrospective review of 1237 appendectomies performed in one single centre from January 2000 to May 2004, was undertaken. Analysis of demographic data, clinical presentation, histopathology, operative reports and outcome was presented. RESULTS: Among the 1237 appendectomies, 5 appendiceal carcinoid tumors were identified (0.4%) in 4 male and 1 female patients, with a mean age of 29.2 years (range: 6-82 years). Acute appendicitis was the clinical presentation for all patients. Four patients underwent open appendectomy and one a laparoscopic procedure. One patient was reoperated to complete the excision of mesoappendix. All tumors were located at the tip of the appendix with a mean diameter of 0.6 cm (range: 0.3-1.0 cm). No adjuvant therapy was performed. All patients were alive and disease-free during a mean follow-up of 33 mo. CONCLUSION: Appendiceal carcinoid tumor most often presents as appendicitis. In most cases, it is found incidentally during appendectomies and its diagnosis is rarely suspected before histological examination. Appendiceal carcinoid tumor can be managed by simple appendectomy and resection of the mesoappendix, if its size is ≤ 1 cm. PMID:17075987

  11. The First Reported Case of Recurrent Carcinoid Tumor in the External Auditory Canal.

    PubMed

    McCrary, Hilary C; Faucett, Erynne A; Reghunathan, Saranya; Aly, F Zahra; Khan, Rihan; Carmody, Raymond F; Jacob, Abraham

    2017-01-01

    Cutaneous neuroendocrine lesions of the external auditory canal (EAC) are exceptionally rare, with only five cases reported in the literature. In this case report, we present a patient with a recurrent carcinoid tumor in the EAC, which has yet to be described. A 38-year-old woman presenting with otalgia, aural fullness, and decreased hearing was found to have a recurrent EAC carcinoid tumor, 8-years after initial resection at an outside facility. The recurrent tumor involved much of the proximal, anterior bony ear canal and was trans-tympanic, extending to the middle ear and epitympanum; therefore, a lateral temporal bone resection was performed to ensure complete resection. Surgical pathology confirmed the presence a recurrent carcinoid tumor in the EAC, with immunohistochemistry positive for pancytokeratin (MAK6), CD56, and synaptophysin, with chromogranin showing rare cells positive for cytoplasmic granules. There was no evidence of metastasis. Lateral temporal bone resection was successful and the patient is being followed with annual imaging. The patient is considering future hearing rehabilitation with a bone anchored hearing device. This case report highlights the first known case of recurrent carcinoid tumor in the EAC, treated with lateral temporal bone resection. Clinical presentation, imaging, treatment, and pathology are reviewed along with a review of the literature.

  12. A Carcinoid Tumor of the Ampulla of Vater Treated by Endoscopic Snare Papillectomy

    PubMed Central

    Pyun, Dae-Keun; Moon, Gyoo; Han, Jimin; Kim, Myung-Hwan; Lee, Sang Soo; Seo, Dong-Wan; Lee, Sung-Koo

    2004-01-01

    Here, a case of a patient with incidental finding of a carcinoid tumor of the ampulla of Vater, who was treated with endoscopic snare papillectomy, is reported. A 62-year-old male was admitted to our hospital due to a carcinoid tumor of the ampulla of Vater, which was found during follow-up endoscopy after an endoscopic mucosal resection of early gastric cancer. No lymphadenopathy or visceral metastasis was found on an abdominal CT scan, In-111 octerotide scan and EUS. The ampulla was then en bloc removed by endoscopic snare papillectomy. The resected specimen revealed a 0.7×0.5×0.1 cm sized carcinoid tumor. All margins of resection were negative for tumor. After six months of follow-up, there was no evidence of recurrence and metastasis, either endoscopically or radiologically. To our knowledge, this case is the first report of an ampullary carcinoid tumor treated by endoscopic snare papillectomy in Korea. PMID:15683115

  13. Atypical presentation of carcinoid tumor with unresolved right shoulder pain: a case report.

    PubMed

    Tun, Nay Thi; Oza, Rajen

    2014-05-07

    Carcinoid tumors are variants of neuroendocrine tumors that typically arise from the gastrointestinal tract and the bronchus, but they can involve any organ. Unresolved right shoulder pain manifesting as the first clinical presentation of carcinoid tumor with unknown primary origin is a rare clinical entity. To the best of our knowledge, herein we present the first case report describing metastasis to the right shoulder joint in a patient who presented with bone pain as the first clinical manifestation of metastatic carcinoid tumor of unknown primary origin. Metastasis to the right scapula as the first presentation of an underlying carcinoid tumor in the primary bronchus has been reported previously. A 72-year-old Caucasian woman presented with pain in her right shoulder after a fall. She delayed seeking medical attention for 4 weeks for personal reasons. Her physical examination revealed no erythema or swelling of the right shoulder. However, tenderness was noted on the right subacromial bursa and the right acromioclavicular joint. Her drop arm test was positive. An X-ray of the right upper extremity showed no fracture. She did not respond to methylprednisolone injections or physical therapy. Because of the unresolved right shoulder pain with disturbance of her daily activities, magnetic resonance imaging of the right shoulder was ordered, which revealed permeative destruction of the right scapula. Because the permeative destruction of the bone could have been an osteolytic malignant feature, positron emission tomography-computed tomography was performed, which produced a scan showing osseous metastasis to the right scapula, multiple liver metastases and a 1.7 cm right-lower-lobe pulmonary nodule. Her serotonin and chromogranin A levels were significantly elevated. The patient was treated with palliative cisplatin and etoposide chemotherapy followed by locoregional treatments for metastatic carcinoid tumor. She had mild improvement in her right shoulder pain, as

  14. Surgical management and outcome of rectal carcinoids in a university hospital.

    PubMed

    Wei, Rockson; Lo, Oswens S H; Law, Wai Lun

    2015-02-07

    Rectal carcinoids are an uncommon entity comprising only 1%-2% of all rectal tumors. Rectal carcinoids are frequently diagnosed during colonoscopy, but management after polypectomy is still controversial. The aims of this study were to review the surgical procedures for rectal carcinoids and to compare the outcomes of patients after different treatment modalities in a university hospital in Hong Kong. All rectal carcinoids diagnosed between January 2003 and September 2012 were reviewed retrospectively, including clinicopathological characteristics, their management, and surgical outcomes. There were 54 patients with a median age of 60 years, and 32 were males (59.3%). All patients underwent colonoscopy, and the most had rectal bleeding (53.7%). Two patients were diagnosed incidentally in the surgical specimens of rectal tissues. Eighteen patients were diagnosed to have rectal carcinoids after snaring polypectomy, and no further intervention was required. Twenty-five patients had local resection either by means of transanal resection or transanal endoscopic operation. Radical resection was performed in seven patients in which one had T3N1 disease and the others did not have any lymph node metastasis. In the median follow-up of 30 months (10-108 months), there was no recurrence in the "incidental" or post-polypectomy group. However, two patients with transanal resection and two patients with radical resection developed hepatic metastases after 13-24 months post-treatment. The 5-year overall survival was 100% in patients having snaring polypectomy only, 83% for those with local resection, and 63% in patients who underwent radical surgery (p = 0.04). Our data suggested that that local resection was an effective treatment for small rectal carcinoids and generally brought about good oncological and surgical outcomes. For larger tumors, radical resection seemed to provide acceptable oncological outcomes. Regular surveillance with colonoscopy and endorectal

  15. Interobserver agreement of proliferation index (Ki-67) outperforms mitotic count in pulmonary carcinoids.

    PubMed

    Warth, Arne; Fink, Ludger; Fisseler-Eckhoff, Annette; Jonigk, Danny; Keller, Marius; Ott, German; Rieker, Ralf J; Sinn, Peter; Söder, Stephan; Soltermann, Alex; Willenbrock, Klaus; Weichert, Wilko

    2013-05-01

    Evaluation of proliferative activity is a cornerstone in the classification of endocrine tumors; in pulmonary carcinoids, the mitotic count delineates typical carcinoid (TC) from atypical carcinoid (AC). Data on the reproducibility of manual mitotic counting and other methods of proliferation index evaluation in this tumor entity are sparse. Nine experienced pulmonary pathologists evaluated 20 carcinoid tumors for mitotic count (hematoxylin and eosin) and Ki-67 index. In addition, Ki-67 index was automatically evaluated with a software-based algorithm. Results were compared with respect to correlation coefficients (CC) and kappa values for clinically relevant grouping algorithms. Evaluation of mitotic activity resulted in a low interobserver agreement with a median CC of 0.196 and a median kappa of 0.213 for the delineation of TC from AC. The median CC for hotspot (0.658) and overall (0.746) Ki-67 evaluation was considerably higher. However, kappa values for grouped comparisons of overall Ki-67 were only fair (median 0.323). The agreement of manual and automated Ki-67 evaluation was good (median CC 0.851, median kappa 0.805) and was further increased when more than one participant evaluated a given case. Ki-67 staining clearly outperforms mitotic count with respect to interobserver agreement in pulmonary carcinoids, with the latter having an unacceptable low performance status. Manual evaluation of Ki-67 is reliable, and consistency further increases with more than one evaluator per case. Although the prognostic value needs further validation, Ki-67 might perspectively be considered a helpful diagnostic parameter to optimize the separation of TC from AC.

  16. A carcinoid tumor arising from a mature cystic teratoma in a 25-year-old patient: a case study.

    PubMed

    Kim, Joo-Yeon

    2016-04-21

    Mature cystic teratomas (MCTs) are common benign tumors occurring in the ovaries. Malignant transformation of teratomas (TMT) occurs in 1-3% of all MCTs, usually in postmenopausal women. Squamous cell carcinoma is the most common tumor type. Primary carcinoid tumors of the ovary are uncommon, representing only 0.3% of all carcinoid tumors and less than 0.1% of all ovarian cancers. A carcinoid tumor of the trabecular type arising from a MCT is presented in a 25-year-old woman. TMT was detected incidentally. Histologically, uniform, polygonal cells were arranged in a cord and trabecular pattern. Immunohistochemical staining showed positivity for neuro-specific enolase, synaptophysin, and CD56. This case represents a very rare example of carcinoid tumor occurring in a woman younger than 30 years of age. Our findings suggest that sufficient tumor sampling is necessary to avoid overlooking small lesions, which, in our case, were not detected on either radiologic or gross examination.

  17. 18F-FDG PET/CT and MR findings of ovarian carcinoid within a dermoid cyst.

    PubMed

    Horikawa, Masahiro; Shinmoto, Hiroshi; Soga, Shigeyoshi; Miyai, Kosuke; Kaji, Tastumi

    2014-09-01

    Ovarian carcinoid is a rare neoplasm of low-grade malignancy occurring within a dermoid cyst or mucinous tumor, predominantly in perimenopausal women. Reports with radiologic features are scarce. We present a case of a 57-year-old woman with an ovarian carcinoid within a dermoid cyst manifested as a multilocular cystic mass with a solid component showing 18F-FDG PET uptake (SUVmax=6.02).

  18. Goblet cells carcinoid with mucinous adenocarcinoma of the vermiform appendix: a step towards the unitary intestinal stem cell theory?

    PubMed

    Gravante, G; Yahia, S; Gopalakrishnan, K; Mathew, G

    2014-06-01

    Associations of various histotypes in appendiceal neoplasms may help elucidate the histogenesis of such uncommon tumors. We present the fourth published case of Goblet Cell Carcinoid (GCC) associated with mucinous adenocarcinoma of the appendix. This association has been described only for GCC and not for classic appendix carcinoids which are thought to originate from neuroendocrine-committed cells. The GCC-mucinous association adds more towards the theory of a pluripotent intestinal stem cell with amphicrine possibilities of differentiation.

  19. Recurrence of a carcinoid tumor of the ovary 13 years after the primary surgery: A case report

    PubMed Central

    AMANO, YASUAKI; MANDAI, MASAKI; BABA, TSUKASA; HAMANISHI, JUNZO; YOSHIOKA, YUMIKO; MATSUMURA, NORIOMI; KONISHI, IKUO

    2013-01-01

    The current study presents the case of a patient with a recurrent carcinoid tumor of the ovary, 13-years after the primary surgery. The primary surgery consisted of a total abdominal hysterectomy and bilateral salpingo-oophorectomy for a left ovarian tumor at 54 years old. Pathologically, the tumor was diagnosed as a carcinoid tumor of the ovary. Following the primary treatment, the patient was admitted to a cardiologist due to carcinoid-induced heart failure. At 67 years old, the patient was referred to Kyoto University Hospital with a solitary mass 8 cm in diameter and located in the paraaortic area, which was detected by routine ultrasonography and subsequent computed tomography (CT) scans. Urinary 5-hydroxyindole acetate (5-HIAA), a serotonin degradation metabolite, was present at elevated levels. With a diagnosis of a recurrent carcinoid tumor, the patient underwent a tumor resection. The pathological diagnosis was that of lymph node metastasis of the trabecular carcinoid. Post-operatively, the 5-HIAA levels returned to normal. Carcinoid tumors occasionally recur following surgery due to borderline malignant potential. Due to the slow growing nature of these tumors, in specific cases, recurrence occurs following a long interval. Therefore, a relatively long follow-up period is required. PMID:24179502

  20. Multifocal Carcinoid Tumor of Small Intestine: A Rare Cause of Chronic Obscure Gastrointestinal Bleeding, Suspected on Capsule Endoscopy and Diagnosed on Double Balloon Enteroscopy

    PubMed Central

    Hernandez, Jose C.; Rojas, Juan; Gurudu, Suryakanth R.

    2011-01-01

    We reported a case of multifocal carcinoid tumor of small intestine causing chronic obscure gastrointestinal bleeding, suspected on capsule endoscopy and diagnosed on double balloon enteroscopy. PMID:27942327

  1. FAIM2, as a novel diagnostic maker and a potential therapeutic target for small-cell lung cancer and atypical carcinoid

    PubMed Central

    Kang, Hio Chung; Kim, Jong In; Chang, Hee Kyung; Woodard, Gavitt; Choi, Young Sik; Ku, Ja-Lok; Jablons, David M.; Kim, Il-Jin

    2016-01-01

    Lung neuroendocrine (NE) tumors are a heterogeneous group of tumors arising from neuroendocrine cells that includes typical carcinoid, atypical carcinoid, small cell lung cancer (SCLC), and large cell NE cancer. The subtyping of NE tumors is based on the number of mitoses per high powered field and the presences of necrosis. However, the best diagnostic criteria to differentiate various subtypes of lung NE tumors remains controversial and few diagnostic markers distinguish typical and atypical carcinoid. In this study, we show that FAIM2, an inhibitory molecule in the Fas-apoptosis pathway, is significantly overexpressed in SCLC compared to non-small cell lung cancer. In addition, FAIM2 expression is significantly higher in atypical carcinoid than typical carcinoid. As atypical carcinoid has been shown to have worse clinical outcomes than typical carcinoid, our data suggests that FAIM2 may be a useful diagnostic marker for atypical carcinoid. Knockdown of FAIM2 expression increases Fas-induced apoptotic cell death in SCLC cells. Etoposide treatment combined with FAIM2 inhibition also shows modest but significant reduction of viable SCLC cells. Taken together, our results suggest that FAIM2 is a potential NE tumor marker with higher expression in atypical carcinoid and SCLC, and could be a new therapeutic target for SCLC. PMID:27677402

  2. 5-HIAA excretion is not associated with bone metabolism in carcinoid syndrome patients.

    PubMed

    van Dijk, S C; de Herder, W W; Kwekkeboom, D J; Zillikens, M C; Feelders, R A; van Schaik, R H N; van Driel, M; van Leeuwen, J P T M

    2012-06-01

    In patients with a carcinoid syndrome and neuroendocrine tumors of the digestive tract (carcinoids), elevated circulating serotonin (5-hydroxytryptamine, 5-HT) levels can be demonstrated. It can be hypothesized that bone metabolism will be affected in these patients, since serotonin receptors are expressed on bone cells and serotonin effects on bone have been demonstrated. However, to date, no data are available on bone metabolism parameters in patients with neuroendocrine tumors of the digestive tract (carcinoids). In the current retrospective study we have measured serum bone formation markers P1CP (pro-collagen type I C-terminal), and osteocalcin, and the bone resorption marker NTx (collagen breakdown product N-terminal), in a group of 61 carcinoid patients with increased circulating serotonin levels as demonstrated by increased excretion of the serotonin breakdown product, 5-hydroxy indole acetic acid (5-HIAA), in the urine (>50 μmol/24 h, so-called "hyper-secretors") and a control group of 23 carcinoid patients, without increased 5-HIAA excretion (so-called non-secretors). The 24-h urinary excretion of 5-HIAA reflects the 24-h production of serotonin. Measurements of markers of bone metabolism were performed in serum samples obtained before the start of medical treatment. The hypersecretor group had on average a 10-fold higher urinary 5-HIAA excretion than the control (non-secretor) group. No significant differences in bone metabolism parameters could be demonstrated between hyper-secretors and controls (non-secretors). Correlation and regression analyses could not demonstrate significant age- and sex-adjusted correlations between urinary 5-HIAA excretion and any of the markers for bone turnover. A limitation is that the exposure time to elevated levels of serotonin is unknown, which might have been too short to induce effects on bone metabolism. Treatment of human pre-osteoblasts SV-HFO with serotonin didn't change alkaline phosphatase activity throughout

  3. High-grade neuroendocrine carcinomas of the lung highly express enhancer of zeste homolog 2, but carcinoids do not.

    PubMed

    Findeis-Hosey, Jennifer J; Huang, Jiaoti; Li, Faqian; Yang, Qi; McMahon, Loralee A; Xu, Haodong

    2011-06-01

    Enhancer of zeste homolog 2, the catalytic subunit of polycomb repressive complex 2, is a histone methyltransferase and plays an important role in cell proliferation and cell cycle regulation. It has been shown to be overexpressed in a number of malignant neoplasms. This study aimed to determine the expression pattern of enhancer of zeste homolog 2 in neuroendocrine tumors of the lung and the potential of enhancer of zeste homolog 2 to serve as a biomarker to segregate carcinoids from high-grade neuroendocrine carcinomas. Fifty-four cases, including 25 typical carcinoids, 7 atypical carcinoids, 9 large-cell neuroendocrine carcinomas, and 13 small-cell lung carcinomas, were immunohistochemically studied using a monoclonal antibody against enhancer of zeste homolog 2. All 13 small-cell lung carcinomas demonstrated moderate to strong nuclear staining with 12 exhibiting more than 90% of tumor cells staining. All 9 large-cell neuroendocrine carcinomas were moderately to strongly positive for enhancer of zeste homolog 2, with 6 cases having staining in more than 80% of tumor cells. In contrast, all 25 typical carcinoids and 6 atypical carcinoids showed only rare scattered enhancer of zeste homolog 2-positive tumor cells, with 1 case of atypical carcinoid exhibiting moderate staining in 40% of tumor cells. A subsequent validation study of the 14 specimens of lung or mediastinal lymph node biopsy and fine-needle aspiration, including 6 small-cell lung carcinomas, 2 large-cell neuroendocrine carcinomas, 5 typical carcinoids, and 1 atypical carcinoid, was performed. Enhancer of zeste homolog 2 was diffusely and strongly positive in all small-cell lung carcinomas and large-cell neuroendocrine carcinomas, even with severe crush artifact, whereas it was only positive in rare tumor cells in carcinoids. These findings support the formulation that enhancer of zeste homolog 2 may play an important role in the regulation of biologic behavior of high-grade neuroendocrine carcinomas

  4. [Carcinoid tumor of the small bowel: value of hydro-MR imaging for diagnosis].

    PubMed

    Azoulay, R; Boudiaf, M; Soyer, Ph; Hamzi, L; Abitbol, M; Najmeh, N; Rymer, R

    2003-12-01

    Hydro-MR imaging is a technique based on the use of a strongly T2-weighted single-shot fast spin-echo (SSFSE) sequence, similar to that used for MR-cholangiography. We report herein one case of carcinoid tumor of the small bowel diagnosed by hydro-MR imaging. This non invasive MR technique showed suggestive features such as radial convergence and segmental dilatation of a small bowel loop, similar to those seen on conventional follow-through studies. This case illustrates the major role that may be played in the future by hydro-MR imaging for the non invasive diagnosis of carcinoid tumor of the small bowel without the use of ionizing radiation.

  5. Renal Carcinoid Tumor Metastatic to the Uvea, Medial Rectus Muscle, and the Contralateral Lacrimal Gland.

    PubMed

    Kiratli, Hayyam; Uzun, Salih; Tarlan, Berçin; Ateş, Deniz; Baydar, Dilek Ertoy; Söylemezoğlu, Figen

    2015-01-01

    Renal carcinoid tumor is an exceedingly rare malignancy. A 57-year-old man with a renal carcinoid tumor discovered after metastasizing to intraocular and bilateral orbital structures is described. The patient presented with a blind painful OS and a right superotemporal subconjunctival mass. Imaging studies revealed a large left intraocular tumor, a mass in the left medial rectus muscle, and right lacrimal gland enlargement. The OS was enucleated, and incisional biopsies were performed from the other 2 lesions. Histopathological studies demonstrated metastatic neuroendocrine tumor with chromogranin and synaptophysin positivity. Systemic work up revealed a right renal mass and multiple hepatic metastatic lesions. Radical nephrectomy was performed, and octreotide, capecitabine, and temozolomide were administered. Removal of the primary tumor and the eye that had no prospect for useful vision and further treatment with octreotide, capecitabine, and temozolomide provided a disease progression-free period of 24 months and allowed the patient to function normally.

  6. Acute appendicitis with a neuroendocrine tumor G1 (carcinoid): pitfalls of conservative treatment.

    PubMed

    Watanabe, Hiroyuki A; Fujimoto, Taketoshi; Kato, Yo; Sasaki, Mayumi; Ikusue, Toshikazu

    2016-08-01

    A man in his early thirties presented to our clinic with right lower abdominal pain. Computed tomography (CT) and ultrasonography (US) revealed a swollen appendix and an appendicolith. Abscess formation was not observed but ongoing appendiceal rupture was not ruled out. Three months after successful conservative therapy, the lumen of the apical portion was kept dilated and laparoscopic interval appendectomy was performed. No tumorous findings were observed macroscopically. However, histology revealed many tiny nests infiltrating the submucosa, muscular layer, and subserosa at the root of the appendix. An appendiceal neuroendocrine tumor G1 (NET G1; carcinoid) was diagnosed immunohistologically. Neither CT nor US visualized the tumor because of its non-tumor-forming but infiltrative growth. In conclusion, after successful conservative treatment, interval appendectomy should be considered to uncover a possible appendiceal NET G1 (carcinoid), particularly when dilatation of the distal lumen is kept under observation.

  7. [Primary retroperitoneal carcinoid tumor associated with multiple endcrine neoplasia (men) type 1: a case report].

    PubMed

    Chiba, Syuji; Numakura, Kazuyuki; Satoyoshi, Kiyofumi; Saito, Mitsuru; Horikawa, Yohei; Takayama, Koichiro; Nara, Taketoshi; Kanda, Sohei; Miura, Yoshiko; Maita, Shinya; Tsuruta, Hiroshi; Obara, Takashi; Kumazawa, Teruaki; Narita, Shintaro; Tsuchiya, Norihiko; Satoh, Shigeru; Habuchi, Tomonori

    2011-11-01

    We report an extremely rare case of a 69-year-old man having a retroperitoneal carcinoid tumor associated with multiple endocrine neoplasia (MEN) type 1. The patient whose son and daughter were previously diagnosed with MEN type 1 was admitted to the Department of Endocrinology at our hospital for evaluation of this disorder. Computed tomography (CT) and ultrasonography revealed a parathyroid and retroperitoneal tumor (43 mm x 34 mm). The patient did not consent to surgical management of the tumor; however three years later, a follow-up CT revealed tumor enlargement (55 mm x 50 mm). We were unable to rule out a malignancy, and subsequently resected the tumor. A pathological diagnosis of retroperitoneal carcinoid was made. No local recurrence or metastasis have been observed for 21 months.

  8. Carcinoid tumor of the middle ear: a case report and review of literature.

    PubMed

    Liu, Guo; Chen, Fei; Li, Jin-Nan; Liu, Shi-Xi

    2014-01-01

    Carcinoid tumors of the middle ear are very rare. Here we describe a 37-year-old man with multiple recurrent carcinoid tumor of the right middle ear. The CT demonstrated the recurrent mass that filled the tympanum and mastoid with osteolytic invasion, and the tumor was removed by surgery. The pathological findings showed the tumor cells, without necrosis and mitotic activity, had round, oval, or slightly irregular nuclei and finely-dispersed chromatin, arranged in cords, nests, and glandular structures. They were strongly positive for synaptophysin and CD56, but were negative for S-100 and chromogranin A. Ki-67 proliferation activity was low (<2%). With a review of the literature, the clinical, pathological characteristics and treatment modalities of this rare tumor are discussed.

  9. A case of uncontrolled severe asthma patient with coexisting carcinoid tumor presenting as pneumomediastinum.

    PubMed

    Biçer, Elif Nur; Öztürk, Ayse Bilge; Ozyigit, Leyla Pur; Erus, Suat; Tanju, Serhan; Dilege, Şükrü; Tabak, Levent

    2015-01-01

    Patients with inadequately controlled or uncontrolled asthma are at a greater risk of attacks for asthma requiring emergency room visits or hospital admissions. There is a significant correlation between the severity of the disease and the severity of exacerbations. Patients with poorly controlled asthma are at a higher risk for complications. We present a 24-year-old aspirin-intolerant, uncontrolled asthma patient with the complication of pneumomediastinum. Severe symptoms persisted after the resolution of the pneumomediastinum despite intense anti-inflammatory and anti-obstructive therapy. A bronchoscopy revealed an endobronchial lesion and she was diagnosed with a carcinoid tumor. This case is an example of the importance of re-evaluating asthma patients who do not respond to standard medical treatment. Clinicians should be aware of the complications associated with asthma attacks such as pneumomediastinum and the possibility of a differential diagnosis that worsen asthma symptoms such as a carcinoid tumor.

  10. Carcinoid tumor of the cecum presenting with acute appendicitis: a case report.

    PubMed

    Cakar, E; Bayrak, S; Bektaş, H; Colak, Ș; Guneyi, A; Sevinc, M Mahsuni; Bag, M

    2015-01-01

    Acute appendicitis is the most common cause of acute abdomen. Carcinoid tumor of the appendix is a rare incidental finding that can present with the clinical picture of acute appendicitis. During open surgery for acute appendicitis, a 3 cm solid mass, not noticed externally, was palpated at the base of the appendix. The mass and the appendix were excised by en-bloc wedge resection. The histopathological examination of the lesion revealed carcinoid tumor. The aim of this presentation is to remind that neoplasms of the appendix may, although rarely, present the clinical picture of acute appendicitis, and to highlight that they, particularly those located at the base of the appendix and in cecum, may be overlooked during laparoscopy. The importance of preoperative computerized tomography ins uch cases has to be underlined. Celsius.

  11. Carcinoid tumor of the middle ear: a case report and review of literature

    PubMed Central

    Liu, Guo; Chen, Fei; Li, Jin-Nan; Liu, Shi-Xi

    2014-01-01

    Carcinoid tumors of the middle ear are very rare. Here we describe a 37-year-old man with multiple recurrent carcinoid tumor of the right middle ear. The CT demonstrated the recurrent mass that filled the tympanum and mastoid with osteolytic invasion, and the tumor was removed by surgery. The pathological findings showed the tumor cells, without necrosis and mitotic activity, had round, oval, or slightly irregular nuclei and finely-dispersed chromatin, arranged in cords, nests, and glandular structures. They were strongly positive for synaptophysin and CD56, but were negative for S-100 and chromogranin A. Ki-67 proliferation activity was low (<2%). With a review of the literature, the clinical, pathological characteristics and treatment modalities of this rare tumor are discussed. PMID:25400805

  12. Synchronous adenocarcinoma and carcinoid tumor of the terminal ileum in a Crohn's disease patient

    PubMed Central

    Cioffi, Ugo; De Simone, Matilde; Ferrero, Stefano; Ciulla, Michele M; Lemos, Alessandro; Avesani, Ettore Contessini

    2005-01-01

    Background Several malignancies have been described in association with inflammatory bowel diseases, the most common being adenocarcinoma. Carcinoid tumor and Crohn disease has also been previously reported, however the coexistence of both neoplasms is quite rare and the clinical diagnosis is very difficult. Here we report what we believe to be the fourth case of a mixed adenocarcinoid tumor coexisting with Crohn's disease. Case report The patient presented with clinical and radiological features of intestinal obstruction. Laparotomy showed a stricturing lesion in the last 6 cm of the terminal ileum with proximal dilation. Only the histology of the resected surgical specimen proved the presence of a mixed adenocarcinoid tumor involving the terminal ileum. Conclusion Carcinoid tumor should be suspected in elderly patients with Crohn's disease presenting with intestinal obstruction and laparotomy should be considered to exclude malignancy. PMID:16336666

  13. Carcinoid syndrome from small bowel endocrine carcinoma in the absence of hepatic metastasis.

    PubMed

    Datta, Sayan; Williams, Nigel; Suortamo, Sari; Mahmood, Asif; Oliver, Colin; Hedley, Nicholas; Ray, Pijush

    2011-11-01

    An 80-year-old male patient presented with abdominal pain, paroxysmal diaphoresis, diarrhoea and vomiting. CT scan revealed a small bowel endocrine carcinoma (or 'carcinoid' tumour), but the absence of hepatic disease. The lesion was excised 'en-bloc'. Intra-operatively, there was wide fluctuation in blood pressure associated with tumour manipulation, with hyper- and hypotension. Carcinoid syndrome usually occurs from gastrointestinal tumours when hepatic metastases occur, causing flushing, diarrhoea, bronchoconstriction and murmurs from cardiac valvular lesions. This patient did not have radiological evidence of hepatic metastasis, but the syndrome could still occur with midgut tumours via local invasion of the retroperitoneal circulation, or by action of substances other than serotonin that do not undergo hepatic metabolism.

  14. The endocrine tumor summit 2008: appraising therapeutic approaches for acromegaly and carcinoid syndrome

    PubMed Central

    Melmed, Shlomo; Clemmons, David R.; Colao, Annamaria; Cunningham, Regina S.; Molitch, Mark E.; Vinik, Aaron I.; Adelman, Daphne T.; Liebert, Karen J. P.

    2009-01-01

    The Endocrine Tumor Summit convened in December 2008 to address 6 statements prepared by panel members that reflect important questions in the treatment of acromegaly and carcinoid syndrome. Data pertinent to each of the statements were identified through review of pertinent literature by one of the 9-member panel, enabling a critical evaluation of the statements and the evidence supporting or refuting them. Three statements addressed the validity of serum growth hormone (GH) and insulin-like growth factor-I (IGF-I) concentrations as indicators or predictors of disease in acromegaly. Statements regarding the effects of preoperative somatostatin analog use on pituitary surgical outcomes, their effects on hormone and symptom control in carcinoid syndrome, and the efficacy of extended dosing intervals were reviewed. Panel opinions, based on the level of available scientific evidence, were polled. Finally, their views were compared with those of surveyed community-based endocrinologists and neurosurgeons. PMID:20012914

  15. Two different types of carcinoid tumors of the lung: immunohistochemical and ultrastructural investigation and their histogenetic consideration.

    PubMed

    Min, Kyung-Whan

    2013-02-01

    Carcinoid tumors have been an interesting clinical and pathological entity for pathologists because of their unique histopathologic pattern of "Zellballen" (cell ball) and the hormones they produce demonstrable by histochemical and biochemical methods, including immunohistochemistry, and the presence of cytoplasmic dense-core particles demonstrable by electron microscopy. Since carcinoid tumors were established as an entity more than a century ago by Oberndorfer, who was credited with coining the term "carcinoid," meaning carcinoma-like tumors, tumors presenting with similar characteristics have been reported in most of parenchymal organs, including lungs. Carcinoid tumors in the lungs usually occur as bronchocentric tumors and present with typical histopathologic characteristics of carcinoid tumors, but they may present with significant variation in their cellular compositions, in contrast to the midgut carcinoid tumors. In the latter, tumor cells are quite similar to enterochromaffin granule containing crypt cells, which are regarded as their progenitor cells. Currently, a similar histogenetic explanation is applied to all carcinoid tumors occurring elsewhere. The bronchus is one of the most common anatomic sites in which the carcinoid tumors occur. However, bronchial carcinoid tumors differ from the midgut counterparts in microscopic appearance, showing more variability in cellular shape and composition from the classical form of midgut carcinoid tumors. In the lungs, neuroendocrine cells (NEC) are normally found in two different ways. Firstly, they are found as randomly scattered single cells (Kultchitsky cells) similar to enteric counterparts, and, secondly, they are found in aggregates known as "neuroepithelial bodies" (NEB) usually found in the branching point of bronchi. Interestingly, they keep a close anatomic relationship with parasympathetic nerve structures and even form synapses. NEB are usually found in the early stage of fetal development and are

  16. Prevalence and clinicopathological characteristics of appendiceal carcinoids in Sharjah (United Arab Emirates).

    PubMed

    Anwar, Khurshid; Desai, Munaf; Al-Bloushi, Noura; Alam, Farheen; Cyprian, Farhan Sachal

    2014-07-15

    To determine the incidence and clinico-pathological profile of appendiceal carcinoids in a cohort of patients undergoing emergency appendicectomies for clinically suspected acute appendicitis in Sharjah, United Arab Emirates (UAE). The study included the retrospective data of 964 patients operated for clinically suspected acute appendicitis, and the resected specimens were received at Al-Qasmi Hospital (Sharjah) from January 2010 to December 2010. The data of the patients who were histologically reported to have carcinoid tumors of the appendix were extensively evaluated for the patient's demographics, indication for surgery, surgical procedure, tumor localization in the appendix, diameter of the lesion, concomitant appendicitis, immunohistochemistry studies and clinical follow-up. Out of the 964 patients included in the study, 9 (0.93%) were found to have appendiceal carcinoids. The mean age reported was 28.7 years with a male to female ratio of 2:1. Eight tumors were located near the tip of the appendix with a mean diameter of 3.3 mm, while the remaining one was near the proximal end of the appendix. All the cases were associated with concomitant suppurative appendicitis. In seven reported cases, tumors were confined to the muscular layer while in one case each there was an extension to the serosa and mesoappendix, respectively. All tumors were found to be positive for chromogranin A, synaptophysin and neuron-specific enolase on immunohistochemistry but negative for cytokeratin-7. None of the patients developed recurrence or any reportable complications in the short follow-up period (12-26 mo) that was arranged as a six-monthly re-evaluation by abdominal ultrasonography. Our study found a higher incidence of appendiceal carcinoids in patients undergoing emergency appendectomy for acute appendicitis in Sharjah, UAE compared to two previous studies from the Persian Gulf region. Interestingly, tumors were found to be more commonly in young males, which is in

  17. Factors associated with complete local excision of small rectal carcinoid tumor.

    PubMed

    Son, Hae-Jung; Sohn, Dae Kyung; Hong, Chang Won; Han, Kyung Su; Kim, Byung Chang; Park, Ji Won; Choi, Hyo Seong; Chang, Hee Jin; Oh, Jae Hwan

    2013-01-01

    Although small rectal carcinoid tumors can be treated using local excision, complete resection can be difficult because tumors are located in the submucosal layer. We evaluate the factors associated with pathologically complete local resection of rectal carcinoid tumors. Data were analyzed of 161 patients with 166 rectal carcinoid tumors who underwent local excision with curative intent from January 2001 to December 2010. A pathologically complete resection (P-CR) was defined as an en bloc resection with tumor-free lateral and deep margins. The study classified treatments into three categories for analysis: conventional polypectomy (including strip biopsy, snare polypectomy, and hot biopsy), advanced endoscopic techniques (including endoscopic mucosal resection with cap and endoscopic submucosal dissection), and surgical local excision (including transanal excision and transanal endoscopic microsurgery). We evaluated the P-CR rate according to treatment method, tumor size, initial endoscopic impression and the use of endoscopic ultrasound (EUS) or transrectal ultrasound (TRUS). The mean tumor size was 5.51 ± 2.43 mm (range 2-18 mm) and all lesions were confined to the submucosal layer. The P-CR rates were 30.9, 72.0, and 81.8 % for conventional polypectomy, advanced endoscopic techniques, and surgical local excision, respectively. Univariate analysis showed that P-CR was associated with treatment method, use of EUS or TRUS, and initial endoscopic impression. Multivariate analysis showed that only treatment method was associated with P-CR. Pathologically complete resection of small rectal carcinoid tumors was more likely to be achieved when using advanced endoscopic techniques or surgical local excision rather than conventional polypectomy.

  18. Primary pure carcinoid tumors of the testis: Clinicopathological and immunophenotypical characteristics of 11 cases

    PubMed Central

    LU, CHANGLI; ZHANG, ZHANG; JIANG, YONG; YANG, ZHIRONG; YANG, QUNPEI; LIAO, DIANYING; BU, HONG

    2015-01-01

    Primary pure carcinoid tumors of the testis (pPCTT) are rare, and there are only a limited number of studies available. In the present study we described the clinicopathological and immunophenotypical characteristics of 11 cases from our institution between 1978 and 2014, and reported our experiences of the diagnosis and treatment of these patients. The patients ranged in age from 26 to 68 years old, with a median age of 48 years. One patient (9%) was classified as pT2 and 10 (91%) were pT1. Histologically, 7 cases were diagnosed as classical carcinoid tumors, while the other 4 cases were identified as atypical carcinoid tumors. The most common growth pattern was a mixed insular, acinar, rosetted, solid and trabecular pattern. Immunohistochemical staining revealed positive expression of neuron-specific enolase in all cases, and CgA, Syn and CD56 markers in 8 (72.7%), 10 (90.9%) and 9 cases (81.7%), respectively. In addition to radical orchiectomy, 9 patients (81.7%) received a combined modality of treatment. Follow-up data were available for 8 patients. Seven were alive at the last follow-up without recurrence, and one patient succumbed to cerebral hemorrhage 7 years after surgery. In summary, localized pPCTT is a rare disease with an indolent clinical course. When a testicular carcinoid tumor is identified, a metastasis or an intestinal primary tumor should be excluded, particularly when the testicular tumor is large. A tumor size ≤6.0 cm and the histological appearance had little relation with metastatic behavior. PMID:26137005

  19. A case of bronchial carcinoid: diagnosis and follow-up with 111In-DTPA-octreotide.

    PubMed

    Orsolon, P; Bagni, B; Basadonna, P; Geatti, O; Talmassons, G; Guerra, U P

    1995-12-01

    Scintigraphy with radiolabelled analogue of somatostatin is highly sensitive in detecting carcinoid tumors especially if performed with Single Photon Computed Tomography (SPECT). In this report we describe our experience with 111In-DTPA-Octreotide in a female patient affected by a small asymptomatic intrabronchial carcinoid demonstrated by CT scan and bronchial endoscopy performed after recurrent left pneumonias. Planar views and SPECT images, using 111In-DTPA-Octreotide, were collected before and four hours after the first endoscopic laser resection. All groups of SPECT images were positive in the left parahilar region but at a different degree. Scans performed after resection showed a low degree of uptake which was considered to be probably secondary to local swelling; CT scan was negative. Follow up endoscopic biopsy repeated at six months, showed a relapse always in the same site; CT scan of the thorax was again negative. 111In-DTPA-Octreotide images obtained at twelve months were positive always in the left parahilar region, CT scan was negative but another biopsy was not possible. Therefore it was suspected a relapse of the carcinoid which was probably growing only through the bronchial wall without spreading towards the bronchial lumen and/or the lung parenchima. In this occasion, it was also thought that images collected four hours after resection could be positive not only for swelling but for a relapse as well. In every scintigraphic session, SPECT images presented higher quality than planar. This case suggests that 111In-DTPA-Octreotide SPECT is a non-invasive diagnostic technique which could be applied as a follow-up tool especially to patients with no-secreting carcinoid neoplasm and/or with negative or doubtful endoscopic and radiological investigations.

  20. Inhibition of Phosphatidylinositol 3-Kinase/Akt Signaling Suppresses Tumor Cell Proliferation and Neuroendocrine Marker Expression in GI Carcinoid Tumors

    PubMed Central

    Pitt, Susan C.; Chen, Herbert; Kunnimalaiyaan, Muthusamy

    2010-01-01

    Background Over-activation of PI3K/Akt signaling facilitates tumor proliferation in several cancers. We have shown that various signal transduction pathways promote tumorigenesis in carcinoid tumors, which exhibit endogenously high levels of active, phosphorylated Akt. Therefore, we hypothesized that inhibition of the PI3K/Akt pathway would suppress carcinoid tumor cell growth and neuroendocrine (NE) marker production. Methods Human carcinoid BON cells were treated in vitro with LY294002, a PI3 kinase inhibitor, or transfected with Akt1 siRNA. Tumor cell proliferation was measured by MTT for six days. The effect of LY294002 or Akt1 siRNA treatment was assessed by western analysis. We examined the levels of phosphorylated Akt, total Akt, Akt1, and the NE markers human achaete-scute homolog1 (ASCL1) and chromogranin A (CgA). Results Treatment of BON cells with LY294002 reduced tumor cell proliferation (76%) in a dose-dependent manner. Growth also decreased in Akt1 siRNA transfected cells (29%). Levels of active, phosphorylated Akt and the NE tumor markers, ASCL1 and CgA, were diminished with both LY294002 and Akt1 siRNA treatments proportional to the degree of Akt inhibition. Total Akt, Akt2, and Akt3 levels were unaffected by these experiments. Conclusions These data indicate that PI3K/Akt signaling performs a critical role in human carcinoid tumor cell survival and NE hormone generation. Furthermore, the development of novel therapeutics targeting Akt1 or components of the PI3K/Akt pathway may enhance the management of carcinoid disease. Synopsis Carcinoid tumor cells were treated with a PI3K inhibitor, LY294002, and Akt1 siRNA to delineate the role of PI3K/Akt signaling in carcinoids. The effects of treatment on cellular proliferation and neuroendocrine marker expression were observed. PMID:19588205

  1. Polysialic acid of the neural cell adhesion molecule distinguishes small cell lung carcinoma from carcinoids.

    PubMed Central

    Komminoth, P.; Roth, J.; Lackie, P. M.; Bitter-Suermann, D.; Heitz, P. U.

    1991-01-01

    The neural cell adhesion molecule (NCAM) exists in various types of neuroendocrine cells and their tumors. A typical feature of NCAM is polysialic acid, of which the chain length is developmentally regulated. The authors have performed a comparative immunohistochemical study on small cell lung carcinomas and bronchial as well as gastrointestinal carcinoids with the monoclonal antibody (MAb) 735 reactive with the long-chain form of polysialic acid. The small cell lung carcinomas, irrespective of their histological type, were positive for polysialic acid. Metastatic tumor cell complexes also exhibited immunostaining. The tumor cell-surface-associated immunostaining for polysialic acid was sensitive to endoneuraminidase. The mature and atypical bronchial and gastrointestinal carcinoids were not immunoreactive for polysialic acid. Cytoplasmic staining in groups of cells of carcinoids (2 of 28 cases) was due to nonspecific antibody binding, which could be prevented by increased ion strength. These data indicate that neuroendocrine tumors of the lung can be distinguished by their content of highly sialylated NCAM. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:1651057

  2. Review of renal carcinoid tumor with focus on clinical and pathobiological aspects.

    PubMed

    Kuroda, N; Tanaka, A; Ohe, C; Mikami, S; Nagashima, Y; Inoue, K; Shuin, T; Taguchi, T; Tominaga, A; Alvarado-Cabrero, I; Petersson, F; Brunelli, M; Martignoni, G; Michal, M; Hes, O

    2013-01-01

    Renal carcinoid tumor is a rare neoplasm. In this article, we review this neoplasm with a focus on clinical and pathobiological aspects. The majority of patients present in the fourth to seventh decades, but there is no gender predilection. Clinically, patients with renal carcinoid tumor frequently present with abdominal, back or flank pain. This tumor is occassionally associated with horseshoe kidney and/or mature cystic teratoma located in the kidney. Macroscopically, these tumors are well demarcated with a lobulated appearance and yellow or tan-brown color cut surface. Microscopically, these tumors are composed of monomorphic round to polygonal cells with granular amphophilic to eosinophilic cytoplasm. Tumor cells are arranged in trabecular, ribbon-like, gyriform, insular, glandular and solid patterns. The nuclei are round to oval and with evenly distributed nuclear chromatin, frequently with a "salt and pepper"-pattern. Immunohistochemically, tumor cells demonstrate immuno-labeling for chromogranin A and synaptophysin. Ultrastructurally, the neoplastic cells contain abundant dense core neurosecretory granules. In previous genetic studies, abnormalities of chromosomes 3 or 13 have been reported. The clinical behavior of renal carcinoid tumors is variable, but is more indolent than most renal cell carcinomas. Further investigations are warranted in order to elucidate the critical genetic abnormalities responsible for the pathogenesis of this rare entity in renal neoplastic pathology.

  3. Acute appendicitis and carcinoid tumor in Meckel's diverticulum. Three pathologies in one: a case report.

    PubMed

    Mudatsakis, N; Paraskakis, S; Lasithiotakis, K; Andreadakis, E; Karatsis, P

    2011-10-01

    Incidental carcinoid tumor of the Meckel's diverticulum is an uncommon event. Herein, a case of a carcinoid tumor in Meckel's diverticulum that was incidentally found in a patient with acute appendicitis is presented. A 42-year-old Caucasian man presented with acute abdomen and clinical signs of acute appendicitis. A typical appendectomy was performed during which further abdominal exploration revealed a Meckel's diverticulum 60 cm proximal to the ileocecal valve, with an irregular and somewhat indurated serosal region on one side. A stapled diverticulectomy was performed. Pathology revealed an incidental carcinoid tumor measuring 1 cm within the Meckel's diverticulum. CT scan of the abdomen and 24-h urine 5-hydroxyindoleacetic acid results were normal. The patient had an uneventful recovery and was discharged at the 5th postoperative day. He is alive and without evidence of disease 23 months after the operation. Coexistence of acute appendicitis along with an incidental Meckel's diverticulum raises controversies in their surgical management. We discuss the issues in managing patients with two or more of these coexistent pathologies.

  4. Acute inflammatory bowel disease complicating chronic alcoholism and mimicking carcinoid syndrome.

    PubMed

    Ballo, Piercarlo; Dattolo, Pietro; Mangialavori, Giuseppe; Ferro, Giuseppe; Fusco, Francesca; Consalvo, Matteo; Chiodi, Leandro; Pizzarelli, Francesco; Zuppiroli, Alfredo

    2012-05-01

    We report the case of a woman with a history of chronic alcohol abuse who was hospitalized with diarrhea, severe hypokalemia refractory to potassium infusion, nausea, vomiting, abdominal pain, alternations of high blood pressure with phases of hypotension, irritability and increased urinary 5-hydroxyindoleacetic acid and cortisol. Although carcinoid syndrome was hypothesized, abdominal computed tomography and colonoscopy showed non-specific inflammatory bowel disease with severe colic wall thickening, and multiple colic biopsies confirmed non-specific inflammation with no evidence of carcinoid cells. During the following days diarrhea slowly decreased and the patient's condition progressively improved. One year after stopping alcohol consumption, the patient was asymptomatic and serum potassium was normal. Chronic alcohol exposure is known to have several deleterious effects on the intestinal mucosa and can favor and sustain local inflammation. Chronic alcohol intake may also be associated with high blood pressure, behavior disorders, abnormalities in blood pressure regulation with episodes of hypotension during hospitalization due to impaired baroreflex sensitivity in the context of an alcohol withdrawal syndrome, increased urinary 5-hydroxyindoleacetic acid as a result of malabsorption syndrome, and increased urinary cortisol as a result of hypothalamic-pituitary-adrenal axis dysregulation. These considerations, together with the regression of symptoms and normalization of potassium levels after stopping alcohol consumption, suggest the intriguing possibility of a alcohol-related acute inflammatory bowel disease mimicking carcinoid syndrome.

  5. Acute Inflammatory Bowel Disease Complicating Chronic Alcoholism and Mimicking Carcinoid Syndrome

    PubMed Central

    Ballo, Piercarlo; Dattolo, Pietro; Mangialavori, Giuseppe; Ferro, Giuseppe; Fusco, Francesca; Consalvo, Matteo; Chiodi, Leandro; Pizzarelli, Francesco; Zuppiroli, Alfredo

    2012-01-01

    We report the case of a woman with a history of chronic alcohol abuse who was hospitalized with diarrhea, severe hypokalemia refractory to potassium infusion, nausea, vomiting, abdominal pain, alternations of high blood pressure with phases of hypotension, irritability and increased urinary 5-hydroxyindoleacetic acid and cortisol. Although carcinoid syndrome was hypothesized, abdominal computed tomography and colonoscopy showed non-specific inflammatory bowel disease with severe colic wall thickening, and multiple colic biopsies confirmed non-specific inflammation with no evidence of carcinoid cells. During the following days diarrhea slowly decreased and the patient's condition progressively improved. One year after stopping alcohol consumption, the patient was asymptomatic and serum potassium was normal. Chronic alcohol exposure is known to have several deleterious effects on the intestinal mucosa and can favor and sustain local inflammation. Chronic alcohol intake may also be associated with high blood pressure, behavior disorders, abnormalities in blood pressure regulation with episodes of hypotension during hospitalization due to impaired baroreflex sensitivity in the context of an alcohol withdrawal syndrome, increased urinary 5-hydroxyindoleacetic acid as a result of malabsorption syndrome, and increased urinary cortisol as a result of hypothalamic-pituitary-adrenal axis dysregulation. These considerations, together with the regression of symptoms and normalization of potassium levels after stopping alcohol consumption, suggest the intriguing possibility of a alcohol-related acute inflammatory bowel disease mimicking carcinoid syndrome. PMID:22949895

  6. Ectopic ACTH secretion due to a bronchopulmonary carcinoid localized by somatostatin receptor scintigraphy.

    PubMed

    Iser, G; Pfohl, M; Dörr, U; Weiss, E M; Seif, F J

    1994-11-01

    We present the case of a 65-year-old woman with an adrenocorticotropic hormone (ACTH) secreting bronchopulmonary carcinoid. This patient showed the typical long history of Cushing's syndrome, including hypokaliemia, impaired glucose tolerance, high levels of ACTH and beta-endorphin, and coproduction of other peptides. At the onset of clinical symptoms in 1979 an adrenal adenoma was suspected, and left-sided adrenalectomy was performed. The symptoms soon recurred, and the diagnosis of ACTH-dependent Cushing's syndrome was made. As no ACTH-secreting tumor was found, the right adrenal was resected, and the patient was followed up regularly. Fourteen years later chest roentgenography and computed tomography revealed a para-aortic pulmonary lesion, which was suspicious for a bronchopulmonary carcinoid. ACTH and beta-endorphin were excessively, pancreatic polypeptide slightly elevated at that time. The final diagnosis was made using somatostatin receptor scintigraphy which confirmed the hormonal activity of the suspicious lesion; no additional focus was found. This method turned out to be not only a useful additional localization technique but also a promising tool for characterization and staging of a suspected ACTH-producing carcinoid. The tumor was resected curatively, and the diagnosis was confirmed histologically.

  7. Roentgenologic diagnosis of primary corticotropin-producing carcinoid tumors of the mediastinum

    SciTech Connect

    Brown, L.R.; Aughenbaugh, G.L.; Wick, M.R.; Baker, B.A.; Salassa, R.M.

    1982-01-01

    A study was undertaken of five patients with Cushing syndrome due to adrenocorticotropin (ACTH) production by thymic carcinoid tumor (primary mediastinal Apudomas), including two recent patients examined by computed tomography (CT) of the chest. Plain roentgenography of the chest initially failed to detect tumor in four of the five patients, while CT of the chest yielded definitive diagnostic information in both patients in whom it was employed. For one of these patients, a mediastinal tumor could be seen retrospectively on plain roentgenograms of the chest, although it had been missed on the first examination. One of the tumors appeared to be partially calcified on CT scan, a finding not previously reported. Blastic osseous metastasis, which is common when malignant carcinoid tumors spread to bone, was seen in one patient. Our data suggest that in patients with suspected ectopic ACTH production, CT scanning of the mediastinum should be performed early in order to avoid delay in diagnosis of an ACTH-secreting carcinoid tumor of the mediastinum.

  8. Carcinoid tumour of the thymus with systemic manifestations: a radiological and pathological study

    PubMed Central

    Lowenthal, R. M.; Gumpel, J. M.; Kreel, L.; McLaughlin, J. E.; Skeggs, D. B. L.

    1974-01-01

    Lowenthal, R. M., Gumpel, J. M., Kreel, L., McLaughlin, J. E., and Skeggs. D. B. L. (1974).Thorax, 29, 553-558. Carcinoid tumour of the thymus with systemic manifestations: a radiological and pathological study. Following recent reports of an unusual mediastinal tumour described as `mediastinal endocrine neoplasm of probable thymic origin, related to carcinoid tumour' (Rosai and Higa, 1972), a further case, in a 72-year-old man, has been studied. Polyarthropathy was the presenting feature, and the patient also had clubbing of the fingers and clinical evidence of a probable proximal myopathy and a peripheral neuropathy. These non-metastatic systemic manifestations have not previously been described with this type of tumour. By retrograde thymic venography the tumour was conclusively shown to be arising in the thymus, as had been suspected but not proven in previously described cases. Histologically the typical picture, including areas of cells with true rosette formation and cells with argyrophil granules, was seen. In contrast to the more usual thymic tumours, this type of neoplasm responds poorly to radiotherapy, but surgical treatment may give good results. Therefore, in a situation where the possibility of carcinoid tumour of the thymus exists, it is imperative that tissue be obtained for histological diagnosis before any therapeutic decision is made. Images PMID:4428455

  9. Cardiac Surgery for Carcinoid Heart Disease: A Weapon Not to Be Misused.

    PubMed

    Bonou, Maria; Kapelios, Chris J; Kaltsas, Gregory; Perreas, Konstantinos; Toutouzas, Konstantinos; Barbetseas, John

    2017-01-01

    Carcinoid heart disease (CHD) complicates approximately 25% of patients with a carcinoid tumor and carcinoid syndrome and leads to heart valve degeneration with mixed-stenotic and regurgitation pathology and consequent heart failure (HF) leading to significant morbidity and mortality. Cardiac surgery in symptomatic, severe CHD leads to significantly better functional capacity and prolonged survival when compared to medical treatment alone. Recent studies have shown improvement in postoperative outcomes of patients undergoing surgery for CHD over the last decades. The trend for early diagnosis and application of surgery prior to the manifestation of HF symptoms, which tended to develop during the previous years, does not seem justifiable based on the findings of recent studies. Therefore, the optimal timing of intervention in CHD and the type of valve that should preferably be used remain issues of controversy. This review comprehensively examines the existing literature on the treatment options for patients with CHD, with a special focus on short- and long-term survival after cardiac surgery, and discusses the selection of the exact patient profile and intervention timing that are more likely to optimize the benefit-to-risk ratio for surgical intervention.

  10. Analysis of the molecular features of rectal carcinoid tumors to identify new biomarkers that predict biological malignancy

    PubMed Central

    Ito, Miki; Igarashi, Hisayoshi; Ishigami, Keisuke; Sukawa, Yasutaka; Tachibana, Mami; Takahashi, Hiroaki; Tokino, Takashi; Maruyama, Reo; Suzuki, Hiromu; Imai, Kohzoh; Shinomura, Yasuhisa

    2015-01-01

    Although gastrointestinal carcinoid tumors are relatively rare in the digestive tract, a quarter of them are present in the rectum. In the absence of specific tumor biomarkers, lymphatic or vascular invasion is generally used to predict the risk of lymph node metastasis. We, therefore, examined the genetic and epigenetic alterations potentially associated with lymphovascular invasion among 56 patients with rectal carcinoid tumors. We also conducted a microRNA (miRNA) array analysis. Our analysis failed to detect mutations in BRAF, KRAS, NRAS, or PIK3CA or any microsatellite instability (MSI); however, we did observe CpG island methylator phenotype (CIMP) positivity in 13% (7/56) of the carcinoid tumors. The CIMP-positive status was significantly correlated with lymphovascular invasion (P = 0.036). The array analysis revealed that microRNA-885 (miR-885)-5p was the most up-regulated miRNA in the carcinoid tumors with lymphovascular invasion compared with that in those without invasion. In addition, high miR-885-5p expression was independently associated with lymphovascular invasion (P = 0.0002). In conclusion, our findings suggest that miR-885-5p and CIMP status may be useful biomarkers for predicting biological malignancy in patients with rectal carcinoid tumors. PMID:26090613

  11. Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement.

    PubMed

    Davar, Joseph; Connolly, Heidi M; Caplin, Martyn E; Pavel, Marianne; Zacks, Jerome; Bhattacharyya, Sanjeev; Cuthbertson, Daniel J; Dobson, Rebecca; Grozinsky-Glasberg, Simona; Steeds, Richard P; Dreyfus, Giles; Pellikka, Patricia A; Toumpanakis, Christos

    2017-03-14

    Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is responsible for substantial morbidity and mortality. The pathophysiology of carcinoid heart disease is poorly understood; however, chronic exposure to excessive circulating serotonin is considered one of the most important contributing factors. Despite recognition, international consensus guidelines specifically addressing the diagnosis and management of carcinoid heart disease are lacking. Furthermore, there is considerable variation in multiple aspects of screening and management of the disease. The aim of these guidelines was to provide succinct, practical advice on the diagnosis and management of carcinoid heart disease as well as its surveillance. Recommendations and proposed algorithms for the investigation, screening, and management have been developed based on an evidence-based review of the published data and on the expert opinion of a multidisciplinary consensus panel consisting of neuroendocrine tumor experts, including oncologists, gastroenterologists, and endocrinologists, in conjunction with cardiologists and cardiothoracic surgeons. Copyright © 2017 American College of Cardiology Foundation. All rights reserved.

  12. Gastric carcinoid tumor in a patient with a past history of gastrointestinal stromal tumor of the stomach

    PubMed Central

    Hung, Chien-Yuan; Chen, Ming-Jen; Shih, Shou-Chuan; Liu, Tsang-Pai; Chan, Yu-Jan; Wang, Tsang-En; Chang, Wen-Hsiung

    2008-01-01

    Gastrointestinal stromal tumor is the most common mesenchymal tumor in the gastrointestinal tract. It may coexist with other type of cancers, and if so, the tumors usually involve the stomach. The most common associated cancers are gastrointestinal carcinomas. We report a 65-year-old woman with a history of gastric gastrointestinal stromal tumor who had undergone subtotal segmental gastrectomy. New polypoid lesions were detected on a follow-up gastroscopy one year later. The lesions were biopsied and found to be carcinoid tumors. There was serum hypergastrinemia, and type 1 gastric carcinoid tumor was diagnosed. A total gastrectomy was performed. Pathologic examination revealed both carcinoid tumors and a recurrent gastrointestinal stromal tumor. PMID:19058321

  13. Composite renal cell carcinoma with clear cell renal cell carcinomatous and carcinoid tumoral elements: a first case report.

    PubMed

    Bressenot, A; Delaunay, C; Gauchotte, G; Oliver, A; Boudrant, G; Montagne, K

    2010-02-01

    Renal endocrine tumours are extremely rare, and carcinoid tumoral elements in renal cell carcinoma have never been reported. This is the first report of a composite renal cell carcinoma containing a clear cell renal cell carcinoma associated with carcinoid tumoral elements, in a patient with synchronous metastatic disease. In the absence of specific radiological and clinical manifestations, typical morphological features as well as an immunostaining profile of neuroendocrine differentiation were identified by microscopy. Secondary nodal and liver localisations were characterised by carcinoid elements only. Despite antiangiogenic therapy, liver metastasis progressed, suggesting that adjuvant therapy cannot be based on the presence of the clear cell renal cell carcinoma component. In this context, extensive tissue sampling is recommended to reveal the endocrine component that is the most aggressive element of such a composite carcinoma.

  14. Limited additive value of the Ki-67 proliferative index on patient survival in World Health Organization-classified pulmonary carcinoids.

    PubMed

    Swarts, Dorian R A; Rudelius, Martina; Claessen, Sandra M H; Cleutjens, Jack P; Seidl, Stefan; Volante, Marco; Ramaekers, Frans C S; Speel, Ernst J M

    2017-02-01

    Currently pulmonary carcinoids are separated into typical and atypical based on mitotic count and presence of necrosis, according to the World Health Organization. At variance with gastroenteropancreatic neuroendocrine tumours, which are graded based on mitotic count and Ki-67 proliferative index, the use of Ki-67 for grading pulmonary carcinoids is still under debate. In this study we evaluated the prognostic impact of Ki-67 assessment in a multicentre cohort of 201 carcinoids [147 typical carcinoids (TCs) and 54 atypical carcinoids (ACs)] using manual analysis (2000 cells counted) and digital image analysis (in-house Leica Qwin program; ≥4500 cells counted). The Ki-67 proliferative index was correlated with overall survival by means of univariate analysis and in comparison to clinical data by means of multivariable analysis. The Ki-67 index was significantly higher in ACs than in TCs for both counting methods (P ≤ 2.7e(-5) ). In addition, using cut-offs of 2.5% and 4% (manual counting) or 1% and 5% (digital analysis), the highest differences in overall survival were observed (P ≤ 0.0067). Nevertheless, histopathological classification into TCs and ACs showed an equally strong association with disease outcome, although Ki-67 had some additive value within TCs. Ki-67 index was not an independent predictor of survival in multivariable analysis. Our study demonstrates that, although Ki-67 is a strong prognostic factor for pulmonary carcinoids, its usefulness in addition to histopathology in prediction of prognosis is limited. None the less, it may have additional value, especially in cases that are difficult to classify, in combination with histopathology and other molecular markers. © 2016 John Wiley & Sons Ltd.

  15. [Bronchial carcinoid tumor and scintigraphy of somatostatin receptors: detection of bone metastasis].

    PubMed

    Banzo, J; Abós, M D; Prats, E; García, F; Freile, E; Razola, P; Escalera, T

    2001-10-01

    Surgery is the treatment of choice for bronchial carcinoid tumor (BCT), whenever the staging is adequate. There is little information about the capability of the somatostatin receptor scintigraphy (SRS) to detect bone metastases in the carcinoid tumor. This work has aimed to evaluate retrospectively the diagnostic accuracy of the SRS in the detection of bone metastases in BCT. Based on their clinical indication, the patients were classified into two different groups: Group A (n = 4), staging of a known BCT; and Group B (n = 6), treatment control. The SRS results could be correlated with the CT results in all 4 patients from the group A, and in one patient from the group B, and the SRS results were compared with the clinical follow up during at least one year in the other 5 patients. The SRS scan detected the 4 BCT from the group A; in 2 of them the patient staging was superior when the SRS was used than with the CT, whereas the scan overestimated the tumor stage (BCT + sarcoidosis) in another patient. During the clinical course, one of these patients developed bone and liver metastases. The SRS was normal in 5 asymptomatic patients from group B, whereas the scan showed disseminated metastatic disease (liver, bone, spleen and lymph nodes) in another patient. In the 2 patients with bone metastases, the total number of bone metastases detected by the bone scan was 12, and by the SRS 8. The four lesions that were not detected by SRS were located in the ribs (n = 3) and 12-D (n = 1). The capability of the SRS to detect bone metastases makes it more useful in BCT staging. Over the next few years, the role of the bone scan and SRS in the detection of bone metastases in carcinoid tumors needs to be established.

  16. Association of autoimmune thyroid diseases, chronic atrophic gastritis and gastric carcinoid: experience from a single institution.

    PubMed

    Castoro, C; Le Moli, R; Arpi, M L; Tavarelli, M; Sapuppo, G; Frittitta, L; Squatrito, S; Pellegriti, G

    2016-07-01

    Autoimmune polyendocrine syndromes (APS) type III are characterized by the association of autoimmune thyroid disease (ATD) with other autoimmune diseases such as diabetes, alopecia, pernicious anemia, vitiligo and chronic atrophic gastritis. A strong association between ATD and atrophic gastritis (AG) has been demonstrated. Moreover 10 % of patients affected by AG have a predisposition to develop gastric carcinoid and adenocarcinoma as a result of chronic hypergastrinemia caused by achlorhydria and subsequent ELC cells neoplastic transformation. The aim of the study is to evaluate, in a consecutive series of patients followed for ATD in our outpatients clinic, the prevalence of AG. In the period 2004-2014, 242 patients with ATD underwent a screening performing APCA, Vitamin B12, ferritin, iron, and hemoglobin and red cells count measurements with subsequent gastroscopy in case of APCA positivity. We found 57/242 (23.5 %) patients with APCA positivity. Of these patients 33/57 (57.8 %), 31 F and 2 M, were affected by Graves disease; 24/57 (42.1 %) 21 F and 3 M by Hashimoto thyroiditis; 10/57 (17.5 %) presented with anemia, 14/57 (24.5 %) with vitamin B12 deficiency, 9/57 (15.7 %) with iron deficiency. In 2/57 a gastric carcinoid was found. Our data confirm the high association rate of AG in ATD which frequently is not an isolated disease but configure the picture of APS type III and need to be followed accordingly. An early diagnosis may be useful for diagnosis of gastric carcinoids and to explain and treat a gastric related L-thyroxine malabsorption and presence of chronic unexplained anemia.

  17. Bronchopulmonary Carcinoids causing Cushing Syndrome: Results from a Multicentric Study Suggesting a More Aggressive Behavior.

    PubMed

    Lococo, Filippo; Margaritora, Stefano; Cardillo, Giuseppe; Filosso, Perluigi; Novellis, Pierluigi; Rapicetta, Cristian; Carleo, Francesco; Bora, Giulia; Cesario, Alfredo; Stefani, Alessandro; Rossi, Giulio; Paci, Massimiliano

    2016-03-01

    Cushing syndrome (CS) caused by bronchopulmonary carcinoids (BCs) is a very rare entity. The aim of this study was to revisit the features of a multicenter clinical series to identify significant prognostic factors. From January 2002 to December 2013, the clinical and pathological data of 23 patients (treated in five different institutions) were retrospectively reviewed. Survival analysis was performed to explore the relative weight of potential prognostic factors. Median age and male/female ratio were 48 years and 14/9, respectively. Most (> 80%) of the patients presented with CS-related symptoms at diagnosis. Tumor location was peripheral in 13 patients (57%) and central in 10 (43%). All patients but two (treated with chemotherapy) underwent surgical resection with curative intent. Definitive cyto/histology was indicative of typical carcinoid (TC) in 16 cases (70%) and atypical carcinoid (AC) in 7 cases (30%). A complete remission of CS was obtained in 16 cases (70%). Lymph nodal involvement was detected in 11 cases (48%), with N2 disease occurring in 7 (∼ 30% of all cases). Four patients (22%) experienced a relapse of the disease after radical surgery. Overall 5-year survival (long-term survival, LTS) was 60%, better in TCs when compared with AC (LTS: 66 v s. 48%, p = 0.28). Log-rank analysis identified ECOG performance status, cTNM and cN staging, pTNM and pN staging, persistence of CS and relapses (local p = 0.006; distant p = 0.001) as significant prognostic factors in this cohort of patients. BCs causing CS are characterized by a high rate of lymph-nodal involvement, a suboptimal prognosis (5-year survival = 60%, 66% in TCs) and a remarkable risk of relapse even after radical resection. Advanced stage, lymph-nodal involvement and the persisting of the CS after treatment correlate with a poor prognosis. Georg Thieme Verlag KG Stuttgart · New York.

  18. Diagnosis and treatment of a carcinoid tumor using iodine-131 meta-iodobenzylguanidine

    SciTech Connect

    Hoefnagel, C.A.; Den Hartog Jager, F.C.; Van Gennip, A.H.; Marcuse, H.R.; Taal, B.G.

    1986-03-01

    Scintigraphy using I-131 meta-iodobenzylguanidine has been introduced as an effective method to detect pheochromocytomas and neuroblastomas, and the radiopharmaceutical also is applied in therapy of these tumors. The authors present a case of a metastatic gastric carcinoid tumor, another neural crest tumor, concentrating I-131 MIBG, which was documented by conventional scintigraphy and SPECT in correlation with CT scans and colloid scintigrams of the liver. Two therapeutic attempts in this patient, using I-131 MIBG, are described. The metabolic basis of this phenomenon is discussed, and the importance of I-131 MIBG imaging in the detection of neural crest tumors is underlined.

  19. A Rare Combination of Ovarian and Uterine Leiomyomas with Goblet Cell Carcinoid of the Appendix

    PubMed Central

    Al-Shaikh, Abdulrahman F.; Darwish, Abdulla; Nagaraj, Veena; Alsada, Abeer

    2015-01-01

    We present a case of the rare combination of unilateral ovarian leiomyoma, uterine leiomyoma, and goblet cell carcinoid tumor of the appendix in a premenopausal woman who presented with right iliac pain. Immunohistochemistry study for desmin (muscle marker) and chromogranin and synaptophysin (neuroendocrine markers) confirmed immunophenotyping origin. Interestingly, both tumors showed positive reaction for estrogen receptor. To our knowledge, such a combination has not been reported previously in the literature. In this paper, the pathogenesis and differential diagnosis of both types of tumors are discussed. PMID:25685587

  20. A Prospective Study of Gastric Carcinoids and Enterochromaffin-Like Cell Changes in Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome: Identification of Risk Factors

    PubMed Central

    Berna, Marc J.; Annibale, Bruno; Marignani, Massimo; Luong, Tu Vinh; Corleto, Vito; Pace, Andrea; Ito, Tetsuhide; Liewehr, David; Venzon, David J.; Delle Fave, Gianfranco; Bordi, Cesare; Jensen, Robert T.

    2008-01-01

    Context: Multiple endocrine neoplasia type 1 (MEN1) patients frequently develop Zollinger-Ellison syndrome (ZES). These patients can develop proliferative changes of gastric enterochromaffin-like (ECL) cells and gastric carcinoids (ECL-cell tumors). ECL-cell changes have been extensively studied in sporadic ZES patients and can be precursor lesions of gastric carcinoids, but little is known about factors influencing their severity or development of carcinoids in MEN1/ZES patients. Objectives: Our objective was to prospectively analyze ECL-cell changes and gastric carcinoids (ECL-cell tumors) in a large series of MEN1/ZES patients to detect risk factors and deduct clinical guidelines. Setting and Patients: Fifty-seven consecutive MEN1/ZES patients participated in this prospective study at two tertiary-care research centers. Interventions and Outcome Measures: Assessment of MEN1, gastric hypersecretion, and gastroscopy with multiple biopsies was done according to a fixed protocol and tumor status. ECL-cell changes and α-human chorionic gonadotropin staining were assessed in each biopsy and correlated with clinical, laboratory, and MEN1 features. Results: ECL-cell proliferative changes were universally present, advanced changes in 53% and carcinoids in 23%. Gastric nodules are common and are frequently associated with carcinoids. Patients with high fasting serum gastrin levels, long disease duration, or a strong α-human chorionic gonadotropin staining in a biopsy are at higher risk for an advanced ECL-cell lesion and/or gastric carcinoid. Conclusions: Gastric carcinoids and/or advanced ECL-cell changes are frequent in MEN1/ZES patients, and therefore, regular surveillance gastroscopy with multiple routine biopsies and biopsies of all mucosal lesions are essential. Clinical/laboratory data and biopsy results can be used to identify a subgroup of MEN1/ZES patients with a significantly increased risk for developing gastric carcinoids, allowing development of better

  1. Transcatheter arterial embolization with trisacryl gelatin microspheres (Embosphere®) leads to life-threatening tumor lysis syndrome in a rectal carcinoid patient with hepatic metastases

    PubMed Central

    Lo, Yuan-Hao; Tsai, Ming-Tsun; Kuo, Chen-Yu; Liu, Wen-Sheng; Lee, Rheun-Chuan; Yeh, Yi-Chen; Li, Chung-Pin; Chen, Jinn-Yang; Chao, Yee

    2012-01-01

    The incidence of gastrointestinal carcinoids appears to be increasing, and the rectum is the third most common location. Transcatheter arterial embolization (TAE) with trisacryl gelatin microspheres (Embosphere®) has been reported as an effective method for hepatic metastases of rectal carcinoids. Complications are uncommon and usually of minor consequence. We report an unusual case of a 34-year-old man with tumor lysis syndrome following TAE with Embosphere® in a patient with multiple hepatic metastases of a rectal carcinoid. Early detection and effective treatment are essential for this rare but potentially catastrophic complication. PMID:23986828

  2. [Unsuspected bronchial carcinoid tumor detected in a somatostatin receptor scintigraphy in a patient with multiple endocrine neoplasia syndrome type 1 and hypergastrinemia].

    PubMed

    Marín-Oyaga, V; Tirado-Hospital, J L; Cuenca-Cuenca, J I; Guerrero-Vázquez, R; Luján-Rodríguez, D; Vázquez-Albertino, R

    2013-03-01

    Multiple Endocrine Neoplasia type 1 syndrome (MEN1) is characterized by the presence of tumors in parathyroid glands, anterior pituitary gland, endocrine pancreas and duodenum. However, other tumors may also occur. One of them is the carcinoid tumor, which in this context, is more common in the gastrointestinal tract. Less common is the presence of carcinoid tumors of bronchial origin, which with histologic confirmation, may occur in 5-8% of cases and that appears more frequently in patients with hypergastrinemia. We report a patient with MEN1 syndrome, hypergastrinemia and an incidental finding in a somatostatin receptor scintigraphy of an unsuspected bronchial carcinoid tumor that was confirmed histologically.

  3. Oncocytic carcinoid tumor of the lung with intense F-18 fluorodeoxyglucose (FDG) uptake in positron emission tomography-computed tomography (PET/CT).

    PubMed

    Tanabe, Yuki; Sugawara, Yoshifumi; Nishimura, Rieko; Hosokawa, Kohei; Kajihara, Makoto; Shimizu, Teruhiko; Takahashi, Tadaaki; Sakai, Shinya; Sawada, Shigeki; Yamashita, Motohiro; Ohtani, Haruhiko

    2013-10-01

    The present report describes a case of typical carcinoid tumor with intense fluorodeoxyglucose (FDG) uptake. The most of tumor cells were characterized by eosinophilic cytoplasm resulting from accumulation of mitochondria, which was called an oncocytic carcinoid tumor. Glucose transporter type 1 (GLUT-1) was expressed in a membranous pattern in the oncocytic component. Oncocytic carcinoid tumors could show intense FDG uptake due to the numerous intracellular mitochondria and the membranous overexpression of GLUT-1. Thus, it could be a potential pitfall of interpreting FDG-PET/CT image.

  4. Demonstration of TGF-β and XIIIα in Endocardial Biopsies of Carcinoid Heart Disease Patients: an Immunofluorescence Study

    PubMed Central

    Diepholz, Dorgrit; Wilke, Andreas; Maisch, Bernhard; Steverding, Dietmar

    2011-01-01

    Background Serotonin and other vasoactive substances play a critical role in the development of carcinoid heart disease, but the exact etiology of the illness is still unknown. Methods By using immunofluorescence microscopy, we investigated the expression of transforming growth factor-β (TGF-β) and the presence of fibrin-stabilizing factor (XIIIα) in endomyocardial biopsy specimens of patients with carcinoid heart disease. In addition, the tissue integrity of the specimens was studied by staining for laminin. Results Both TGF-β and XIIIα co-localized in the endocardium beneath carcinoid plaques: while TGF-β was found within myocytes, XIIIα was detected on the surface of cells in fibrotic lesions stretching out into the tissue. Laminin staining revealed that the integrity of the endocardium was dissolved and that the tissue consisted of hypertrophic and hypotrophic myocytes. Conclusions The results suggest that the presence of TGF-β and XIIIα in carcinoid heart lesions indicates that endocardial damage induced by serotonin and other vasoactive substances gives rise to an overshooting wound healing process.

  5. Glucagon-, glicentin-, and pancreatic polypeptide-like immunoreativities in rectal carcinoids and related colorectal cells.

    PubMed Central

    Fiocca, R.; Capella, C.; Buffa, R.; Fontana, R.; Solcia, E.; Hage, E.; Chance, R. E.; Moody, A. J.

    1980-01-01

    Three nonargentaffin rectal carcinoids have been investigated immunohistochemically. In one case most tumor cells reacted with antiglucagon sera as well as with antiglicentin, antibovine pancreatic polypeptide (BPP), and antihuman pancreatic polypeptide (HPP) sera; they were identified ultrastructurally as L cells. Another case showed glucagon-, glicentin-, and BPP-immunoreactive cells but lacked HPP immunoreactivity. In the third case glucagon- and glicentin-immunoreactive cells were well represented, while PP immunoreactivities were scarce. Parallel investigations of human rectal and sigmoid mucosa showed numerous cells reacting with glucagon, glicentin, and BPP antisera, most of which lacked HPP immunoreactivity. Cells reacting with glucagon and glicentin antisera, while lacking PP immunoreactivities, were also found. Thus, both tumor and nontumor cells produce glucagonlike immunoreactive (GLI) peptides--one of which may be glicentin or a related molecule--as well as PP-related sequences, although differing histochemically and ultrastructurally from glucagon or PP cells of the human pancreas. It is concluded that nonargentaffin rectal carcinoids are histogenetically linked to nonargentaffin endocrine cells of the human rectum. Images p[92]-a Figures 1-3 Figure 4 Figure 5 PMID:7395969

  6. Atypical thymic carcinoid manifesting with nephrotic-range proteinuria in a 7-year-old boy.

    PubMed

    Soltysiak, Jolanta; Ostalska-Nowicka, Danuta; Zaorska, Katarzyna; Sterzyńska, Karolina; Derwich, Katarzyna; Zachwieja, Jacek

    2017-08-01

    Nephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria. We present the case of a 7-year-old boy with an extremely rare atypical thymic carcinoid accompanied by nephrotic-range proteinuria as PNS. The kidney biopsy was consistent with minimal change disease (MCD). Tests for a neuroendocrine tumor were performed due to symptoms of hypercortisolemia and an elevated concentration of chromogranin A in the serum. The chest computed tomography revealed a tumor in the anterior mediastinum, which was diagnosed as an atypical thymic carcinoid. A complete resolution of the nephrotic-range proteinuria was observed within 1 week after the first thoracoscopic surgery, with almost complete reduction of the tumor mass. This extremely rare case shows that MCD can occur as a PNS even in children. Nephrotic-range proteinuria can be a symptom of malignant solid tumor. This case highlights the possibility of secondary causes of MCD in children.

  7. Primary carcinoid tumor in a retroperitoneal mature teratoma: report of a case.

    PubMed

    Shindo, Koji; Ueda, Junji; Toubo, Taro; Nakamura, Masafumi; Oda, Yoshinao; Eguchi, Toru; Tanaka, Masao

    2013-06-01

    Primary retroperitoneal teratoma in an adult is rare, as is the occurrence of a malignant tumor within a mature teratoma, known as "malignant transformation". A 24-year-old woman was admitted to our hospital for investigation of an abdominal mass. Computed tomography and magnetic resonance imaging revealed a multilocular mass in the right upper abdomen. The tumor consisted of fat, soft tissue, and bone, with a slightly enhanced solid component. The tumor was diagnosed preoperatively as a retroperitoneal mature teratoma with an immature component, and excised. Histologically, it was composed mainly of mature fat, soft tissue, and bone, accompanied by a solid component of prostate-like tissue. In addition, a latent carcinoid tumor was recognized in the middle of the tumor. The tumor was finally diagnosed as a primary carcinoid tumor within the retroperitoneal mature teratoma. The patient has been followed-up for 24 months since her operation without any evidence of recurrence. We report this case to highlight the possibility of malignant transformation in adult retroperitoneal teratoma, even when the preoperative diagnosis is benign mature teratoma.

  8. Primary renal carcinoid tumour with lung metastasis misdiagnosed as renal cell carcinoma.

    PubMed

    Daboul, Nour; Monga, Dulabh; Bunker, Mark

    2016-03-07

    A 58-year-old man with a history of metastatic renal cell carcinoma (RCC) diagnosed 10 years prior, status post right nephrectomy, presented for evaluation of pulmonary nodules. A year after the nephrectomy, he had undergone cutaneous metastasectomy in the right flank area, and a further 2 years later he had had his second cutaneous metastasectomy in the right chest wall. Both cutaneous pathologies had, at the time, shown metastatic neoplasm with histological features compatible with those of the previous renal tumour. He was treated with sorafenib. 3 years later he developed asymptomatic pulmonary nodules, which gradually doubled in size over the next 2.5 years. He underwent bronchoscopy and left lower lobe biopsy. Pathology revealed a metastatic renal carcinoid/neuroendocrine tumour. Second review of the previous renal neoplasm and the cutaneous metastatic pathology showed trabecular architecture, consistent with carcinoid, but mimicking the long parallel arrays that have been described in some cases of papillary RCC. 2016 BMJ Publishing Group Ltd.

  9. Update in Cancer Chemotherapy: Gastrointestinal Cancer, Cancer of the Stomach and Carcinoid Tumors

    PubMed Central

    Wright, Jane C.

    1986-01-01

    Cancer chemotherapy in the treatment of advanced gastric cancer provides only palliation with perhaps increased survival time in some patients. The primary treatment of gastric carcinomas is surgical, as this is the only hope for cure. It is estimated that 80 to 85 percent of patients with newly diagnosed cases of stomach cancer will be dead of their disease within five years. Radiation therapy alone is seldom employed, except as a palliative measure to control hemorrhage or pain. There are no data to suggest that postoperative radiation increases survival rates. Single-agent chemotherapy is of temporary palliative value in 20 to 30 percent of cases with a duration of response from three to five months. Combination chemotherapy has shown a somewhat higher response rate than single-agent therapy. In advanced gastric cancer, there is no evidence of improved long-term disease-free survival rates with any combination yet reported. The treatment of carcinoid cancer of the intestinal tract is surgical removal, as this offers the only hope of cure. Radiation therapy is of little benefit, except for moderate palliation in cases of extensive liver metasasis. Carcinoid cancers are moderately sensitive to chemotherapy. While some adjuvant chemotherapy trials suggest improvement, major survival gains remain to be demonstrated. Uncertainty as to the role of chemotherapy in the treatment of gastrointestinal cancers is probably due to lack of data. PMID:3528508

  10. Carcinoid Syndrome and Costs of Care During the First Year After Diagnosis of Neuroendocrine Tumors Among Elderly Patients.

    PubMed

    Shen, Chan; Chu, Yiyi; Halperin, Daniel M; Dasari, Arvind; Zhou, Shouhao; Xu, Ying; Yao, James C; Shih, Ya-Chen Tina

    2017-06-22

    Neuroendocrine tumors (NETs) can secrete hormonal peptides that lead to additional symptom burdens. However, it is largely unknown whether and to what extent the additional symptom burdens translate into higher costs of care. This study aimed to examine the cost pattern of elderly NET patients during the first year of diagnosis, taking into account of the carcinoid syndrome status. We used Surveillance, Epidemiology, and End Results Medicare data to identify elderly NET patients diagnosed between January 2003 and December 2011. Patients who had at least two claims indicative of carcinoid syndrome during the 3 months before and after the NET diagnosis were considered to have carcinoid syndrome. We adopted a payer's perspective and quantified economic outcomes using the following three measures: (a) total Medicare reimbursement amount, (b) inpatient amount, and (c) outpatient amount. We used a generalized linear model (GLM) to examine the association between syndrome and costs. Our study cohort included 6,749 elderly NET well-differentiated and moderately differentiated patients. Of these patients, 5,633 (83%) were alive 1 year after diagnosis with continuous enrollment, and 1,116 (17%) died within 1 year. The multivariable GLM showed significant association between the syndrome and higher total, inpatient, and outpatient costs among the group who survived the whole year; the association was insignificant among the group who died within the first year of diagnosis. This population-based study showed that NET patients with carcinoid syndrome incurred higher costs of care especially among those who survived the first year of diagnosis. The Oncologist 2017;22:1-13 IMPLICATIONS FOR PRACTICE: This is the first population-based study that examines the health care costs associated with carcinoid syndrome among neuroendocrine tumor patients. Among patients alive throughout the first year, the unadjusted analyses showed that total median monthly costs were above $1,000 higher

  11. Carcinoid tumor of the duodenum: a rare tumor at an unusual site. Case series from a single institution.

    PubMed

    Waisberg, Jaques; Joppert-Netto, George; Vasconcellos, Cidia; Sartini, Gustavo Henrique; Miranda, Lucimar Sonja Villela de; Franco, Maria Isete Fares

    2013-01-01

    Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. Twenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed. The most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months). Twelve (60%) of the 20 cases in this series are alive without any evidence of active disease. Only one (5%) patient died due to

  12. Combination of carbonic anhydrase inhibitor, acetazolamide, and sulforaphane, reduces the viability and growth of bronchial carcinoid cell lines

    PubMed Central

    2013-01-01

    Background Bronchial carcinoids are pulmonary neuroendocrine cell-derived tumors comprising typical (TC) and atypical (AC) malignant phenotypes. The 5-year survival rate in metastatic carcinoid, despite multiple current therapies, is 14-25%. Hence, we are testing novel therapies that can affect the proliferation and survival of bronchial carcinoids. Methods In vitro studies were used for the dose–response (AlamarBlue) effects of acetazolamide (AZ) and sulforaphane (SFN) on clonogenicity, serotonin-induced growth effect and serotonin content (LC-MS) on H-727 (TC) and H-720 (AC) bronchial carcinoid cell lines and their derived NOD/SCID mice subcutaneous xenografts. Tumor ultra structure was studied by electron microscopy. Invasive fraction of the tumors was determined by matrigel invasion assay. Immunohistochemistry was conducted to study the effect of treatment(s) on proliferation (Ki67, phospho histone-H3) and neuroendocrine phenotype (chromogranin-A, tryptophan hydroxylase). Results Both compounds significantly reduced cell viability and colony formation in a dose-dependent manner (0–80 μM, 48 hours and 7 days) in H-727 and H-720 cell lines. Treatment of H-727 and H-720 subcutaneous xenografts in NOD/SCID mice with the combination of AZ + SFN for two weeks demonstrated highly significant growth inhibition and reduction of 5-HT content and reduced the invasive capacity of H-727 tumor cells. In terms of the tumor ultra structure, a marked reduction in secretory vesicles correlated with the decrease in 5-HT content. Conclusions The combination of AZ and SFN was more effective than either single agent. Since the effective doses are well within clinical range and bioavailability, our results suggest a potential new therapeutic strategy for the treatment of bronchial carcinoids. PMID:23927827

  13. Variation in Cardiac Screening and Management of Carcinoid Heart Disease in the UK and Republic of Ireland.

    PubMed

    Dobson, R; Valle, J W; Burgess, M I; Poston, G J; Cuthbertson, D J

    2015-12-01

    Screening for carcinoid heart disease is an important, yet frequently neglected aspect of the management of patients with neuroendocrine tumours (NETs). Screening is advocated in international guidelines, although recommendations on the modality and frequency are poorly defined. We mapped current practice for the screening and management of carcinoid heart disease in specialist NET centres throughout the UK and Republic of Ireland. Thirty-five NET centres were invited to complete an online questionnaire outlining the size of NET service, patient selection criteria for carcinoid heart disease screening and the modality and frequency of screening. Twenty-eight centres responded (80%), representing over 5500 patients. Eleven per cent of centres screen all patients with any NET, 14% screen only patients with midgut NETs, 32% screen all patients with liver metastases and/or carcinoid syndrome and 43% screen all patients with evidence of syndrome or raised urinary/serum/plasma 5-hydroxyindoleacetic acid (5HIAA). The mode of screening included clinical examination, echocardiography and biomarker measurement: 89% of centres carry out echocardiography, ranging from at initial presentation only (24%), periodically without clearly defined intervals (28%), annually (36%) or less than annually (12%); three centres use a scoring system to report their echocardiograms. Fifty per cent of centres utilise biomarkers for screening (chromogranins, plasma/urinary 5HIAA or most commonly N-terminal pro-brain natriuretic peptide) at varying time intervals. There is considerable heterogeneity across the UK and Ireland in multiple aspects of screening and management of carcinoid heart disease. Copyright © 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

  14. Dual Tracer PET Imaging (68Ga-DOTATATE and 18F-FDG) Features in Pulmonary Carcinoid: Correlation with Tumor Proliferation Index

    PubMed Central

    Bhatkar, Dhiraj; Utpat, Ketaki; Basu, Sandip; Joshi, Jyotsna M.

    2017-01-01

    Pulmonary carcinoid tumors are rare group of lung neoplasms representing 1% of all the lung tumors. The typical bronchial carcinoids showed higher and more selective uptake of 68Ga-DOTATATE than of 18F-FDG on PET-CT. The Ki-67(MIB-1), a tumor proliferation index is a prognostic marker in neuroendocrine tumors for estimating tumor progression. Atypical carcinoids have higher Ki-67 index and have an increased propensity to metastasize as compared to typical ones. 68Ga-DOTATATE PET imaging along with Ki-67 can be correlated for better management of patients with neuroendocrine tumors. We describe the dual tracer imaging features in a patient of pulmonary carcinoid with avid 68Ga-DOTATATE and minimal 18FDG (18Flurodeoxyglucose) uptake diagnosed on the basis of imaging and bronchoscopic biopsy and its correlation with tumor proliferation index. PMID:28242984

  15. Phase III trial of chemotherapy using 5-fluorouracil and streptozotocin compared with interferon alpha for advanced carcinoid tumors: FNCLCC-FFCD 9710.

    PubMed

    Dahan, Laetitia; Bonnetain, Frank; Rougier, Philippe; Raoul, Jean-Luc; Gamelin, Eric; Etienne, Pierre-Luc; Cadiot, Guillaume; Mitry, Emmanuel; Smith, Denis; Cvitkovic, Frédérique; Coudert, Bruno; Ricard, Floriane; Bedenne, Laurent; Seitz, Jean-François

    2009-12-01

    The aim of this randomized multicenter phase III trial was to compare chemotherapy and interferon (IFN) in patients with metastatic carcinoid tumors. Patients with documented progressive, unresectable, metastatic carcinoid tumors were randomized between 5-fluorouracil plus streptozotocin (day 1-5) and recombinant IFN-alpha-2a (3 MU x 3 per week). Primary endpoint was progression-free survival (PFS). From February 1998 to June 2004, 64 patients were included. The two arms were well matched for median age, sex ratio, PS 0-1, previous chemotherapy, surgery, or radiotherapy. The median PFS for chemotherapy was 5.5 months versus 14.1 for IFN (hazard ratio=0.75 (0.41-1.36)). Overall survival (OS), tolerance, and effects on carcinoid symptoms were not significantly different. Despite a trend in favor of IFN, there was no difference in PFS and OS in advanced metastatic carcinoid tumors and therapeutic effect of both treatments was mild.

  16. [Pulmonary Carcinoid Tumor with Cushing's Syndrome in a Patient who Underwent Pulmonary Resection by Video-Assisted Thoracic Surgery;Report of a Case].

    PubMed

    Sato, Hiroshi; Mishina, Taijiro; Miyajima, Masahiro; Watanabe, Atsushi

    2015-07-01

    Pulmonary carcinoid tumor with Cushing's syndrome is comparatively rare disease. It is difficult to make an early diagnosis due to small size lesion in its early stage. We report a case of pulmonary carcinoid tumor with Cushing's syndrome successfully localised by positron emission tomography/computed tomography and was resected in the early stage. The levels of serum cortisol and adrenocorticotropic hormone( ACTH) decreased immediately after surgery, and the symptoms of Cushing's syndrome were relieved.

  17. Development of a gastric carcinoid tumor following allogeneic hematopoietic stem cell transplantation for early T-cell precursor acute lymphoblastic leukemia.

    PubMed

    Chang, Tsung-Yen; Lai, Jin-Yao; Wang, Chao-Jan; Chen, Shih-Yen; Jaing, Tang-Her; Hsueh, Chuen; Shih, Lee-Yung; Chen, Shih-Hsiang

    2017-06-01

    Gastric carcinoid tumor is rarely diagnosed in children. We report a case of gastric carcinoid tumor that occurred after allogeneic HSCT. A 13-year-old girl with ETP acute lymphoblastic leukemia underwent allogeneic HSCT from a 7/8 HLA-matched unrelated donor. She presented with rashes, abdominal pain, and diarrhea, which were suggestive of GVHD, 7 months after HSCT. Immunosuppressive agents failed to resolve these symptoms well. After a series of evaluations, carcinoid syndrome caused by a gastric carcinoid tumor was diagnosed. The tumor was located in the antral region and resulted in partial gastric outlet obstruction. She received subtotal gastrectomy with regional lymph node dissection. However, she had a flare-up of GVHD 1 month after surgery, and immunosuppressive therapy was intensified accordingly. Although her GVHD was getting better, she developed respiratory syncytial viral pneumonia with rapid progression to respiratory failure. She died of multiple organ failure 2 months postoperatively. This is the first pediatric case of a gastric carcinoid tumor following allogeneic HSCT. Our case also highlights the necessity for pediatric transplant physicians to be aware of carcinoid syndrome caused by this rare tumor in the setting of GVHD with poor response to immunosuppressive agents. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Overdiagnosis of a typical carcinoid tumor as an adenocarcinoma of the lung: a case report and review of the literature

    PubMed Central

    2012-01-01

    Background Overdiagnosis of bronchopulmonary carcinoid tumors together with overtreatment can cause serious postoperative consequences for the patient. We report of a patient with a typical bronchopulmonary carcinoid tumor, which was initially misdiagnosed and treated as an adenocarcinoma of the lung. GnrH receptors and the associated Raf-1/MEK/ERK-1/2-pathway are potential targets for analogs in cancer treatment. We suspected a correlation between the lack of tumor growth, application of leuprolide and the Raf-1/MEK/ERK-1/2-pathway. Therefore, we examined GnrH receptor status in the examined specimen. Case presentation In 2010 a 77 year-old male patient was shown to have a tumor mass of about 1.7 cm diameter in the inferior lobe of the left lung. Since 2005, this tumor had hitherto been known and showed no progression in size. The patient suffered from prostate cancer 4 years ago and was treated with TUR-P, radiation therapy and the application of leuprolide. We conducted an explorative thoracotomy with atypical segment resection. The first histological diagnosis was a metastasis of prostate cancer with lymphangiosis carcinomatosa. After several immunohistochemical stainings, the diagnosis was changed to adenocarcinoma of the lung. We conducted a re-thoracotomy with lobectomy and systematic lymphadenectomy 12 days later. The tumor stage was pT1 N0 MX G2 L1 V0 R0. Further immunohistochemical studies were performed. We received the results 15 days after the last operation. The diagnosis was ultimately changed to typical carcinoid tumor without any signs of lymphatic vessel invasion. The patient recovered well from surgery, but still suffers from dyspnea and lack of physical performance. Lung function testing revealed no evidence of impairment. Conclusion The use of several immunohistochemical markers, careful evaluation of hematoxylin-eosin sections and the Ki-67 labelling index are important tools in discriminating between carcinoids and other bronchopulmonary

  19. A case of parenchymal-sparing right mainstem bronchial sleeve resection for carcinoid tumor.

    PubMed

    Pont, Bert Du; Decaluwé, Herbert; Van Raemdonck, Dirk

    2016-02-01

    Introduction Neuroendocrine tumors are rare bronchial carcinomata often presenting in a central airway. Resection usually includes a sleeve of the bronchus with the underlying lobe. Case report We present a 19-year old male with retro-obstructive pneumonia from a tumor in the right mainstem bronchus. Bronchoscopy showed an obstructive mass confirmed as being a typical carcinoid on biopsy. Sleeve resection of the mainstem bronchus only was successfully performed sparing the entire right lung. Discussion and conclusion This type of limited tumoral resection should be reserved for carefully selected patients with a low-grade neoplasm without extrabronchial extension and with both tumor-negative lymph nodes and bronchial margins on frozen section.

  20. Carcinoid heart disease: a guide for screening and timing of surgical intervention.

    PubMed

    Hart, E A; Meijs, T A; Meijer, R C A; Dreijerink, K M; Tesselaar, M E; de Groot, C A; Valk, G D; Chamuleau, S A J

    2017-06-19

    The cardiac manifestations of a neuroendocrine tumour are referred to as carcinoid heart disease (CaHD) and are associated with a poor prognosis. Surgical intervention is the only proven therapeutic option and may prolong survival and quality of life. No consensus has been reached internationally with regard to screening for CaHD and the optimal timing for surgery. Although limited evidence is available on this matter, a trend towards early surgery and subsequent reduced mortality has been observed. In this review we provide an overview of the current understanding and propose a protocol to guide cardiologists in the screening for CaHD and the timing of referral to a specialised surgical centre.

  1. [Ectopic Adrenocorticotropic Hormone-producing Pulmonary Carcinoid Tumor Presenting as Cushing Syndrome].

    PubMed

    Taniguchi, Daisuke; Sawada, Takahiro; Ebisui, Osamu; Kito, Katsumi; Nagayasu, Takeshi

    2015-02-01

    The patient was a 57-year-old female who felt muscle weakness and visited a physician. Hypokalemia was pointed out, and she was referred to our hospital for detailed examination and treatment. Hormone-related tests and imaging were performed, and the patient was diagnosed as Cushing syndrome. Moreover, an ectopic adrenocorticotropic hormone (ACTH)-producing tumor was suspected. The whole body was examined to find a tumor, but no apparent lesion was found, except for a small nodule of 5-mm in size was present in the right middle pulmonary lobe on chest computed tomography (CT). It was decided to perform surgical resection for both diagnosis and treatment. Pathological diagnosis was a typical carcinoid. On immunostaining, ACTH-positive cells were detected, and the lesion was definitely diagnosed as an ectopic ACTH-producing tumor. Since the ACTH level after surgery returned to normal, the lesion was concluded to be completely excised.

  2. AACE/ACE DISEASE STATE CLINICAL REVIEW: DIAGNOSIS AND MANAGEMENT OF MIDGUT CARCINOIDS

    PubMed Central

    Katznelson, Laurence; Vinik, Aaron I.; Wong, Richard; Randolph, Gregory

    2016-01-01

    Objective Neuroendocrine tumors (NETs) are a collection of complex tumors that arise from the diffuse endocrine system, primarily from the digestive tract. Carcinoid tumors most commonly originate from the small intestine. These tumors are either referred to as small intestinal neuroendocrine tumors or midgut carcinoids (MGCs). The purpose of this review article is to survey the diagnostic and therapeutic pathways for patients with MGC and provide an overview of the complex multidisciplinary care involved in improving their quality of life, treatment outcomes, and survival. Methods The current literature regarding the diagnosis and management of MGCs was reviewed. Results Dry flushing and secretory diarrhea are the hallmarks of the clinical syndrome of MGC. Managing MGC requires attention to the overall symptom complex, including the physical effects of the tumor and biomarker levels. The somatostatin analogs (SAs) octreotide and lanreotide are highly efficacious for symptomatic improvement. MGCs require resection to encompass the primary tumor and mesenteric lymph node metastases and should include cholecystectomy if the patient is likely to receive SA therapy. Debulking of liver metastasis by resection in combination with ablative therapies and other liver-directed modalities may help palliate symptoms and hormonal overproduction in carefully selected patients. Quality of life is an important measure of patients’ perception of the burden of their disease and impact of treatment modalities and may be a useful guide in deciding changes in therapy to alter apparent health status. Conclusion MGC is a challenging malignancy that requires the input of a multidisciplinary team to develop the best treatment plan. Consultation with expert centers that specialize in NETs may also be indicated for complex cases. With expert care, patients can be cured or live with the disease and enjoy good quality of life. PMID:25962092

  3. [A case of rectal carcinoid tumor with lymph node and liver metastasis 5 years after curative resection].

    PubMed

    Shindo, Yoshitaro; Hazama, Shoichi; Maeda, Yoshinari; Sakamoto, Kazuhiko; Tamesa, Takao; Yoshino, Shigefumi; Tanaka, Shinsuke; Kawano, Hiroo; Oka, Masaaki

    2013-11-01

    A 65-year-old man was diagnosed with a rectal carcinoid tumor (10 mm in diameter) in July 2007. We performed low anterior resection with lymph node dissection. Histological depth of penetration of the rectal wall by the primary tumor was up to the submucosa, and lymph node metastasis was observed at station 251 (Japanese Classification of Colorectal Carcinoma, seventh Edition). Five years later, abdominal enhanced computed tomography (CT) revealed multiple liver tumors and swelling of the right obturator lymph nodes. During surgery, ultrasonography revealed 10 hypoechoic masses in both hepatic lobes. We performed right pelvic lymph node dissection, partial hepatectomy (S5/6, S7, and S8), and microwave coagulation therapy. After surgery, the patient was treated with octreotide long-acting repeatable( LAR). The patient remained disease-free for 10 months after surgery. Our findings suggest that careful monitoring is necessary for metachronous lymph node and liver metastasis during follow-up treatment for rectal carcinoid tumors.

  4. Paraneoplastic Cushing's syndrome associated with bronchopulmonary carcinoid tumor in youth: A case report and review of the literature.

    PubMed

    Li, Wen-Ya; Liu, Xu-Dong; Li, Wei-Nan; Dong, Si-Yuan; Qu, Xiao-Han; Gong, Shu-Lei; Shao, Ming-Rui; Zhang, Lin

    2016-07-01

    Paraneoplastic Cushing's syndrome (CushingPS) caused by bronchopulmonary carcinoid tumors presents a diagnostic challenge for clinicians. The present study reports the case of an 18-year-old male patient presenting with rapid weight gain, polyuria, polydipsia and progressive muscle weakness. Chemical and imaging findings suggested ectopic secretion of adrenocorticotropin. Whole-body (18)fluorine-fluorodeoxyglucose ((18)FDG-PET/CT) positron-emission tomography revealed an increased uptake of (18)FDG-PET/CT in the right middle lung mass and lobar lymph node. Postoperative pathology confirmed the presence of a typical carcinoid, as well as a lobar lymph node metastasis. The patient underwent a right middle lobectomy with mediastinal lymph node resection, which resulted in symptom clearance, followed by rapid weight loss. No CushingPS or tumor recurrence was observed at the 3-month postoperative follow-up.

  5. [Primary Carcinoid Tumor of the Urinary Bladder: A Case Report and Questionnaire Survey on the Reported Cases in Japan].

    PubMed

    Ueda, Norichika; Arai, Hiroki; Honda, Masahito; Yoshida, Kyotaro

    2015-11-01

    An 83-year-old man presented with asymptomatic macroscopic hematuria while being treated for prostate cancer with hormonal therapy in January 2009. Cystoscopy revealed a 5 mm surface-smooth non pedunculated tumor lateral to the left ureteral orifice. Transurethral resection was carried out to examine the histology of the tumor. Histopathologic examination revealed the tumor arranged in a pseudoglandular pattern covered with erosive urothelial cells. The tumor had inconspicuous nucleoli, and abundant eosinophilic cytoplasm. An immunohistochemical study showed the tumor cells were positively stained for chromogranin A, synaptophysin, CD56, and NSE. Ki67 index of the tumor was below 2 %, indicating that the tumor was a carcinoid tumor. He showed no carcinoid syndrome, and neither recurrence nor metastasis has been detected for 66 months.

  6. Paraneoplastic Cushing's syndrome associated with bronchopulmonary carcinoid tumor in youth: A case report and review of the literature

    PubMed Central

    LI, WEN-YA; LIU, XU-DONG; LI, WEI-NAN; DONG, SI-YUAN; QU, XIAO-HAN; GONG, SHU-LEI; SHAO, MING-RUI; ZHANG, LIN

    2016-01-01

    Paraneoplastic Cushing's syndrome (CushingPS) caused by bronchopulmonary carcinoid tumors presents a diagnostic challenge for clinicians. The present study reports the case of an 18-year-old male patient presenting with rapid weight gain, polyuria, polydipsia and progressive muscle weakness. Chemical and imaging findings suggested ectopic secretion of adrenocorticotropin. Whole-body 18fluorine-fluorodeoxyglucose (18FDG-PET/CT) positron-emission tomography revealed an increased uptake of 18FDG-PET/CT in the right middle lung mass and lobar lymph node. Postoperative pathology confirmed the presence of a typical carcinoid, as well as a lobar lymph node metastasis. The patient underwent a right middle lobectomy with mediastinal lymph node resection, which resulted in symptom clearance, followed by rapid weight loss. No CushingPS or tumor recurrence was observed at the 3-month postoperative follow-up. PMID:27347101

  7. Misdiagnosed Case of Bronchial Carcinoid Presenting with Refractory Dyspnoea and Wheeze: A Rare Case Report and Review of Literature

    PubMed Central

    Santra, Avradip; Dutta, Pravati; Pothal, Sudarsan; Manjhi, Rekha

    2013-01-01

    A 59-year-old male smoker presented with persistent wheezing and occasional coughing that had been ongoing for two years and had been unsuccessfully treated with an inhalational β2 agonist, an anticholinergic and an inhalational steroid in the last year. On clinical examination, a left-sided wheeze was detected. The initial chest X-ray was normal. A computed tomography (CT) scan of thorax demonstrated a mass lesion in the left main bronchus. On subsequent bronchoscopy, an endobronchial polypoid mass was detected in the left main bronchus, completely occluding the bronchial lumen. A biopsy taken from the mass revealed features of bronchial carcinoid. Bronchial carcinoid can present uncommonly with wheezes, resulting in misdiagnosis as bronchial asthma or chronic obstructive pulmonary disease (COPD). If an asthma or COPD patient does not respond to conventional therapy, a CT scan and subsequent bronchoscopy is warranted. PMID:23966830

  8. A false positive fluorodeoxyglucose lymphadenopathy in a patient with pulmonary carcinoid tumor and previous breast reconstruction after bilateral mastectomy.

    PubMed

    Billè, Andrea; Girelli, Lara; Leo, Francesco; Pastorino, Ugo

    2014-03-01

    We present a case of a 60-year-old woman with a positive fluorodeoxyglucose integrated positron emission tomography and computed tomography (PET/TC) mammary chain lymphadenopathy and carcinoid tumor of the left lower lobe who had a previous bilateral mastectomy and breast reconstruction for breast cancer. She underwent a right muscle sparing mini-thoracotomy and mammary chain lymphadenectomy; the final histopathology showed granulomatous reaction to silicone.

  9. Comparison of endoscopic resection therapies for rectal carcinoid tumor: endoscopic submucosal dissection versus endoscopic mucosal resection using band ligation.

    PubMed

    Choi, Cheol W; Kang, Dae H; Kim, Hyung W; Park, Su B; Jo, Woo S; Song, Geun A; Cho, Mong

    2013-01-01

    Endoscopic mucosal resection (EMR) has been the endoscopic treatment of choice for rectal carcinoid tumors <10 mm in size. Endoscopic submucosal dissection (ESD) may cause more severe complications, longer operation time, and higher cost than EMR. : To compare EMR using band ligation (EMR-B) method with ESD for the endoscopic treatment of rectal carcinoid tumors. From November 2008 to September 2011, we enrolled consecutive patients with rectal carcinoid tumors <10 mm in diameter and without lymph node enlargement. Rate of complete resection rate, incidence of complications, and length of procedures were evaluated. Sixty patients were enrolled (31 ESD cases and 29 EMR-B cases). The mean age was 48.03±13.09 years. Both groups had similar mean tumor diameter (EMR-B 4.34±1.75 vs. ESD 5.22±2.09 mm; P=0.084). Resection time was longer in the ESD group than in the EMR-B group (15.09±5.73 vs. 6.37±5.52 min; P<0.001). The complete resection rate was 80.6% (25 of 31) in the ESD group and 82.8% (24 of 29) in the EMR-B group (P=0.833). In incomplete resection cases, neither local recurrence nor distant metastasis was detected during the follow-up period. Compared with ESD, EMR-B resulted in a comparable histologically complete resection rate and took less time to perform. Given the advantages of easier and shorter procedure time, EMR-B may be considered the treatment of choice for small rectal carcinoid tumors.

  10. The CCK(2) receptor antagonist, YF476, inhibits Mastomys ECL cell hyperplasia and gastric carcinoid tumor development.

    PubMed

    Kidd, M; Siddique, Z-L; Drozdov, I; Gustafsson, B I; Camp, R L; Black, J W; Boyce, M; Modlin, I M

    2010-06-08

    YF476 is a potent and highly selective cholecystokin 2 (CCK(2)) receptor antagonist of the benzodiazepine class. It inhibits gastric neuroendocrine enterochromaffin-like (ECL) cell secretion, proliferation and spontaneous formation of gastric neuroendocrine tumors (carcinoids) in cotton rats. The Mastomys rodent species exhibits a genetic predisposition to gastric ECL neuroendocrine tumor formation which can be accelerated by acid suppression and induction of hypergastrinemia. In this respect, it mimics the human condition of atrophic gastritis, hypergastrinemia and gastric carcinoid development. We investigated whether YF476 could inhibit acid suppression-induced ECL cell hyperplasia and neoplasia in this model. In addition, we examined whether YF476 could reverse established ECL cell hyperplasia and neoplasia. Targeting the CCK(2) receptor during Loxtidine-induced hypergastrinemia resulted in a reduction in ECL cell secretion (plasma and mucosal histamine, and histidine decarboxylase (HDC) transcripts, p<0.05) and proliferation (numbers of HDC-positive cells, connective tissue growth factor (CTGF) and cyclin D1 transcription). This was associated with a decrease in ECL cell hyperplasia and a 60% reduction in gastric ECL cell microcarcinoid (tumors <0.3mm in size) formation. YF476 inhibited ECL cell neoplasia (gastric carcinoid) in animals with hyperplasia, inhibited the formation of ECL cell tumors when co-administered with Loxtidine and reversed the growth and developement of gastric ECL cell carcinoids in long-term acid suppressed Mastomys. Variable importance analysis using a logistic multinomial regression model indicated the effects of YF476 were specific to the ECL cell and alterations in ECL cell function reflected inhibition of transcripts for HDC, Chromogranin A (CgA), CCK(2) and the autocrine growth factor, CTGF. We conclude that specifically targeting the CCK(2) receptor inhibits gastrin-mediated ECL cell secretion and ECL cell proliferation and tumor

  11. A Study of Appendiceal Crypt Cell Adenocarcinoma (So-Called Goblet Cell Carcinoid and Its Related Adenocarcinoma).

    PubMed

    Nonaka, Daisuke; Papaxoinis, George; Lamarca, Angela; Fulford, Paul; Valle, Juan; Chakrabarty, Bipasha

    2017-08-17

    Goblet cell carcinoids (GCCs) of the appendix are rare tumors, characterized by a carcinoid-like organoid growth pattern. Despite the term carcinoid, neuroendocrine features are inconspicuous, and its behavior is distinct from carcinoid. Its high grade counterpart is designated as adenocarcinoma ex GCC. We conducted a retrospective study of 105 tumors to find prognostic values of a variety of clinico-pathologic features. The tumors were subclassified as low grade, equivalent to classic type, and high grade, defined as loss of organoid pattern, and a proportion (%) of low and high grades were documented in each tumor. Correlations between survival and various clinico-pathologic parameters were investigated. One-third were pure low grade while the remainder contained variable high grade component ranging 5-95%. Neuroendocrine cell component ranged 0-90% (median 5) while mucus cell component ranged 5-100% (median 70). By univariate analysis, size, stage, high grade component, nuclear grade, surgery and chemotherapy correlated with cancer-related survival (CSS), and by multivariate analysis, stage (P=.001), high grade component (P=.008) and tumor size (P=.005) correlated with CSS. There was significant difference in CSS when the cases were grouped in high grade component <40%, 40-90 and ≤90% (P<.001). Our results indicate that staging and proportion of high grade histology may provide important prognostic information. Neuroendocrine component was insignificant in both low and high grade areas. In light of our findings, this tumor type is best regarded as a variant of adenocarcinoma, and the term crypt cell adenocarcinoma more appropriately reflects the nature and origin of this tumor group. Copyright © 2017. Published by Elsevier Inc.

  12. A Case of Pulmonary Carcinoid Tumor with a Superimposed Aspergilloma Presenting As a Covert Ectopic Adrenocorticotropic Hormone Syndrome

    PubMed Central

    Kim, Kyoung Jin; Yu, Ji Hee; Kim, Nan Hee; Kim, Young Hye; Kim, Young Sik; Seo, Ji A

    2017-01-01

    Ectopic adrenocorticotropic hormone (ACTH) syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Laboratory results revealed ACTH-dependent Cushing syndrome, but imaging studies did not show any discrete lesions secreting ACTH. The petrosal to peripheral ACTH gradient resulted in no evidence of pituitary adenoma. As the only lesion suspicious for ectopic ACTH secretion was a right lower round cystic lesion that did not appear to be a carcinoid tumor on computed tomography scan of the chest, the patient underwent video-assisted thoracic surgical resection to provide a definitive diagnosis. The final diagnosis was a small ectopic ACTH-secreting carcinoid tumor with unusual superimposed aspergilloma in the periphery of the lung. Postoperatively, the abnormal endocrine levels were normalized, and all of the clinical symptoms and signs were ameliorated. This is an informative case of ectopic ACTH syndrome (EAS) that was the cause of hypokalemia, hypertension, metabolic alkalosis, and hypercortisolism despite its poorly specific cushingoid morphology and uncommon imaging findings. Therefore, we recommend that clinicians investigate any possible lesion as a potential source of EAS. PMID:28642733

  13. A Case of Pulmonary Carcinoid Tumor with a Superimposed Aspergilloma Presenting As a Covert Ectopic Adrenocorticotropic Hormone Syndrome.

    PubMed

    Kim, Kyoung Jin; Yu, Ji Hee; Kim, Nan Hee; Kim, Young Hye; Kim, Young Sik; Seo, Ji A

    2017-01-01

    Ectopic adrenocorticotropic hormone (ACTH) syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Laboratory results revealed ACTH-dependent Cushing syndrome, but imaging studies did not show any discrete lesions secreting ACTH. The petrosal to peripheral ACTH gradient resulted in no evidence of pituitary adenoma. As the only lesion suspicious for ectopic ACTH secretion was a right lower round cystic lesion that did not appear to be a carcinoid tumor on computed tomography scan of the chest, the patient underwent video-assisted thoracic surgical resection to provide a definitive diagnosis. The final diagnosis was a small ectopic ACTH-secreting carcinoid tumor with unusual superimposed aspergilloma in the periphery of the lung. Postoperatively, the abnormal endocrine levels were normalized, and all of the clinical symptoms and signs were ameliorated. This is an informative case of ectopic ACTH syndrome (EAS) that was the cause of hypokalemia, hypertension, metabolic alkalosis, and hypercortisolism despite its poorly specific cushingoid morphology and uncommon imaging findings. Therefore, we recommend that clinicians investigate any possible lesion as a potential source of EAS.

  14. Carcinoid syndrome

    MedlinePlus

    ... signs of: Heart valve problems, such as murmur Niacin-deficiency disease ( pellagra ) Tests that may be done ... Endoscopic thoracic sympathectomy Gastrointestinal bleeding Intestinal obstruction Iontophoresis Niacin Protein in diet Serotonin blood test Wheezing Review ...

  15. Carcinoid Syndrome

    MedlinePlus

    ... specializes in treating cancer (oncologist), disorders of the endocrine system (endocrinologist) or a surgeon. Because appointments can be ... Niederhuber JE, et al., eds. Cancer of the endocrine system. In: Abeloff's Clinical Oncology. 5th ed. Philadelphia, Pa.: ...

  16. Typical laryngeal carcinoid tumor with recurrence and lymph node metastasis: a case report and review of the literature.

    PubMed

    Wang, Qinying; Chen, Haihong; Zhou, Shuihong

    2014-01-01

    Typical carcinoid tumor of the larynx is an extremely rare lesion which arises from neuroendocrine cells scanning in the laryngeal mucosa or submucosal glands. Conventionally, it is a well-differentiated neuroendocrine carcinoma, conservative surgery represents the treatment of choice, and the patient usually has a good prognosis with rarely recurrence and metastasis. In this report, we present a case of typical laryngeal carcinoid tumor with recurrence and lymph node metastasis. The patient was a 58-year-old man, complaints of intermittent burning pain in his both ears for 2 years, and for the recent one month the pain turn to continuous accompanied with a mild discomfort in the throat, he had neither hoarseness, dysphagia, nor any otorrhea and hearing loss. The patient was scheduled to undergo a tracheotomy and then a biopsy under supporting laryngoscopy. During the operation, the frozen section diagnosis from the first and the second time both indicated that the biopsy specimens originated from musculo-epithelia, it couldn't be differed from malignant to benign. So the mass was simply removed under supporting laryngoscopy. The histopathology from paraffin sections revealed typical carcinoid of the larynx and the second procedure consisted of supraglottic laryngectomy with clear margins, the otalgia resolved and the patient had no difficulty with phonation or swallowing. After 1 year follow-up, the patient was found a mass on his right neck with symptom free, B-ultrasonography indicated several enlargement lymph nodes with some merged on both sides of the neck, the patient was scheduled to undergo a "total-laryngectomy with radical neck dissection on the left side and an elective neck dissection on the right side". The specimens were positive for cytokeratin, chromogranin A and synaptophysin, a final diagnosis of typical carcinoid was made for the recurrence lesion and the metastasis of the lymph nodes. Though the post-operative recovery was uneventful, the

  17. Typical laryngeal carcinoid tumor with recurrence and lymph node metastasis: a case report and review of the literature

    PubMed Central

    Wang, Qinying; Chen, Haihong; Zhou, Shuihong

    2014-01-01

    Typical carcinoid tumor of the larynx is an extremely rare lesion which arises from neuroendocrine cells scanning in the laryngeal mucosa or submucosal glands. Conventionally, it is a well-differentiated neuroendocrine carcinoma, conservative surgery represents the treatment of choice, and the patient usually has a good prognosis with rarely recurrence and metastasis. In this report, we present a case of typical laryngeal carcinoid tumor with recurrence and lymph node metastasis. The patient was a 58-year-old man, complaints of intermittent burning pain in his both ears for 2 years, and for the recent one month the pain turn to continuous accompanied with a mild discomfort in the throat, he had neither hoarseness, dysphagia, nor any otorrhea and hearing loss. The patient was scheduled to undergo a tracheotomy and then a biopsy under supporting laryngoscopy. During the operation, the frozen section diagnosis from the first and the second time both indicated that the biopsy specimens originated from musculo-epithelia, it couldn’t be differed from malignant to benign. So the mass was simply removed under supporting laryngoscopy. The histopathology from paraffin sections revealed typical carcinoid of the larynx and the second procedure consisted of supraglottic laryngectomy with clear margins, the otalgia resolved and the patient had no difficulty with phonation or swallowing. After 1 year follow-up, the patient was found a mass on his right neck with symptom free, B-ultrasonography indicated several enlargement lymph nodes with some merged on both sides of the neck, the patient was scheduled to undergo a “total-laryngectomy with radical neck dissection on the left side and an elective neck dissection on the right side”. The specimens were positive for cytokeratin, chromogranin A and synaptophysin, a final diagnosis of typical carcinoid was made for the recurrence lesion and the metastasis of the lymph nodes. Though the post-operative recovery was uneventful, the

  18. Ectopic Cushing syndrome associated with thymic carcinoid tumor as the first presentation of MEN1 syndrome-report of a family with MEN1 gene mutation.

    PubMed

    Hasani-Ranjbar, Shirin; Rahmanian, Masoud; Ebrahim-Habibi, Azadeh; Soltani, Akbar; Soltanzade, Akbar; Mahrampour, Elnaz; Amoli, Mahsa M

    2014-06-01

    Multiple endocrine neoplasia type 1(MEN1) is an autosomal dominant syndrome. Although thymic carcinoid tumor is recognized as a part of MEN1 syndrome but functioning thymic carcinoid tumor as the first presentation of the MEN1 seems to be very rare. In this report, we present a 29-year-old male who developed ectopic Cushing syndrome secondary to thymic carcinoid tumor and was diagnosed as MEN1 syndrome 2 years later. Further evaluation revealed the presence of carcinoid tumor and other MEN 1 manifestations in several other member of family. Genetic evaluation showed presence of a previously reported mutation in exon 10(R527X) of MEN1 gene in these patients. This presentation showed that thymic neuroendocrine tumor could be the first manifestation of the MEN1 syndrome and it might be diagnosed as a dominant manifestation of this syndrome in a family. We suggest biochemical or genetic screening for MEN-1 syndrome for patients with thymic carcinoid.

  19. Long-term follow up of patients affected by pulmonary carcinoid at the Istituto Nazionale Tumori of Milan: a retrospective analysis

    PubMed Central

    Pusceddu, S; Catena, L; Valente, M; Buzzoni, R; Formisano, B; Del Vecchio, M; Ducceschi, M; Tavecchio, L; Fabbri, A; Bajetta, E

    2010-01-01

    Neuroendocrine tumors of the lung involve an heterogeneous group of tumors representing a wide range of histological variants, from well-differentiated typical carcinoid (TC) tumors to poorly differentiated small cell carcinomas. The epidemiology, clinical outcome, and management of these neoplasms differ significantly from other lung malignancies. The main aim of this report consists in describing the single Center experience of the Istituto Nazionale Tumori of Milan on neuroendocrine lung tumors, with an emphasis on bronchopulmonary carcinoid subtypes. From 1986 to 2009, 91 cases of carcinoid tumors were diagnosed; these were divided in two series, according to typical (66 patients) or atypical [25] histotypes. These two groups were compared in relation to various features, including pathologic classification, clinical behavior, treatment modalities and long-term survival. At the moment of diagnosis 11 patients had locally advanced/metastatic disease, while 80 patients showed non metastatic disease. The comparative analysis between typical and atypical series disclosed significant differences in terms of long-term survival; in fact, 5-year and 10-year survival rates were 98 % and 94 % for the first carcinoid series versus 76 % and 18 % for the atypical series, respectively (p<0.001). The median overall survival (OS) was 76 months (range 3-182) for atypical carcinoids and has not yet been reached for TCs patients. PMID:22263011

  20. Long-term follow up of patients affected by pulmonary carcinoid at the Istituto Nazionale Tumori of Milan: a retrospective analysis.

    PubMed

    Pusceddu, S; Catena, L; Valente, M; Buzzoni, R; Formisano, B; Del Vecchio, M; Ducceschi, M; Tavecchio, L; Fabbri, A; Bajetta, E

    2010-03-01

    Neuroendocrine tumors of the lung involve an heterogeneous group of tumors representing a wide range of histological variants, from well-differentiated typical carcinoid (TC) tumors to poorly differentiated small cell carcinomas. The epidemiology, clinical outcome, and management of these neoplasms differ significantly from other lung malignancies. The main aim of this report consists in describing the single Center experience of the Istituto Nazionale Tumori of Milan on neuroendocrine lung tumors, with an emphasis on bronchopulmonary carcinoid subtypes. From 1986 to 2009, 91 cases of carcinoid tumors were diagnosed; these were divided in two series, according to typical (66 patients) or atypical [25] histotypes. These two groups were compared in relation to various features, including pathologic classification, clinical behavior, treatment modalities and long-term survival. At the moment of diagnosis 11 patients had locally advanced/metastatic disease, while 80 patients showed non metastatic disease. The comparative analysis between typical and atypical series disclosed significant differences in terms of long-term survival; in fact, 5-year and 10-year survival rates were 98 % and 94 % for the first carcinoid series versus 76 % and 18 % for the atypical series, respectively (p<0.001). The median overall survival (OS) was 76 months (range 3-182) for atypical carcinoids and has not yet been reached for TCs patients.

  1. The role of endogenous gastrin in the development of enterochromaffin-like cell carcinoid tumors in Mastomys natalensis: a study with the specific gastrin receptor antagonist AG-041R.

    PubMed

    Chiba, T; Kinoshita, Y; Sawada, M; Kishi, K; Baba, A; Hoshino, E

    1998-01-01

    We examined the effects of a newly synthesized gastrin receptor antagonist, AG-041R, on the growth of enterochromaffin-like (ECL) carcinoid tumors in Mastomys natalensis both in vitro and in vivo. AG-041R was as potent as the well known gastrin antagonist L365,260 in inhibiting not only the gastrin-induced release of histamine from but also histidine decarboxylase (HDC) gene expression in the ECL carcinoid tumor cells. AG-041R also inhibited gastrin-induced DNA synthesis and c-fos gene expression in the tumor cells. Furthermore, AG-041R significantly inhibited the growth of the transplanted Mastomys ECL carcinoid tumors in vivo. From these data, it is concluded that endogenous gastrin is involved in the growth of ECL carcinoid tumors in Mastomys natalensis. Moreover, AG-041R is shown to have a potential as an anti-neoplastic agent for ECL carcinoid tumor of the stomach.

  2. Antitumour activity of somatostatin analogues in sporadic, progressive, metastatic pulmonary carcinoids.

    PubMed

    Sullivan, Ivana; Le Teuff, Gwénaël; Guigay, Joël; Caramella, Caroline; Berdelou, Amandine; Leboulleux, Sophie; Déandréis, Désirée; Hadoux, Julien; Ducreux, Michel; Duvillard, Pierre; Adam, Julien; Scoazec, Jean-Yves; Baudin, Eric; Planchard, David

    2017-04-01

    Antiproliferative activity of somatostatin analogues (SSAs) has been demonstrated in digestive neuroendocrine tumours but few data have been published on pulmonary carcinoids (PC). The aim of this retrospective study was to report the antitumour activity of SSAs in patients with progressive, metastatic PC. Patients with PC and treated with SSA monotherapy were reviewed. Disease was classified according to the tumour slope prior to SSA initiation as rapidly progressive (at least 20% increase in the sum of the longest diameter of target lesions or the appearance of one or more new lesions within 6 months) or slowly progressive (if progression occurred over 6 months). Survival outcomes were progression-free survival (PFS) and overall survival (OS). We additionally examined the overall response rate and safety. Prognostic factors associated with PFS and OS were sought. Hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using the Cox model. Among 67 patients reviewed, 61 were included in the study. Forty-one (67%) of them exhibited slowly progressive disease prior to SSAs, 41 (67%) had atypical carcinoids and 29 (48%) had functioning tumours. Forty-six (76%) patients had received SSAs as first-line therapy. The best overall response was stable disease in 47 (77%) patients. The median duration of SSAs was 13.7 months. With a median follow-up of 5.8 years, median PFS and OS were 17.4 (95% CI: 8.7-26.0) and 58.4 (95% CI: 44.2-102.7) months, respectively. Functioning tumours and slowly progressive disease were significantly associated with longer PFS: HR = 0.48 ([95% CI: 0.24-0.95], p = 0.03) and HR = 7.43 ([95% CI: 3.02-18.25], p < 0.0001), respectively. Only functioning tumours remained significantly associated with OS: HR = 0.33 ([95% CI: 0.14-0.79], p = 0.01). Treatment had been discontinued in two patients due to side-effects. Median PFS observed in our study is encouraging for PC patients. Patients with functioning tumours and slowly

  3. Healthcare and economic impact of diarrhea in patients with carcinoid syndrome

    PubMed Central

    Broder, Michael S; Chang, Eunice; Romanus, Dorothy; Cherepanov, Dasha; Neary, Maureen P

    2016-01-01

    AIM: To examine healthcare resource utilization patterns and costs accrued by carcinoid syndrome (CS) patients with and without diarrhea. METHODS: We conducted a retrospective cohort study using MarketScan® data from 1/1/2002-12/31/2012. Newly diagnosed CS patients had 1 medical claim for CS (ICD-9-CM code 259.2) plus either ≥ 1 additional claim for CS or for carcinoid tumors (ICD-9-CM 209.x), and had no evidence of CS for 1 year prior to index CS diagnosis, in commercially-insured patients < 65 years old. Patients were required to have continuous enrollment one year prior and after index date (first claim with CS diagnosis in the ID period). We identified patients with evidence of non-infectious diarrhea (ICD-9-CM codes 564.5 and 787.91) within one year from the index date. Overall and CS-related healthcare resource utilization and costs were compared between patients with and without non-infectious diarrhea during the one year period after the index date. RESULTS: There were 2822 newly diagnosed CS patients; 534 (18.9%) had evidence of non-infectious diarrhea. Compared to patients without non-infectious diarrhea, non-infectious diarrhea patients more commonly had at ≥ 1 CS-related hospitalization (13.7% vs 7.2%), ≥ 1 CS-related ED visit (11.0% vs 4.4%), and CS-related office visits in one year (6.9 vs 4.1; all P < 0.001). After adjusting for demographics, region, number of chronic conditions and the Charlson Comorbidity Index, the proportions of patients with any and with CS-related hospitalizations were 9.7% and 6.8% higher, respectively, among non-infectious diarrhea patients compared to those with without non-infectious diarrhea (P < 0.001). Unadjusted costs were significantly higher among non-infectious diarrhea patients vs those without non-infectious diarrhea. The non-infectious diarrhea group was also more costly, with adjusted mean annual costs of $81610, compared to $51719 in the group without non-infectious diarrhea (P < 0.001). CONCLUSION

  4. Erroneous diagnosis of small cell lung cancer based on small biopsies with far-reaching consequences: case report of a typical carcinoid tumor

    PubMed Central

    Krebs, Bettina; Kampe, Sandra; Theegarten, Dirk; Aigner, Clemens

    2017-01-01

    Although neuroendocrine tumors (NETs) of the lung are frequently discussed together, carcinoids are very different from high-grade small cell lung carcinoma (SCLC). SCLC is found in heavy-smoking, older patients, whereas smoking is not strongly associated with carcinoid tumors. We present the case of a 46-year-old never smoking woman who was misdiagnosed with SCLC. The patient was not responsive to radio-chemotherapy plus prophylactic cranial irradiation (PCI); she had a typical carcinoid (TC) tumor according to the final pathology report. We aim to demonstrate that diagnosis of SCLC based on cytology or small biopsy specimens must be scrutinized when the clinical constellation is unusual, or when the follow-up assessment shows no response to systemic treatment. PMID:28275490

  5. Carcinoid tumor of the lung with massive ossification: report of a case showing the evidence of osteomimicry and review of the literature.

    PubMed

    Tsubochi, Hiroyoshi; Endo, Shunsuke; Oda, Yoshinao; Dobashi, Yoh

    2013-01-01

    Carcinoid tumor is one of the commonly encountered primary pulmonary neoplasms. Although it has been known to be accompanied by calcification and/or ossification, presentation with a large ossified mass is rare. We describe here the case of a 29-year-old female with the radiological finding of a single bony nodular lesion. Pathological examination of the surgically resected specimen led to the diagnosis of carcinoid tumor of the lung with massive ossification. Although histological features showed the tumor of low grade malignancy, subcarinal and right hilar lymph nodes were found to be positive for metastasis. Further immunohistochemical analysis revealed that the tumor cells expressed the osteogenic inducer protein, bone morphogenic protrein-2 [BMP-2] and osteoblastic marker protein, osteocalcin. We interpreted this to mean that the carcinoid tumor cells had acquired an osteoblastic phenotype and had subsequently developed marked intratumoral ossification. The relevant literature is reviewed and possible mechanisms of tumor-related osteogenesis are discussed.

  6. Temsirolimus and Bevacizumab in Treating Patients With Advanced Endometrial, Ovarian, Liver, Carcinoid, or Islet Cell Cancer

    ClinicalTrials.gov

    2017-07-10

    Adult Hepatocellular Carcinoma; Advanced Adult Hepatocellular Carcinoma; Endometrial Serous Adenocarcinoma; Localized Non-Resectable Adult Liver Carcinoma; Lung Carcinoid Tumor; Malignant Pancreatic Gastrinoma; Malignant Pancreatic Glucagonoma; Malignant Pancreatic Insulinoma; Malignant Pancreatic Somatostatinoma; Metastatic Digestive System Neuroendocrine Tumor G1; Ovarian Carcinosarcoma; Ovarian Endometrioid Adenocarcinoma; Ovarian Seromucinous Carcinoma; Ovarian Serous Surface Papillary Adenocarcinoma; Pancreatic Alpha Cell Adenoma; Pancreatic Beta Cell Adenoma; Pancreatic Delta Cell Adenoma; Pancreatic G-Cell Adenoma; Pancreatic Polypeptide Tumor; Recurrent Adult Liver Carcinoma; Recurrent Digestive System Neuroendocrine Tumor G1; Recurrent Fallopian Tube Carcinoma; Recurrent Ovarian Carcinoma; Recurrent Pancreatic Neuroendocrine Carcinoma; Recurrent Primary Peritoneal Carcinoma; Recurrent Uterine Corpus Carcinoma; Regional Digestive System Neuroendocrine Tumor G1; Stage IIIA Fallopian Tube Cancer; Stage IIIA Ovarian Cancer; Stage IIIA Primary Peritoneal Cancer; Stage IIIA Uterine Corpus Cancer; Stage IIIB Fallopian Tube Cancer; Stage IIIB Ovarian Cancer; Stage IIIB Primary Peritoneal Cancer; Stage IIIB Uterine Corpus Cancer; Stage IIIC Fallopian Tube Cancer; Stage IIIC Ovarian Cancer; Stage IIIC Primary Peritoneal Cancer; Stage IIIC Uterine Corpus Cancer; Stage IV Fallopian Tube Cancer; Stage IV Ovarian Cancer; Stage IV Primary Peritoneal Cancer; Stage IVA Uterine Corpus Cancer; Stage IVB Uterine Corpus Cancer; Uterine Carcinosarcoma

  7. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    PubMed Central

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  8. [Neuroendocrine tumors of the stomach (gastric carcinoids) are on the rise: good prognosis with early detection].

    PubMed

    Scherübl, H; Faiss, S; Jahn, H U; Liehr, R-M; Schwertner, C; Steinberg, J; Stölzel, U; Weinke, T; Zimmer, T; Klöppel, G

    2009-07-01

    Neuroendocrine tumors (NET) of the stomach are on the rise. In the United States they have increased about tenfold in the last 35 years. Prognosis has been much improved over the last three to four decades. Nowadays most of such NETs are diagnosed at an early stage. Quite often gastric NETs are found incidentally during a gastroscopy, performed for other reasons. Most of the asymptomatic, well differentiated gastric NETs are less than 2 cm in diameter. Conservative management and endoscopic surveillance is adequate for well differentiated, multifocal type 1 or type 2 gastric NETs (gastric carcinoids) of 10-20 mm , unless they are angio-invasive, have infiltrated into the muscularis propria or have metastasized. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. Surgery is, however, indicated for all NETs larger than 20 mm. For optimal management tumor biology, type and stage of the neoplasm as well as the individual situation of the patient have to be taken into account. Most of the patients can be treated conservatively and be followed up with endoscopic surveillance.

  9. Metastatic type 1 gastric carcinoid: A real threat or just a myth?

    PubMed Central

    Grozinsky-Glasberg, Simona; Thomas, Dimitrios; Strosberg, Jonathan R; Pape, Ulrich-Frank; Felder, Stephan; Tsolakis, Apostolos V; Alexandraki, Krystallenia I; Fraenkel, Merav; Saiegh, Leonard; Reissman, Petachia; Kaltsas, Gregory; Gross, David J

    2013-01-01

    AIM: To describe disease characteristics and treatment modalities in a group of rare patients with metastatic gastric carcinoid type 1 (GCA1). METHODS: Information on clinical, biochemical, radiological, histopathological findings, the extent of the disease, as well as the use of different therapeutic modalities and the long-term outcome were recorded. Patients’ data were assessed at presentation, and thereafter at 6 to 12 monthly intervals both clinically and biochemically, but also endoscopically and histopathologically. Patients were evaluated for the presence of specific symptoms; the presence of autoimmune disorders and the presence of other gastrointestinal malignancies in other family members were also recorded. The evaluation of response to treatment was defined using established WHO criteria. RESULTS: We studied twenty consecutive patients with a mean age of 55.1 years. The mean follow-up period was 83 mo. Twelve patients had regional lymph node metastases and 8 patients had liver metastases. The primary tumor mean diameter was 20.13 ± 10.83 mm (mean ± SD). The mean Ki-67 index was 6.8% ± 11.2%. All but one patient underwent endoscopic or surgical excision of the tumor. The disease was stable in all but 3 patients who had progressive liver disease. All patients remained alive during the follow-up period. CONCLUSION: Metastatic GCA1 carries a good overall prognosis, being related to a tumor size of ≥ 1 cm, an elevated Ki-67 index and high serum gastrin levels. PMID:24379587

  10. Goblet cell carcinoids of the appendix: Tumor biology, mutations and management strategies

    PubMed Central

    Shenoy, Santosh

    2016-01-01

    Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers. Goblet cell carcinoids (GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features. This review focuses on the current diagnostic procedures, pathogenesis, possible signaling mechanisms and treatment options for GCC. Perspectives for future research are discussed. The tumor likely arises from pluripotent intestinal epithelial crypt base stem cells. Previous findings of Notch signaling as a tumor suppressor in Neuroendocrine tumors may have a similar role in this tumor too. Loss of Notch signaling may be the driver mutation with other successive downstream mutations likely favors them into progressing and behavior similar to poorly differentiated adenocarcinoma with minimal neuroendocrine differentiation. A multidisciplinary approach is suggested for optimal outcomes. Surgery remains the main treatment modality. Simple appendectomy may be sufficient in early stages while right hemicolectomy is recommended for advanced tumors. Cytoreductive surgery with heated intraperitoneal chemotherapy may improve survival in a select few with metastatic peritoneal disease. These tumors have an unpredictable behavior even in early stages and local recurrence and delayed metastases may be seen. Lifelong surveillance is warranted. PMID:27830037

  11. Laparoscopic antrectomy: a safe and definitive treatment in managing type 1 gastric carcinoids.

    PubMed

    Jenny, Hillary E; Ogando, Paul A; Fujitani, Kenji; Warner, Richard R P; Divino, Celia M

    2016-04-01

    Treatment for type 1 gastric carcinoid (T1GC) includes esophagogastroduodenoscopy (EGD), polypectomy, and antrectomy, but few studies compare outcomes. This study assessed risk-benefit ratio to determine the most effective treatment for T1GC. A retrospective review of 52 T1GC patients (ages 30 to 88 years; 77% female) presenting to Mount Sinai Medical Center between 2004 and 2012 was conducted. Patient demographics, procedures, and outcomes were reviewed, and patient satisfaction was assessed using a phone-administered validated questionnaire. Data were analyzed using SPSS version 20 software. Average EGDs needed per follow-up year was significantly lower for antrectomy than polypectomy or EGD surveillance (.395 vs 1.038 vs 1.380, P = .002). Antrectomy patients exhibited decreased recurrence risk than polypectomy patients (11% vs 44%, P = .049), despite longer follow-up time (6.10 vs 4.39 years, P = .023). Antrectomy treats T1GC with lower recurrence risk and less postintervention monitoring, whereas allowing patients to avoid the discomfort of repeated EGD surveillance and anxiety over a lingering condition. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. Treatment of the carcinoid syndrome with the longacting somatostatin analogue lanreotide: a prospective study in 39 patients.

    PubMed Central

    Ruszniewski, P; Ducreux, M; Chayvialle, J A; Blumberg, J; Cloarec, D; Michel, H; Raymond, J M; Dupas, J L; Gouerou, H; Jian, R; Genestin, E; Bernades, P; Rougier, P

    1996-01-01

    BACKGROUND: Somatostatin analogues effectively control flushing and diarrhoea in patients with the carcinoid syndrome. The octapeptide lanreotide is available in slow release form, which could eliminate the necessity of twice a day injections as with octreotide. PATIENTS AND METHODS: 39 patients with carcinoid syndrome were included in a prospective multicentre study. Patients received lanreotide 30 mg intramuscularly every 14 days for six months. The number and intensity of flushing episodes and bowel movements, urinary 5 hydroxy-indolacetic acid (5 HIAA) concentrations, and variations of tumour mass were recorded. RESULTS: After one month of treatment, flushing episodes (median (range)) decreased significantly (3 (0.3-24) episodes per day v 1 (0-15), p = 0.04) and completely resolved in 39% of the patients. A significant decrease was seen in the number of bowel movements and discomfort related to diarrhoea. Urinary 5 HIAA concentrations were unchanged in 57% of the patients and decreased in 18%. After six months of treatment, the actuarial proportions of patients with at least a 50% decrease in the number of flushing episodes and bowel movements were 54% and 56%, respectively. Forty two per cent of the patients who were treated for six months had at least a 50% reduction in 5 HIAA values. No clear signs of regression of tumours were seen in any of the patients. Lanreotide was well tolerated despite transient mild pain or erythema at the injection site in 25% of the patients. Biliary lithiasis appeared in two patients after six months of lanreotide. CONCLUSION: Lanreotide, 30 mg intramuscularly every other week, is an effective and convenient treatment in patients with the carcinoid syndrome. PMID:8977344

  13. Overexpression of PY1289-HER3 in sporadic pulmonary carcinoid from patients bearing MEN1 gene variants

    PubMed Central

    LATTANZIO, ROSSANO; VESCHI, SERENA; ACETO, GITANA MARIA; CURIA, MARIA CRISTINA; CAMA, ALESSANDRO; DE LELLIS, LAURA; FANTINI, FABIANA; ANGELUCCI, DOMENICO; IACOBELLI, STEFANO; PIANTELLI, MAURO; BATTISTA, PASQUALE

    2016-01-01

    The present study aimed to investigate the expression of human epidermal growth factor receptors (HERs) (HER1/HER2/HER3/HER4) and their phosphorylated forms (p-HER1/p-HER2/p-HER3/p-HER4) in pulmonary carcinoids (PCs). HER and p-HER protein expression was assessed by immunohistochemistry on tissue microarrays in 37 specimens of sporadic PCs, 29 typical carcinoids (TCs) and 8 atypical carcinoids (ACs). When compared with the ACs, the TCs did not exhibit any differences in terms of HER/p-HER expression. The tumors of this study have previously been characterized for the expression of menin and the mutational status of menin 1 (MEN1), a gene strongly implicated in the pathogenesis of PCs. In the present study, it was found that the cytoplasmic (‘disarrayed’), but not nuclear (‘arrayed’) expression of menin was positively correlated with HER3 (P=0.004), HER4 (P=0.015), p-HER1 (P=0.005), p-HER3 (P<0.001), and p-HER4 (P=0.001) expression. Moreover, HER3 and p-HER3 were found to be significantly more expressed in PCs with MEN1 variants, than in tumors with MEN1 wild-type (P=0.000 and P=0.025, respectively). These findings suggest the potential clinical use of HER inhibitors in the treatment of patients with PCs, particularly for individuals with p-HER3-positive PCs harboring MEN1 gene variants. PMID:27347164

  14. [Unilateral hyperlucent lung induced by a carcinoid tumor: comments on the differential diagnosis and mechanisms of hypoperfusion].

    PubMed

    Schmitz, N; Bugnet, A-S; Demian, M; Massard, G; De Blay, F; Pauli, G

    2005-04-01

    We report the case of a 35-year-old woman in whom a systematic thoracic x-ray led to the diagnosis of unilateral hyperlucent lung due to a carcinoid tumor obstructing the main left bronchus almost completely. Injected computed tomography permitted diagnosis of left lung hypoperfusion and visualization of the tumor. After enlarged inferior left lobar resection, normal perfusion was observed six months later on the isotopic lung perfusion scan. Other reported causes of unilateral hyperlucent lung are discussed as well as pathophysiological mechanisms of lung hypoperfusion and hypoxic vasoconstriction.

  15. Atypical carcinoid and large cell neuroendocrine carcinoma of the lung: a proteomic dataset from formalin-fixed archival samples

    PubMed Central

    Tanca, Alessandro; Addis, Maria Filippa; Pisanu, Salvatore; Abbondio, Marcello; Pagnozzi, Daniela; Eccher, Albino; Rindi, Guido; Cossu-Rocca, Paolo; Uzzau, Sergio; Fanciulli, Giuseppe

    2016-01-01

    Here we present a dataset generated using formalin-fixed paraffin-embedded archival samples from two rare lung neuroendocrine tumor subtypes (namely, two atypical carcinoids, ACs, and two large-cell neuroendocrine carcinomas, LCNECs). Samples were subjected to a shotgun proteomics pipeline, comprising full-length protein extraction, SDS removal through spin columns, in solution trypsin digestion, long gradient liquid chromatography peptide separation and LTQ-Orbitrap mass spectrometry analysis. A total of 1260 and 2436 proteins were identified in the AC and LCNEC samples, respectively, with FDR <1%. MS data are available in the PeptideAtlas repository at http://www.peptideatlas.org/PASS/PASS00375. PMID:27054153

  16. Hypokalaemia: common but not always benign. Severe, persistent hypokalaemia secondary to ectopic ACTH from a carcinoid tumour.

    PubMed

    Mahmood, Muhammad Muzaffar; John, Kurien

    2012-11-14

    Hypokalaemia is a common and often benign observation. There is usually an obvious underlying cause for the anomaly. However, hypokalaemia can very rarely be the sole presentation of a more sinister disease. A high index of suspicion and a systematic approach are therefore required to avoid delays in the management, especially in the context of presentation to a medical team. We present a case of a patient with severe and persistent hypokalaemia due to ectopic adrenocorticotropic hormone (ACTH) secretion secondary to a carcinoid tumour. The case report is followed by a brief discussion on the approach to the management of hypokalaemia and additional tests to confirm ectopic ACTH.

  17. [Carcinoid tumours of the lung and definition of the medico-legal term "lung cancer" used in the list of occupational disease in Germany--results of the German Mesothelioma Register].

    PubMed

    Neumann, V; Fischer, M; Tannapfel, A

    2008-09-01

    Carcinoid tumours are considered to be malignant epithelial tumours according to the recent WHO classification. This study is based on the examinations of tissue from 108 patients with carcinoid tumours. Our data agree with those of other studies: carcinoid tumours developed mainly in the right lung (40 %) and the lower lobe (30 %), mean age was 56 years, typical carcinoid tumours (74 %) predominated, comparatively high proportion of females (32 %), the mean latency period after asbestos exposure - assuming asbestos as one causal factor - was 35 years. A higher incidence of carcinoid tumours (1.3 %) in the collective of the mesothelioma register compared to the incidence in the collective of all lung carcinomas (1 - 2 %) was not observed. The increased pulmonary asbestos burden analysed in 26 % of patients is explained by the exposure-dependent selection of patients in the register. So far, no association between smoking habits or exposure to other, i. e., occupational pollutions and the development of carcinoid tumours could be established. In the list of occupational diseases (No. 4104) the term "lung cancer" is used without further specification. Thus the following question remains open for discussion: does the term "lung cancer" include carcinoid tumours such as malignant epithelial lung tumours, or is it restricted to the common subtypes such as small cell carcinoma, squamous cell carcinoma, adenocarcinoma, large cell carcinoma with regard to occupational disease and compensation?

  18. Everolimus as a new potential antiproliferative agent in aggressive human bronchial carcinoids.

    PubMed

    Zatelli, Maria Chiara; Minoia, Mariella; Martini, Chiara; Tagliati, Federico; Ambrosio, Maria Rosaria; Schiavon, Marco; Buratto, Mattia; Calabrese, Fiorella; Gentilin, Erica; Cavallesco, Giorgio; Berdondini, Lisa; Rea, Federico; degli Uberti, Ettore C

    2010-09-01

    Bronchial carcinoids (BCs) are rare tumors originating from endocrine cells dispersed in the respiratory epithelium. It has been previously demonstrated that everolimus, or RAD001, an mTOR inhibitor, has potent antiproliferative effects in human endocrine tumors. Our aim was to evaluate the possible antiproliferative effects of everolimus in human BCs in primary culture. We collected 24 BCs that were dispersed in primary cultures, treated without or with 1 nM-1 muM everolimus, 10 nM SOM230 (pasireotide, a somatostatin receptor multiligand), and/or 50 nM IGF1. Cell viability was evaluated after 48 h, and chromogranin A (CgA) as well as vascular endothelial growth factor (VEGF) secretion was assessed after 8 h incubation. Somatostatin receptors, mTOR, and AKT expression were investigated by quantitative PCR. We found that in 15 cultures (67.5%), everolimus significantly reduced cell viability (by approximately 30%; P<0.05 versus control), inhibited p70S6K activity (-30%), and blocked IGF1 proliferative effects. Everolimus also significantly reduced CgA (by approximately 20%) and VEGF (by approximately 15%) secretion. Cotreatment with SOM230 did not exert additive effects on cell viability and secretory activity. AKT expression was similar in responder and nonresponder tissues, while mTOR expression was significantly higher in the responder group, which was characterized by higher CgA plasma levels and bigger tumors with higher mitotic index and angiogenesis. Our data demonstrate that everolimus reduces VEGF secretion and cell viability in BCs with a mechanism likely involving IGF1 signaling, suggesting that it might represent a possible medical treatment for BCs.

  19. Atypical carcinoid tumor of the lung: a surveillance, epidemiology, and end results database analysis.

    PubMed

    Steuer, Conor E; Behera, Madhusmita; Kim, Sungjin; Chen, Zhengjia; Saba, Nabil F; Pillai, Rathi N; Owonikoko, Taofeek K; Khuri, Fadlo R; Ramalingam, Suresh S

    2015-03-01

    Atypical carcinoid (AC) of the lung is a rare form of thoracic malignancy. The limited knowledge of its biology and outcome stems largely from small, single institution experiences. We analyzed the Surveillance, Epidemiology, and End Results database (SEER) to better understand the clinical characteristics of this disease. Demographic, treatment, and outcome data on all patients with pulmonary AC were obtained from the SEER database with 18 reporting sites from 1973 to 2010 using SEER*Stat 8.1.2. Statistical analysis was performed using SAS 9.3 (SAS Institute, Inc., Cary, NC). There were 947,463 patients diagnosed with lung and bronchus tumors in the SEER database, of which 441 had AC (0.05%). Median age of AC patients was 65 years; 69% were women and 87% of white ethnicity. Metastatic disease was present in 20% of patients at diagnosis. In terms of treatment, 78% of patients underwent resection and 12.5% received radiation. The overall 1-year and 3-year survival rates were 86% and 67%, respectively. The 3-year survival rates for distant (M1), regional (lymph node involvement), and localized (lung only) disease were 26% (13 of 50), 69% (50 of 72), and 85% (99 of 116), respectively. On univariate analysis, patients treated with surgery had reduced risk of death (hazard ratio, HR 0.19; p < 0.001), whereas radiation treatment was associated with increased risk of death (HR 2.45; p < 0.001). AC accounted for less than 1% of all lung cancers diagnosed and was more frequent in women. The best outcomes were observed with surgical resection for localized disease.

  20. Evaluation of diagnostic and prognostic significance of Ki-67 index in pulmonary carcinoid tumours.

    PubMed

    Clay, V; Papaxoinis, G; Sanderson, B; Valle, J W; Howell, M; Lamarca, A; Krysiak, P; Bishop, P; Nonaka, D; Mansoor, W

    2017-05-01

    Pulmonary carcinoid (PC) tumours are classified as either typical (TC) or atypical (AC) according to mitotic index (MI) and presence of necrosis. The aim of this study was to analyse the diagnostic and prognostic values of the Ki-67 index in PC. Between January 2001 and March 2015, we evaluated 94 consecutive patients with a confirmed diagnosis of TC (n = 75) or AC (n = 19) at our institution. Diagnostic histology was centrally reviewed by a local expert neuroendocrine pathologist, with assessment of Ki-67, MI, and necrosis. Median patient follow-up was 35 months. Eighty-four patients who underwent curative surgical resection were included in the survival analysis for identification of prognostic factors. Ki-67 index showed high diagnostic accuracy to predict histological subtype when assessed by receiver operator characteristic curves with an area under the curve of 0.923 (95% CI 0.852-0.995, p < 0.001). Multivariate analysis showed that MI, Ki-67 index, and the presence or absence of necrosis were independent prognostic factors for relapse-free survival. Combination of MI, Ki-67, and necrosis led to the classification of patients into four different prognostic groups (very low, low, intermediate, and high risks of relapse). The current study proposes the incorporation of Ki-67 index in the prognostic classification of PC tumours. Due to the limited number of patients and length of follow-up, the current model needs validation by larger cohort studies. Nevertheless, our results suggest that Ki-67 index and MI have continuous effect on prognosis. Prognostic models incorporating multiple cutoffs of Ki-67 and MI might better predict outcome and inform clinical decisions.

  1. Incremental value of live/real time three-dimensional transthoracic echocardiography over the two-dimensional technique in assessing carcinoid heart disease involving the aortic valve.

    PubMed

    Bulur, Serkan; Hsiung, Ming C; Nanda, Navin C; Hardas, Shalaka; Mohamed, Ahmed; ElKaryoni, Ahmed; Srialluri, Swetha; Barssoum, Kirolos; Elsayed, Mahmoud; Wei, Jeng; Yin, Wei-Hsian

    2016-11-01

    We present a case of an adult with metastatic carcinoid heart disease, in whom live/real time three-dimensional transthoracic echocardiography provided incremental value over two-dimensional transthoracic echocardiography in assessing involvement of the aortic valve. © 2016, Wiley Periodicals, Inc.

  2. Concurrent primary carcinoid tumor arising within mature teratoma and clear cell renal cell carcinoma in the horseshoe kidney: report of a rare case and review of the literature.

    PubMed

    Sun, Ke; You, Qihan; Zhao, Ming; Yao, Hongtian; Xiang, Hua; Wang, Lijun

    2013-01-01

    Primary carcinoid tumor arising in a mature teratoma of the horseshoe kidney is exceptionally rare and only 4 such cases have been reported in the world literature to date. The simultaneous occurrence of different subtypes of renal cell carcinoma (RCC) or RCC coexistence with non-RCC neoplasms from the same kidney is unusual and infrequently reported. Herein we report a case of primary carcinoid tumor arising within mature teratoma, concurrent with a clear cell RCC in the horseshoe kidney of a 37-year-old man. Histologically, both the carcinoid tumor and clear cell RCC demonstrated the characteristic morphology in their classic forms. In addition to the carcinoid tumor, the mature teratoma consisted of variably sized, large cystic spaces lined by cytologically bland mucinous columnar epithelium, pseudostratified columnar epithelium, ciliated epithelium and mature smooth muscle fibers were also identified within the cystic wall. Furthermore, foci of round, small nodules composed of mature prostatic acinus were noted in the teratoma which was confirmed by exhibiting strong immunoreactivity for prostate specific antigen. The present case serves to expand the histologic component that may be encountered in the mature terotoma of the kidney and further broadens the spectrum of primary tumors occurring in the horseshoe kidney.

  3. Evaluation of the natural killer cell-interferon system in patients with mid-gut carcinoid tumours treated with leucocyte interferon.

    PubMed Central

    Funa, K; Alm, G V; Rönnblom, L; Oberg, K

    1983-01-01

    Nine patients with mid-gut carcinoid tumours received leucocyte interferon (IFN) i.m. daily for 90 days. Six patients clearly ameliorated in symptoms typical of the carcinoid syndrome (flushing, diarrhoea, asthma) which correlated with reduced serum levels of tumour related polypeptides and urinary output of 5-hydroxyindole acetic acid (5-HIAA). Before IFN treatment, peripheral blood mononuclear leucocytes (PBLs) from carcinoid patients showed markedly deficient production of pH 2 labile IFN-alpha in response to Staphylococcus aureus Cowan I (SACoI) in vitro. In contrast, IFN-alpha responses to the inducers Sendai virus and beta-haemolytic streptococcus group G and IFN-gamma responses to Lens culinaris lectin and concanavalin A were normal. Also, basal and in vitro IFN enhanced natural killer (NK) cell activity and T cell mitogen-induced cell proliferation were similar in patients and controls. During 90 days of IFN therapy, SACoI-induced IFN responses became entirely undetectable. There were transient declines at 1 and 30 days in IFN responses to the other IFN inducers, of mitogen-induced lymphocyte proliferation and of basal NK activities. The increments of NK cell activities after in vitro IFN exposure were clearly decreased in IFN treated patients, suggesting in vivo activation of these cells. Thus, the results demonstrate one remarkable abnormality in carcinoid patients: a deficient IFN response to SACoI and a clear influence of IFN therapy on several parameters of the IFN-NK system. PMID:6616963

  4. Concurrent primary carcinoid tumor arising within mature teratoma and clear cell renal cell carcinoma in the horseshoe kidney: report of a rare case and review of the literature

    PubMed Central

    Sun, Ke; You, Qihan; Zhao, Ming; Yao, Hongtian; Xiang, Hua; Wang, Lijun

    2013-01-01

    Primary carcinoid tumor arising in a mature teratoma of the horseshoe kidney is exceptionally rare and only 4 such cases have been reported in the world literature to date. The simultaneous occurrence of different subtypes of renal cell carcinoma (RCC) or RCC coexistence with non-RCC neoplasms from the same kidney is unusual and infrequently reported. Herein we report a case of primary carcinoid tumor arising within mature teratoma, concurrent with a clear cell RCC in the horseshoe kidney of a 37-year-old man. Histologically, both the carcinoid tumor and clear cell RCC demonstrated the characteristic morphology in their classic forms. In addition to the carcinoid tumor, the mature teratoma consisted of variably sized, large cystic spaces lined by cytologically bland mucinous columnar epithelium, pseudostratified columnar epithelium, ciliated epithelium and mature smooth muscle fibers were also identified within the cystic wall. Furthermore, foci of round, small nodules composed of mature prostatic acinus were noted in the teratoma which was confirmed by exhibiting strong immunoreactivity for prostate specific antigen. The present case serves to expand the histologic component that may be encountered in the mature terotoma of the kidney and further broadens the spectrum of primary tumors occurring in the horseshoe kidney. PMID:24228123

  5. Ghrelin-producing well-differentiated neuroendocrine tumor (carcinoid) of tailgut cyst. Morphological, immunohistochemical, ultrastructural, and RT-PCR study of a case and review of the literature.

    PubMed

    La Rosa, Stefano; Boni, Luigi; Finzi, Giovanna; Vigetti, Davide; Papanikolaou, Nikolaos; Tenconi, Silvia Maria; Dionigi, Gianlorenzo; Clerici, Moira; Garancini, Silvana; Capella, Carlo

    2010-09-01

    Well-differentiated neuroendocrine tumors (carcinoids) arising in the presacral space are rare neoplasms that can arise in association with either sacrococcygeal teratomas or tailgut cysts. Although tumors arising in tailgut cysts are more frequent than those associated with teratomas, they are still very rare, and only 13 cases have been reported in the literature. We describe the first case of a carcinoid composed of ghrelin-producing cells arising in a tailgut cyst. Ghrelin production was demonstrated using immunohistochemistry, electron microscopy, and reverse transcription-polymerase chain reaction methods. A 73-year-old woman with back and pelvic pain was found to have a presacral mass histologically diagnosed, on needle biopsy, as a well-differentiated neuroendocrine tumor. Workup did not show another primary tumor or metastatic disease. The patient underwent laparoscopic resection of the mass, and the pathological diagnosis of the surgical specimen was of a tailgut cyst-associated carcinoid composed of ghrelin-producing cells. In addition, we have accurately reviewed the literature on presacral carcinoids, associated or unassociated with tailgut cysts, to give the reader a comprehensive overview of these very rare tumor types.

  6. Incidental finding of carcinoid tumor on Meckel’s diverticulum: case report and literature review, should prophylactic resection be recommended?

    PubMed Central

    2014-01-01

    Meckel’s diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and is caused by incomplete obliteration of the vitelline duct during intrauterine life. MD affects less than 2% of the population. In most cases, MD is asymptomatic and the estimated average complication risk of MD carriers, which is inversely proportional to age, ranges between 2% and 4%. The most common MD-related complications are gastrointestinal bleeding, intestinal obstruction and acute phlogosis. Excision is mandatory in the case of symptomatic diverticula regardless of age, while surgical treatment for asymptomatic diverticula remains controversial. According to the majority of studies, the incidental finding of MD in children is an indication for surgical resection, while the management of adults is not yet unanimous. In this case report, we describe the prophylactic resection of an incidentally detected MD, which led to the removal of an occult mucosal carcinoid tumor. In literature, the association of MD and carcinoid tumor is reported as a rare finding. Even though the strategy for adult patients of an incidental finding of MD during surgery performed for other reasons divides the experts, we recommend prophylactic excision in order to avoid any further risk. PMID:24884768

  7. Incidental finding of carcinoid tumor on Meckel's diverticulum: case report and literature review, should prophylactic resection be recommended?

    PubMed

    Caracappa, Daniela; Gullà, Nino; Lombardo, Francesco; Burini, Gloria; Castellani, Elisa; Boselli, Carlo; Gemini, Alessandro; Burattini, Maria Federica; Covarelli, Piero; Noya, Giuseppe

    2014-05-08

    Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and is caused by incomplete obliteration of the vitelline duct during intrauterine life. MD affects less than 2% of the population. In most cases, MD is asymptomatic and the estimated average complication risk of MD carriers, which is inversely proportional to age, ranges between 2% and 4%. The most common MD-related complications are gastrointestinal bleeding, intestinal obstruction and acute phlogosis. Excision is mandatory in the case of symptomatic diverticula regardless of age, while surgical treatment for asymptomatic diverticula remains controversial. According to the majority of studies, the incidental finding of MD in children is an indication for surgical resection, while the management of adults is not yet unanimous. In this case report, we describe the prophylactic resection of an incidentally detected MD, which led to the removal of an occult mucosal carcinoid tumor. In literature, the association of MD and carcinoid tumor is reported as a rare finding. Even though the strategy for adult patients of an incidental finding of MD during surgery performed for other reasons divides the experts, we recommend prophylactic excision in order to avoid any further risk.

  8. Residual ovarian tissue mimicking malignancy in a patient with mucinous carcinoid tumor of the ovary. A case report.

    PubMed

    Burke, M; Talerman, A; Carlson, J A; Bibbo, M

    1997-01-01

    Mucinous carcinoid tumor of the ovary is an uncommon lesion in reproductive-age women. If a patient status post total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO) for this tumor presents with a pelvic mass, recurrence of the tumor must be considered, as must the presence of residual ovarian tissue producing physiologic cysts with mass effect, termed the "ovarian remnant syndrome." Benign ovarian follicle cysts may have cellular atypia and mimic malignancy. A female, one year status post TAH-BSO for mucinous carcinoid tumor of the ovary, presented with pelvic mass. Clinical and radiologic evidence supported the diagnosis of recurrent tumor. Aspiration biopsy material was compatible with malignancy, and immunocytochemical stains supported a neuroendocrine origin of the cells. Surgical excision and histologic examination of the mass revealed ovarian tissue with features of corpus luteum and follicular cyst. Numerous pitfalls exist when considering an unusual tumor. While the patient's history, clinical impressions and immunocytochemistry may strongly suggest malignancy, more common benign entities may mimic malignancy and should be considered in the differential diagnosis.

  9. Thymic and Bronchial Carcinoid Tumors in Multiple Endocrine Neoplasia Type 1: The Mayo Clinic Experience from 1977 to 2013.

    PubMed

    Singh Ospina, Naykky; Thompson, Geoffrey B; C Nichols, Francis; Cassivi, Stephen D; Young, William F

    2015-12-01

    The clinical features of thymic carcinoid (TC) and bronchial carcinoid (BC) tumors as part of multiple endocrine neoplasia type 1 (MEN1) have been rarely described and their importance in clinical practice is debated. The objective of this study was to describe the clinical presentation and outcome of this uncommon manifestation of MEN1 in a tertiary care center setting. We present the clinical features of patients with MEN1 and either TC or BC evaluated at the Mayo Clinic from 1977 to 2013. A total of 348 patients with MEN1 were evaluated and the prevalence of TC was 2.0% (n = 7) and of BC 4.9% (n = 17). The majority of the patients with BC were men (61%) diagnosed on routine screening (77%) and BC was not the confirmed cause of death in any patient. In contrast, TC patients were all men and during follow-up 43% died due to TC complications. We conclude that TC and BC tumors are uncommon, but important components of MEN1. BC were most commonly diagnosed during routine screening and associated with an indolent course. TC were predominantly seen in men and associated with a more aggressive behavior.

  10. Carcinoid tumor associated with adjacent dysplastic columnar epithelium in the renal pelvis: A case report and literature review.

    PubMed

    Kim, Sung Sun; Choi, Chan; Kang, Taek Won; Choi, Yoo Duk

    2016-01-01

    Carcinoid tumors are well documented in the pulmonary and gastrointestinal systems, but very rare in the urinary tract, especially in the renal pelvis. We report on a 60-year-old female patient who presented with left flank pain and fever. Abdominal computed tomography demonstrated a heterogeneously enhancing mass in the left renal pelvis and a stone at the left proximal ureter. Multiple parenchymal lesions were also observed, which were identified as uneven caliectasis displaying air-fluid levels and renal parenchymal atrophy. The patient underwent simple nephro-ureterectomy. Macroscopically, a polypoid mass was observed in the renal pelvis. Microscopically, the tumor revealed acinar, tubular, and solid pattern and was composed of small, monotonous and hyperchromatic cells. Lining epithelia in renal pelvis and ureter revealed columnar epithelia with dysplastic change. The tumor cells were positive for chromogranin A, synaptophysin, CD56, and focally positive for cytokeratin. Immunohistochemical staining of synaptophysin and chromogranin A highlighted the neuroendocrine cells in the columnar epithelium. Ki-67 (1:50; MIB-1) labeling index was less than 1% in the area with highest uptake. We report here a case of carcinoid tumor of the renal pelvis that was associated with adjacent dysplastic columnar epithelium. © 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  11. Carcinoid tumours of the lung and the 'PEPPS' approach: evaluation of preoperative bronchoscopic tumour debulking as preparation for subsequent parenchyma-sparing surgery.

    PubMed

    Neuberger, Michael; Hapfelmeier, Alexander; Schmidt, Michael; Gesierich, Wolfgang; Reichenberger, Frank; Morresi-Hauf, Alicia; Hatz, Rudolf A; Lindner, Michael

    2015-01-01

    Preoperative bronchoscopic tumour ablation has been suggested as a beneficial treatment for bronchopulmonary carcinoid tumours, although data regarding its effects and long-term outcome are lacking. In our case-matched cohort study with 208 patients with bronchopulmonary carcinoid tumours we investigated the role of preoperative bronchoscopic interventions before subsequent surgery and analysed the safety of this Procedure of Endobronchial Preparation for Parenchyma-sparing Surgery (PEPPS) based on metastasis and recurrence rates as well as survival data from 1991 to 2010. The subsequent surgery was classified into parenchyma-sparing procedures and classical lobectomies, bilobectomies and pneumonectomies. Data were obtained from the tumour registry and medical reports. Outcomes were the frequency of parenchyma-sparing surgery after bronchoscopic treatment as well as rates of metastasis, recurrence and survival. 132 of 208 carcinoids were located centrally. Among them, 77 patients could be recanalised preoperatively. After bronchoscopic preparation, the rate of subsequent parenchyma-sparing surgery methods was higher (p=0.021). The effect was measured by the number of segments removed. The 10-year survival rate was 89% (typical carcinoids) and 68% (atypical carcinoids), respectively. After applying PEPPS, long-term survival was slightly higher (p=0.23). Metastasis and recurrence rates showed no relevant differences between the bronchoscopically treated or non-treated groups, or between the two types of surgery classes or between the PEPPS and non-PEPPS groups. After preoperative bronchoscopic treatment, parenchyma-sparing surgery techniques can be applied more frequently. Furthermore, we detected no negative effects after PEPPS based on metastasis, recurrence and survival rates.

  12. Carcinoid tumours of the lung and the ‘PEPPS’ approach: evaluation of preoperative bronchoscopic tumour debulking as preparation for subsequent parenchyma-sparing surgery

    PubMed Central

    Neuberger, Michael; Hapfelmeier, Alexander; Schmidt, Michael; Gesierich, Wolfgang; Reichenberger, Frank; Morresi-Hauf, Alicia; Hatz, Rudolf A; Lindner, Michael

    2015-01-01

    Background Preoperative bronchoscopic tumour ablation has been suggested as a beneficial treatment for bronchopulmonary carcinoid tumours, although data regarding its effects and long-term outcome are lacking. Methods In our case-matched cohort study with 208 patients with bronchopulmonary carcinoid tumours we investigated the role of preoperative bronchoscopic interventions before subsequent surgery and analysed the safety of this Procedure of Endobronchial Preparation for Parenchyma-sparing Surgery (PEPPS) based on metastasis and recurrence rates as well as survival data from 1991 to 2010. The subsequent surgery was classified into parenchyma-sparing procedures and classical lobectomies, bilobectomies and pneumonectomies. Data were obtained from the tumour registry and medical reports. Outcomes were the frequency of parenchyma-sparing surgery after bronchoscopic treatment as well as rates of metastasis, recurrence and survival. Results 132 of 208 carcinoids were located centrally. Among them, 77 patients could be recanalised preoperatively. After bronchoscopic preparation, the rate of subsequent parenchyma-sparing surgery methods was higher (p=0.021). The effect was measured by the number of segments removed. The 10-year survival rate was 89% (typical carcinoids) and 68% (atypical carcinoids), respectively. After applying PEPPS, long-term survival was slightly higher (p=0.23). Metastasis and recurrence rates showed no relevant differences between the bronchoscopically treated or non-treated groups, or between the two types of surgery classes or between the PEPPS and non-PEPPS groups. Conclusions After preoperative bronchoscopic treatment, parenchyma-sparing surgery techniques can be applied more frequently. Furthermore, we detected no negative effects after PEPPS based on metastasis, recurrence and survival rates. PMID:26203359

  13. Effect of Valvular Surgery in Carcinoid Heart Disease: An Observational Cohort Study.

    PubMed

    Edwards, N C; Yuan, M; Nolan, O; Pawade, T A; Oelofse, T; Singh, H; Mehrzad, H; Zia, Z; Geh, J I; Palmer, D H; May, C J H; Ayuk, J; Shah, T; Rooney, S J; Steeds, R P

    2016-01-01

    Carcinoid heart disease (NET-CHD) is associated with the development of symptom-limited exercise capacity and high rates of morbidity and mortality. This study sought to determine the survival, cardiac function, and functional class following surgery. This was a retrospective observational cohort study between 2005 and 2015 at a European Centre of Excellence for Neuroendocrine Tumours, Queen Elizabeth Hospital Birmingham. England consisting of 62 consecutive patients referred to the NET-Cardiology Service. Subjects were assessed at referral using transthoracic echocardiography (with saline contrast) and transesophageal echocardiography, and 77% with confirmed NET-CHD underwent cardiovascular magnetic resonance imaging. Symptomatic patients with concomitant severe valvular dysfunction were referred for surgery with stable NET disease. Survival of patients with proven NET-CHD following medical and surgical treatments was measure. In total, 47/62 patients were diagnosed with NET-CHD. Thirty-two patients (68%) underwent surgery with bioprosthetic valve replacements in all subjects; tricuspid, n = 31; pulmonary, n = 30; mitral, n = 3; and aortic, n = 3. Four patients underwent concomitant coronary artery bypass grafting. There were 4 (13%) early post-operative deaths. One- and 2-y survival rates after surgery were 75 and 69% compared with 45 and 15% in un-operated patients. Post-operatively, functional class was improved (pre-New York Heart Association Classification [NYHA], 2.6 [0.5] vs post-NYHA, 1.7 [1.1]), P < .05, right-ventricular (RV) size was reduced (136 ml/m(2) [25] vs 71 ml/m(2) [7]; P < .01) with preserved RV ejection fraction (61% ± 9 vs 55% ± 10; P = .26). Valve surgery improved functional class and resulted in RV reverse remodelling with improved survival rates at 2 y compared with those not proceeding to operation. These data highlight the importance of close collaboration between NET clinicians, cardiology, and cardiothoracic surgery teams. Early

  14. Regional Gastrointestinal Transit Times in Patients With Carcinoid Diarrhea: Assessment With the Novel 3D-Transit System.

    PubMed

    Gregersen, Tine; Haase, Anne-Mette; Schlageter, Vincent; Gronbaek, Henning; Krogh, Klaus

    2015-07-30

    The paucity of knowledge regarding gastrointestinal motility in patients with neuroendocrine tumors and carcinoid diarrhea re-stricts targeted treatment. 3D-Transit is a novel, minimally invasive, ambulatory method for description of gastrointestinal motility. The system has not yet been evaluated in any group of patients. We aimed to test the performance of 3D-Transit in patients with carcinoid diarrhea and to compare the patients' regional gastrointestinal transit times (GITT) and colonic motility patterns with those of healthy subjects. Fifteen healthy volunteers and seven patients with neuroendocrine tumor and at least 3 bowel movements per day were inves-tigated with 3D-Transit and standard radiopaque markers. Total GITT assessed with 3D-Transit and radiopaque markers were well correlated (Spearman's rho = 0.64, P = 0.002). Median total GITT was 12.5 (range: 8.5-47.2) hours in patients versus 25.1 (range: 13.1-142.3) hours in healthy (P = 0.007). There was no difference in gastric emptying (P = 0.778). Median small intestinal transit time was 3.8 (range: 1.4-5.5) hours in patients versus 4.4 (range: 1.8-7.2) hours in healthy subjects (P = 0.044). Median colorectal transit time was 5.2 (range: 2.9-40.1) hours in patients versus 18.1 (range: 5.0-134.0) hours in healthy subjects (P = 0.012). Median frequency of pansegmental co-lonic movements was 0.45 (range: 0.03-1.02) per hour in patients and 0.07 (range: 0-0.61) per hour in healthy subjects (P = 0.045). Three-dimensional Transit allows assessment of regional GITT in patients with diarrhea. Patients with carcinoid diarrhea have faster than normal gastrointestinal transit due to faster small intestinal and colorectal transit times. The latter is caused by an increased frequency of pansegmental colonic movements.

  15. Regional Gastrointestinal Transit Times in Patients With Carcinoid Diarrhea: Assessment With the Novel 3D-Transit System

    PubMed Central

    Gregersen, Tine; Haase, Anne-Mette; Schlageter, Vincent; Gronbaek, Henning; Krogh, Klaus

    2015-01-01

    Background/Aims The paucity of knowledge regarding gastrointestinal motility in patients with neuroendocrine tumors and carcinoid diarrhea restricts targeted treatment. 3D-Transit is a novel, minimally invasive, ambulatory method for description of gastrointestinal motility. The system has not yet been evaluated in any group of patients. We aimed to test the performance of 3D-Transit in patients with carcinoid diarrhea and to compare the patients’ regional gastrointestinal transit times (GITT) and colonic motility patterns with those of healthy subjects. Methods Fifteen healthy volunteers and seven patients with neuroendocrine tumor and at least 3 bowel movements per day were investigated with 3D-Transit and standard radiopaque markers. Results Total GITT assessed with 3D-Transit and radiopaque markers were well correlated (Spearman’s rho = 0.64, P = 0.002). Median total GITT was 12.5 (range: 8.5–47.2) hours in patients versus 25.1 (range: 13.1–142.3) hours in healthy (P = 0.007). There was no difference in gastric emptying (P = 0.778). Median small intestinal transit time was 3.8 (range: 1.4–5.5) hours in patients versus 4.4 (range: 1.8–7.2) hours in healthy subjects (P = 0.044). Median colorectal transit time was 5.2 (range: 2.9–40.1) hours in patients versus 18.1 (range: 5.0–134.0) hours in healthy subjects (P = 0.012). Median frequency of pansegmental colonic movements was 0.45 (range: 0.03–1.02) per hour in patients and 0.07 (range: 0–0.61) per hour in healthy subjects (P = 0.045). Conclusions Three-dimensional Transit allows assessment of regional GITT in patients with diarrhea. Patients with carcinoid diarrhea have faster than normal gastrointestinal transit due to faster small intestinal and colorectal transit times. The latter is caused by an increased frequency of pansegmental colonic movements. PMID:26130638

  16. Effects of the single and combined treatment with dopamine agonist, somatostatin analog and mTOR inhibitors in a human lung carcinoid cell line: an in vitro study.

    PubMed

    Pivonello, Claudia; Rousaki, Panagoula; Negri, Mariarosaria; Sarnataro, Maddalena; Napolitano, Maria; Marino, Federica Zito; Patalano, Roberta; De Martino, Maria Cristina; Sciammarella, Concetta; Faggiano, Antongiulio; Rocco, Gaetano; Franco, Renato; Kaltsas, Gregory A; Colao, Annamaria; Pivonello, Rosario

    2016-09-29

    Somatostatin analogues and mTOR inhibitors have been used as medical therapy in lung carcinoids with variable results. No data are available on dopamine agonists as treatment for lung carcinoids. The main aim of the current study was to evaluate the effect of the combined treatment of somatostatin analogue octreotide and the dopamine agonist cabergoline with mTOR inhibitors in an in vitro model of typical lung carcinoids: the NCI-H727 cell line. In NCI-H727 cell line, reverse transcriptase-quantitative polymerase chain reaction and immunofluorescence were assessed to characterize the expression of the somatostatin receptor 2 and 5, dopamine receptor 2 and mTOR pathway components. Fifteen typical lung carcinoids tissue samples have been used for somatostatin receptor 2, dopamine receptor 2, and the main mTOR pathway component p70S6K expression and localization by immunohistochemistry. Cell viability, fluorescence-activated cell sorting analysis and western blot have been assessed to test the pharmacological effects of octreotide, cabergoline and mTOR inhibitors, and to evaluate the activation of specific cell signaling pathways in NCI-H727 cell line. NCI-H727 cell line expressed somatostatin receptor 2, somatostatin receptor 5 and dopamine receptor 2 and all mTOR pathway components at messenger and protein levels. Somatostatin receptor 2, dopamine receptor 2, and p70S6K (non phosphorylated and phosphorylated) proteins were expressed in most typical lung carcinoids tissue samples. Octreotide and cabergoline did not reduce cell viability as single agents but, when combined with mTOR inhibitors, they potentiate mTOR inhibitors effect after long-term exposure, reducing Akt and ERK phosphorylation, mTOR escape mechanisms, and increasing the expression DNA-damage-inducible transcript 4, an mTOR suppressor. In conclusion, the single use of octreotide and cabergoline is not sufficient to block cell viability but the combined approach of these agents with mTOR inhibitors

  17. Atypical or typical adrenocorticotropic hormone-producing pulmonary carcinoids and the usefulness of 11C-5-hydroxytryptophan positron emission tomography: two case reports.

    PubMed

    Wahlberg, Jeanette; Ekman, Bertil

    2013-03-19

    Pulmonary carcinoids associated with ectopic adrenocorticotropic hormone secretion have a good prognosis if histological examination shows typical pulmonary carcinoid and low proliferation, whereas a poor outcome is linked to atypical pulmonary carcinoid and high proliferation. Here we describe the diagnostic challenges to find the tumor in Cushing's syndrome secondary to ectopic adrenocorticotropic hormone secretion in two cases with an atypical and a typical pulmonary carcinoid, respectively. A 63-year-old Caucasian woman presented with aggressive clinical features related to Cushing's syndrome, having very high levels of urinary cortisol and circulating adrenocorticotropic hormone and cortisol. Magnetic resonance imaging showed no pituitary tumor, and bilateral inferior petrosal sinus sampling revealed no central peripheral ratio of adrenocorticotropic hormone. Computed tomography and 111Indium-pentetreoide somatostatin receptor scintigraphy could not visualize any ectopic tumor. The patient was referred for an 11C-5-hydroxytryptophan positron emission tomography, and a small 8mm nodule in her left lung was found. The tumor was removed via a lateral thoracic incision and wedge excision. The histological examination showed an atypical carcinoid with Ki-67 index of 9 to 10%, and an additional lobectomy was performed.The second patient, a 22-year-old Caucasian man, also presented with aggressive Cushing's syndrome, with very high urinary cortisol levels and increased circulating cortisol as well as adrenocorticotropic hormone levels. A magnetic resonance imaging scan of the pituitary showed no tumor, whereas a 12×9×14mm tumor was detected in the right lung on the primary computed tomography scan and no further investigation was performed. The tumor was removed via a lateral thoracic incision and wedge excision. A typical carcinoid with Ki-67 index of 1 to 2% was found and no further surgery was performed.After surgical removal, the biochemical disturbances

  18. Cushing's syndrome secondary to ectopic ACTH secretion from carcinoid tumor within an ovarian mature teratoma: a case report and review of the literature.

    PubMed

    Huang, Baoyou; Wu, Xueqing; Zhou, Qing; Hu, Yan; Zhao, Hongqin; Zhu, Hua; Zhang, Qian; Zheng, Feiyun

    2014-03-01

    A 46-year-old woman with Cushing's syndrome secondary to ectopic adrenocorticotropic hormone (ACTH) secretion caused by primary ovarian mature teratoma with carcinoid components was presented in our case. The patient manifested sustained hypercortisolemia without circadian rhythm and a lack of suppression of either low-dose dexamethasone suppression test (LDDST) or high-dose dexamethasone suppression test (HDDST). There was no evidence of a pituitary mass or secretion of other hormones. After careful clinical evaluation, no other tumor masses were found. Resection of the ovarian tumors led to sharp reduction of serum ACTH and cortisol concentrations. Immunohistochemistry showed positivity in CgA, Syn, CK, NSE. To the best of our knowledge, there are rare reports of an ACTH-secreting carcinoid components located in an ovarian mature teratoma, and bilateral ovarian mature teratoma makes it rarer.

  19. Type 1 and 2 gastric carcinoid tumors: long-term follow-up of the efficacy of treatment with a slow-release somatostatin analogue.

    PubMed

    Manfredi, Sylvain; Pagenault, Mael; de Lajarte-Thirouard, Anne-Sophie; Bretagne, Jean-François

    2007-11-01

    Little is known about the long-term results of treating gastric carcinoid tumors with a slow-release somatostatin analogue. We report three patients with type 1 and 2 gastric carcinoid tumors who were treated in the above mentioned way and followed for 27-50 months. In all cases, alternative endoscopic or surgical management was considered but deemed inappropriate. Treatment with a slow-release somatostatin analogue was begun in light of a favorable recent report. The result was regression or complete disappearance of macroscopic fundal tumors. No side-effects were reported and, most notably, none of the patients developed gallstones. This small study may help define the optimal duration, dose, and administration interval of the treatment. Slow-release somatostatin analogue is a safe and efficacious treatment for type 1 and 2 gastric carcinoid tumors, and can be used when tumors are growing rapidly. Slow-release somatostatin analogue represents an alternative to repeated endoscopic treatment or high-risk surgery.

  20. Use of radioguided surgery with [111In]-pentetreotide in the management of an ACTH-secreting bronchial carcinoid causing ectopic Cushing's syndrome.

    PubMed

    Grossrubatscher, E; Vignati, F; Dalino, P; Possa, M; Belloni, P A; Vanzulli, A; Bramerio, M; Marocchi, A; Rossetti, O; Zurleni, F; Loli, P

    2005-01-01

    Intraoperative [111In]-pentetreotide scintigraphy with a hand-held gamma detector probe has recently been proposed to increase the intraoperative detection rate of small neuroendocrine tumors and their metastases. We report a case of a 28-yr-old woman with ectopic Cushing's syndrome due to an ACTH-secreting bronchial carcinoid, in whom the use of radioguided surgery improved disease management. At presentation, radiolabeled pentetreotide scintigraphy was the only procedure able to detect the ectopic source of ACTH. After radiologic confirmation, the patient underwent removal of a bronchial carcinoid, with disease persistence. After surgery, pentetreotide scintigraphy showed pathologic uptake in the mediastinum not previously detected at surgery and only subsequently confirmed by radiologic studies. Despite a second thoracic exploration, hormonal, scintigraphic, and radiological evidence of residual disease persisted. Radioguided surgery was then performed using a hand-held gamma probe 48 h after iv administration of a tracer dose of radiolabeled [111In-DTPA-D-Phe1]-pentetreotide, which permitted detection and removal of multiple residual mediastinal lymph node metastases. Clinical and radiologic cure, with no evidence of tracer uptake at pentetreotide scintigraphy, was subsequently observed. The use of an intraoperative gamma counter appears a promising procedure in the management of metastatic ACTH-secreting bronchial carcinoids.

  1. Clinical outcomes for rectal carcinoid tumors according to a new (AJCC 7th edition) TNM staging system: a single institutional analysis of 122 patients.

    PubMed

    Kim, Min Sung; Hur, Hyuk; Min, Byung Soh; Baik, Seung Hyuk; Lee, Kang Young; Kim, Nam Kyu

    2013-06-01

    This study aimed to describe clinical outcomes of rectal carcinoids according to the 7th American Joint Council on Cancer (AJCC) TNM staging system. We retrospectively reviewed 122 patients who were treated for rectal carcinoids between 1995 and 2010. Among 122 patients, 81.2% (n = 99) were classified as stage I, 4.9% (n = 6) as stage II, 11.5% (n = 14) as stage III, and 2.5% (n = 3) as stage IV. Lymph node (LN) metastasis rates for pT1a, 1b, 2, and 3 stages were 1.2% (1/85), 16.7% (3/18), 0% (0/4) and 84.6% (11/13), respectively. The 5-year overall survival (OS) rate was 88.4%. The 5-year OS rates were estimated to be 100%, 80%, 51.4% and 0% for stage I, II, III, and IV, respectively (P < 0.001). The 5-year disease-free survival (DFS) rate was 82.3%. The 5-year DFS rates were estimated to be 97.7%, 62.5%, 17.1%, and 0% for stages I, II, III, and IV, respectively (P < 0.001). Using the new TNM stage, we confirmed a prognostic difference in LN metastasis rates, OS, and DFS for rectal carcinoids. In clinical practice, the new TNM stage can be very useful for predicting prognosis. Copyright © 2013 Wiley Periodicals, Inc.

  2. [A case of peritonitis carcinomatosa from goblet cell carcinoid of the appendix treated by intraperitoneal paclitaxel and systemic S-1 chemotherapy].

    PubMed

    Nakamura, Shingen; Kimura, Shigeaki; Kashima, Masahiro; Shichijo, Kana; Yoshida, Sumiko; Harada, Eiji; Matsushita, Takaya; Oshima, Yasushi; Tamaki, Yasutami; Horiuchi, Noriaki; Takeichi, Toshiaki; Fujimoto, Hiroshi; Masuda, Kazuhiko; Iwasaka, Naohito; Shinomiya, Sadao

    2008-12-01

    Goblet cell carcinoid of the appendix is a rare neoplasm and clinically tends to take a malignant course. Most cases are young and early stage, and the surgical strategy is available. But appropriate chemotherapy for inoperable cases with peritoneal dissemination is not established. A 77-year-old woman with a past history of appendectomy was admitted to our hospital complaining of abdominal fullness. Abdominal computed tomography showed massive ascites and slight contrast enhancement of appendix. A tumor was found by colonoscopic examination at the orifice of vermiform and was diagnosed pathologically as goblet cell carcinoid of the appendix. Laparoscopy showed multiple peritoneal dissemination. We performed intraperitoneal paclitaxel(PTX)administration at 70 mg/m(2) week without any resection of the tumor. Ascites were reduced immediately, but drug-induced interstitial pneumonia occurred due to PTX. After steroid therapy, we switched to systemic S-1 therapy. For about one year, her tumor was controlled but became worse thirteen months after diagnosis and died. It is thought that intraabdominal paclitaxel administration and systemic S-1 therapy can be one of appropriate forms of chemotherapy for inoperable peritoneal carcinomatosis from goblet cell carcinoid of appendix.

  3. The predictive value of mean platelet volume, plateletcrit and red cell distribution width in the differentiation of autoimmune gastritis patients with and without type I gastric carcinoid tumors.

    PubMed

    Tüzün, Ali; Keskin, Onur; Yakut, Mustafa; Kalkan, Cagdas; Soykan, Irfan

    2014-01-01

    Autoimmune gastritis is an autoimmune and inflammatory condition that may predispose to gastric carcinoid tumors or adenocarcinomas. The early diagnosis of these tumors is important in order to decrease morbidity and mortality. Platelet indices such as mean platelet volume and plateletcrit levels increase in inflammatory, infectious and malign conditions. The primary aim of this study was to explore wheter platelet indices and red cell distribution width have any predictive role in the discrimination of autoimmune gastritis patients with and without gastric carcinoid tumors. Also secondary aim of this study was to investigate whether any changes exist betwenn autoimmune gastritis and functional dyspepsia patients by means of platelet indices. Plateletcrit (0.22 ± 0.06 vs. 0.20 ± 0.03%, p < 0.001) and red cell distribution width (16.11 ± 3.04 vs. 13.41 ± 0.95%, p < 0.001) were significantly higher in autoimmune gastritis patients compared to control group. Receiver operating curve analysis suggested that optimum plateletcrit cut-off point was 0.20% (AUC: 0.646), and 13.95% as the cut off value for red cell distribution width (AUC: 0.860). Although plateletcrit (0.22 ± 0.06 vs. 0.21 ± 0.04%, p = 0.220) and mean platelet volume (8.94 ± 1.44 vs. 8.68 ± 0.89 fl, p = 0.265) were higher in autoimmune gastritis patients without carcinoid tumor compared to patients with carcinoid tumors, these parameters were not statistically significant. Changes in plateletcrit and red cell distribution width values may be used as a marker in the discrimination of autoimmune gastritis and fucntional dyspepsia patients but not useful in patients with gastric carcinoid tumor type I.

  4. The role of endogenous gastrin in the development of enterochromaffin-like cell carcinoid tumors in Mastomys natalensis: a study with the specific gastrin receptor antagonist AG-041R.

    PubMed Central

    Chiba, T.; Kinoshita, Y.; Sawada, M.; Kishi, K.; Baba, A.; Hoshino, E.

    1998-01-01

    We examined the effects of a newly synthesized gastrin receptor antagonist, AG-041R, on the growth of enterochromaffin-like (ECL) carcinoid tumors in Mastomys natalensis both in vitro and in vivo. AG-041R was as potent as the well known gastrin antagonist L365,260 in inhibiting not only the gastrin-induced release of histamine from but also histidine decarboxylase (HDC) gene expression in the ECL carcinoid tumor cells. AG-041R also inhibited gastrin-induced DNA synthesis and c-fos gene expression in the tumor cells. Furthermore, AG-041R significantly inhibited the growth of the transplanted Mastomys ECL carcinoid tumors in vivo. From these data, it is concluded that endogenous gastrin is involved in the growth of ECL carcinoid tumors in Mastomys natalensis. Moreover, AG-041R is shown to have a potential as an anti-neoplastic agent for ECL carcinoid tumor of the stomach. Images Figure 1 Figure 5 Figure 6 PMID:10461356

  5. Patient characteristics, treatment and survival in pulmonary carcinoid tumours: an analysis from the UK National Lung Cancer Audit

    PubMed Central

    Hobbins, Stephanie; West, Doug; Peake, Michael; Beckett, Paul; Woolhouse, Ian

    2016-01-01

    Objectives Pulmonary carcinoid (PC) is a rare tumour with good prognosis following surgical resection. However, little is known regarding patient characteristics and use of other treatments modalities. Our objective was to review patient characteristics, treatment and survival for patients with PC and contrast these results with other forms of non-small cell lung cancer (NSCLC). Setting Audit data from UK National Lung Cancer Audit (NLCA) 2008–2013. Participants 184 906 lung cancer cases were submitted to the NLCA. Outcome measures Primary outcome—survival rates between PC and NSCLC. Secondary outcome—differences in performance status, lung function and treatment modality between PC and NSCLC. Results PC histology was recorded in 1341 (0.73%) patients and non-carcinoid NSCLC histology in 162 959 (87.4%) cases. 91% of patients with PC had good performance status (Eastern Cooperative Oncology Group (ECOG) 0–1), compared with only 53% of NSCLC. 66% of PC had localised disease. Of all PC cases, 77% were treated with surgery, 6.2% received chemotherapy and 3.6% received radiotherapy, with the remainder treated with best supportive care. Overall 1-year and 3-year survival rates for PC were 92% and 84.7%, respectively. In contrast, 1-year and 3-year survival rates for NSCLC were 36.2% and 15.6%, However, 3-year survival for PC markedly decreased with worsening performance status and advanced disease to 23.8% for performance status ECOG 3–4 and 33.6% for stage IV disease. Conclusions In contrast to other forms of NSCLC, the majority of patients with PC present with good performance status, preserved lung function and early stage disease amenable to surgical resection. However, 1 in 5 patients with PC has metastatic disease which is associated with poor prognosis, as is poor performance status at presentation. We believe these data will help clinicians provide accurate prognostic predictions stratified according to patient characteristics at presentation, as

  6. [Autopsy case of von Recklinghausen's disease associated with lung cancer, gastrointestinal stromal tumor of the stomach, and duodenal carcinoid tumor].

    PubMed

    Satoh, Miki; Wakabayashi, Osamu; Araya, Yoshikazu; Jinushi, Eisei; Yoshida, Fumiaki

    2009-09-01

    A 58-year-old man with von Recklinghausen's disease was admitted for further investigation of right chest pain. Chest X-ray revealed multiple emphysematous bullae in both lungs and a tumor shadow in the right upper lobe. Bronchofiberscopy was performed, but an adequate specimen was not obtained. The tumor was diagnosed as a non-small-cell lung cancer with direct invasion to the adjacent rib. Although chemotherapy and radiotherapy resulted in decrease in tumor size, the tumor subsequently increased in size and the patient died 14 months after the first admission. Autopsy revealed multiple emphysematous bullae, poorly differentiated adenosquamous cell carcinoma of the lung, gastrointestinal stromal tumor of the stomach, and duodenal carcinoid tumor. This case suggests the possibility that von Recklinghausen's disease associated with emphysematous bullae is a risk factor for lung cancer. It has also been suggested that the genetic abnormality responsible for von Recklinghausen's disease increases the risk for various types of malignancy. Although von Recklinghausen's disease is reportedly associated with various malignant tumors, it is quite rare for von Recklinghausen's disease to be associated with triple non-neurogenic tumors. Careful observation is mandatory for patients with von Recklinghausen's disease.

  7. L1 cell adhesion molecule as a predictor for recurrence in pulmonary carcinoids and large-cell neuroendocrine tumors.

    PubMed

    Kim, Hyo Song; Yi, Seong Yoon; Jun, Hyun Jung; Ahn, Jin Seok; Ahn, Myung-Ju; Lee, Jeeyun; Kim, Youngwook; Cui, Zheng Yun; Hong, Hyo Jeong; Kim, Jin-Man; Li, Shengjin; Hwang, In Gyu; Park, Keunchil

    2009-02-01

    Pulmonary neuroendocrine tumors are a distinct subset of neoplasms with indolent to aggressive behavior. This study was conducted to evaluate the prognostic role of L1 cell adhesion molecule (L1CAM) in pulmonary neuroendocrine tumors. We retrospectively analyzed L1 expression in 55 cases of completely resected carcinoids and large-cell neuroendocrine carcinomas, by the immunohistochemistry with monoclonal antibody A10-A3 against human L1. L1 immunoreactivity was detected in 34 (61.8%) of 55 specimens. There was a significant correlation between L1 expression and the World Health Organization classification of this tumor (Spearman rank correlation, rho=0.60, p<0.001). With median follow-up of 52.0 months, the 5-year survival rate for patients with low expression of L1 (<20% of tumor cells stained) was significantly better compared with those with high expression of L1 (82.6% vs. 43.7%, p=0.005). L1 was also a significant independent predictor of disease-free survival, and patients with high L1 expression have a higher risk for recurrence compared with those with low L1 expression (hazard ratio, 3.0; 95% confidence interval, 1.2-8.3; p=0.034). L1 expression is significantly associated with aggressiveness and further studies with larger samples are needed to validate potential prognostic value for pulmonary neuroendocrine tumors.

  8. Cytopathologic characteristics of the primary strumal carcinoid tumor of the ovary: a case report with emphasis on differential diagnostic considerations.

    PubMed

    Hayashi, Toshitetsu; Haba, Reiji; Kushida, Yoshio; Kadota, Kyuichi; Katsuki, Naomi; Miyai, Yumi; Shibuya, Shinsuke; Sasaki, Makiko; Bando, Kenji; Matsunaga, Toru; Hata, Toshiyuki

    2013-09-01

    Primary strumal carcinoid tumor of the ovary (SCTO) is an extremely rare entity, though the survival rate is excellent if the disease is confined to one ovary. A case is presented here in which intraoperative squash smears in a 45-year-old woman with a left adnexal mass revealed dispersed or small clusters of neoplastic cells forming loosely cohesive gland-like structures with abundant cytoplasm. The nuclear chromatin was finely granular with a "salt and pepper" appearance and occasional tiny nucleoli. The position of the nucleus presented a vaguely plasmacytoid appearance. Small fragments of thyroidal colloid-like structures were also identified. A cytopathologic diagnosis of a SCTO was suggested. Further evaluation and immunohistochemical studies were conducted on formalin-fixed, paraffin-embedded material. Cords or nests of uniform cells with abundant cytoplasm, and eccentric nuclei with coarse chromatin and occasional colloidal tissue were identified on H&E sections. The tumor cells showed diffuse and strong cytoplasmic staining for chromogranin A, synaptophysin, CD56, and vimentin but were negative for calretinin, α-inhibin or CDX2. The proliferative index with MIB-1 was around 3%. Thyroidal colloid-like structures were immunoreactive for thyroglobulin and TTF-1 stains. The diagnosis of primary SCTO was confirmed based on cytopathologic, histopathological, and immunohistochemical results, and the location of the tumor. Awareness of the cytopathological findings of SCTO can assist in diagnosing this rare entity correctly.

  9. A Case of Primary Combined Squamous Cell Carcinoma with Neuroendocrine (Atypical Carcinoid) Tumor in the Floor of the Mouth

    PubMed Central

    Terada, Kazuhiro; Uchida, Fumihiko; Kanno, Naomi; Yanagawa, Toru; Bukawa, Hiroki

    2016-01-01

    The combined squamous cell carcinoma (SCC) with neuroendocrine (atypical carcinoid (AC)) tumor is extremely rare in the head and neck. We present here the first case of SCC with AC arising in the floor of the mouth of 65-year-old man. The tumor is comprised of two components of SCC and AC in the biopsy specimen. Neuroendocrine tumor component was classified as AC from the punctate necrosis and 2–10>/10 HPF. Immunohistochemical staining was HMW-CK/34B (+) and P63 (+) in SCC and synaptophysin (+) and CD56 (+) in AC. The pathological diagnosis of SCC with AC was made from both the morphological and immunological exam. Concurrent chemoradiotherapy was performed with radiotherapy 70.2 Gy and chemotherapy of CDDP and VP-16. Although the treatment effect was complete response both of primary tumor and of neck metastases, the recurrence of the primary tumor was after 6 months. Bilateral modified radical neck dissection and tumor resection of the floor of the mouth with reconstructive surgery of anterior lateral thigh free flap were performed. Although the primary and neck tumor did not recur, the multiple lung metastases and mediastinum lymph node metastases occurred at 6 months after surgery. PMID:28116178

  10. Endoscopic ultrasound-guided ethanol ablation of a large metastatic carcinoid tumor: success with a note of caution

    PubMed Central

    Mathers, Bradley W.; Harvey, Harold A.; Dye, Charles E.; Dougherty-Hamod, Brandy; Moyer, Matthew T.

    2014-01-01

    Endoscopic ultrasonography with fine needle infusion (EUS-FNI) of alcohol is the most reported method for EUS-guided tumor ablation. Several studies have reported successful EUS-guided ablation of pancreatic neuroendocrine tumors. However, these tumors have been relatively small (< 3 cm). In this report, a 50-year-old man with a metastatic carcinoid tumor with a large porta hepatis mass was referred to our clinic for EUS-guided ethanol ablation. After two separate EUS-FNI ablations, there was a 36 % reduction in tumor size (9.0 × 11.4 cm to 6.7 × 9.8 cm) with associated tumor lysis syndrome. Chromogranin A levels decreased from 460 to 132 ng/mL. The patient reported complete resolution of abdominal pain within 2 weeks, but only mild improvement in flushing and diarrhea. In conclusion, large metastatic neuroendocrine tumors can be successfully treated with EUS-guided ethanol ablation. Evidence-based guidelines are needed with regard to the appropriate volume of ethanol injected in EUS-guided ablation to promote the efficacy and safety of this emerging procedure. PMID:26135103

  11. Virilization due to ovarian androgen hypersecretion in a patient with ectopic adrenocorticotrophic hormone secretion caused by a carcinoid tumour: case report.

    PubMed

    Netea-Maier, R T; Nieuwlaat, W-A; Sweep, C G J; Wesseling, P; Massuger, L; Hermus, A R M M

    2006-10-01

    A 52-year-old woman presented with symptoms of virilization, which had been ongoing for 5 months. At the age of 34 years, she had a large abdominal carcinoid tumour removed. Twelve years later, she presented with Cushing's syndrome due to ectopic adrenocorticotrophic hormone (ACTH) production by carcinoid metastases localized in the right parametrium, fornix posterior and right diaphragm. Debulking laparotomy was performed followed by remission of hypercortisolism. Relapse of hypercortisolism followed 3 years later, and a second debulking laparotomy was performed including resection of the right ovary. In the following year, relapses of hypercortisolism were observed until bilateral adrenalectomy was performed. Laboratory evaluation revealed elevated serum levels of testosterone (23.0 nmol/l), androstenedione and 17-hydroxyprogesterone, and a serum estradiol (E2) level in the premenopausal range. The computerized tomography (CT) of the abdomen showed a large pelvic mass on the left side of the uterus without a recognizable left ovary. Treatment with a GnRH agonist (goserelin, 3.6 mg s.c., monthly) was initiated, resulting in normalization of the androgen levels. One year later, obstruction of the right ureter occurred due to progression of the pelvic metastases, thus a third debulking laparotomy with resection of the pelvic metastases including the left ovary was performed. The microscopic examination of the removed pelvic mass showed malignant carcinoid tissue with focal remnants of atrophic ovarian tissue. Two years after surgery, serum androgen levels are undetectable. We hypothesize that the high levels of ACTH at the site of the left ovary have induced androgen hypersecretion by steroid-producing cells in the ovary of our patient.

  12. Squamous cell carcinoma of the cecum developing in a patient with long-standing ulcerative colitis and a coexistent carcinoid tumor in the appendix: A case report.

    PubMed

    Ahmad, Saqib; Watson, Nicholas; Shamim, Kiran; Menon, Achyuth; Abdullah, Khorrum

    2015-09-01

    Squamous cell carcinoma (SCC) of the colon is a rare tumor that accounts for 0.1%-0.2% of colonic malignancies. However, pure SCC of the cecum is extremely rare. Although SCC of the colon is a rare complication of idiopathic inflammatory bowel disease (IBD), SCC of the cecum in the context of IBD has not been previously reported in the English literature. We report a case of the coexistence of SCC of the cecum and a carcinoid tumor in a 46-year-old female with long-standing pan-ulcerative colitis.

  13. The experience of a referral centre and literature overview of GIST and carcinoid tumours in inflammatory bowel diseases.

    PubMed

    Pellino, Gianluca; Marcellinaro, Rosa; Candilio, Giuseppe; De Fatico, G Serena; Guadagno, Elia; Campione, Severo; Santangelo, Giuseppe; Reginelli, Alfonso; Sciaudone, Guido; Riegler, Gabriele; Canonico, Silvestro; Selvaggi, Francesco

    2016-04-01

    Patients suffering from Inflammatory Bowel Diseases (IBD) are at increased risk of developing cancers of the gastrointestinal tract (GI). Adenocarcinomas are the most commonly observed GI tumours in IBD, and occur through an in inflammation-driven pathway. A trend toward reduced risk of bowel cancers has been observed in IBD in recent years, presumably related to improved medical treatments. However, some cancers may be independent from active inflammation, probably originating from altered interactions between the extremely active immune system of IBD patients and environmental factors. Data concerning gastrointestinal stromal tumours (GIST) and carcinoids tumours (CaT) of the GI in IBD patients are scanty. We report our experience with these rare cancers, and provide the readers with an overview on the topic, focussing on distinguishing and peculiar features of GIST and CaT of the GI in IBD compared with other cancer types and with general population, and address the treatment of such challenging conditions. Available data do not support an increased risk of GIST in IBD patients, but GI CaT may be more commonly observed in Crohn's disease. However, the presentation of GIST and GI CaT is protean and does not seem to be associated with disease activity in the involved GI segment in IBD. Conversely, some evidences suggest a potential role of inflammation in sustaining GI CaT in IBD. Increased awareness, longer duration of disease, and improved diagnostic modalities should also be considered when evaluating the increasing trend of CaT in CD patients. Treatment of GIST and CaT is not dissimilar from that of non-IBD patients, but prompt suspicion and diagnosis are crucial to achieve optimal outcomes.

  14. (99m)Tc-EDDA/HYNIC-octreotate - a new radiotracer for detection and staging of NET: a case of metastatic duodenal carcinoid.

    PubMed

    Hubalewska-Dydejczyk, Alicja; Szybiński, Piotr; Fröss-Baron, Katarzyna; Mikolajczak, Renata; Huszno, Bohdan; Sowa-Staszczak, Anna

    2005-01-01

    Somatostatin receptor scintigraphy (SRS) has become a routine imaging method for the diagnostics of neuroendocrine tumours (NET). (99m)Tc-EDDA/HYNIC-octreotate (Polatom, Poland) is a new radiotracer with high affinity for SSTR2 and similar physiological biodistribution to (111)In-Octreoscan. We present a case of a 47-year-old man with disseminated duodenal carcinoid. The patient had been operated due to the tumour mass detected in pancreatic head area. Histopathology revealed carcinoid of the duodenal wall with local lymph node and liver metastases. The patient was qualified for chemotherapy stopped due to severe leucopenia. (99m)Tc EDDA/HYNIC-octreotate scintigraphy was performed for staging and to determine SSTR status of the tumour before planned 90Y-DOTATATE therapy. The multiple metastatic lesions were detected all over the body. The high quality images with high target/non target ratio were obtained. (99m)Tc-MDP scintigraphy confirmed multiple bone metastases. On the basis of SRS result the patient was qualified for 90Y-DOTA-TATE therapy. In conclusion, (99m)Tc EDDA/HYNIC-octreotate can be regarded as a promising tracer for staging and to determine SSTR status of NET.

  15. The use of immunohistochemistry in the diagnosis of composite and collision tumors: exemplified by pleural mesothelioma and carcinoid tumor of the lung.

    PubMed

    Ordóñez, Nelson G

    2012-07-01

    A case of a collision lymph node metastasis of a mesothelioma and a carcinoid tumor in a 73-year-old man with a history of asbestos exposure is reported. An interesting finding in this case was that both the mesothelioma and its lymph node metastases exhibited a wide variety of histologic patterns, including one characterized by a solid growth of large cells with abundant, clear, and foamy cytoplasm and another exhibiting deciduoid features. Pathologists should be aware that mesotheliomas can present very unusual morphologic features, such as those seen in the present case, and therefore, should be included in the differential diagnosis of those tumors that can display similar morphology and can metastasize to the serosal membranes. Reexamination of the pneumonectomy specimen in the current case identified a primary peripheral carcinoid tumor. The recognition of a nonasbestos-related tumor in a patient with mesothelioma is important since its presence may have an impact on the patient's life expectancy and, therefore, may affect any compensation settlement.

  16. ECL-cell carcinoids and carcinoma in patients homozygous for an inactivating mutation in the gastric H(+) K(+) ATPase alpha subunit.

    PubMed

    Fossmark, Reidar; Calvete, Oriol; Mjønes, Patricia; Benitez, Javier; Waldum, Helge L

    2016-07-01

    A family with a missense variant of the ATP4A gene encoding the alpha subunit of the gastric proton pump (H(+) K(+) ATPase) has recently been described. Homozygous siblings were hypergastrinemic (median gastrin 486 pM) and had gastric tumours diagnosed at a median age of 33 years. In the current histopathological study, we further characterized the tumours found in the gastric corpus. The tumours had the histological appearance of carcinoids (NET G1 or G2) and were immunoreactive for the general neuroendocrine markers chromogranin A (CgA) and synaptophysin as well as the ECL-cell markers vesicular monoamine transporter 2 (VMAT2) and histidine decarbozylase (HDC). One of the tumours consisted of a NET G2 component, but also had a component with glandular growth, which morphologically was classified as an intestinal type adenocarcinoma. Many glands of the adenocarcinoma contained a large proportion of cells positive for neuroendocrine markers, especially the small vesicle marker synaptophysin and the cytoplasmic enzyme HDC. In conclusion, patients homozygous for an inactivating ATP4A mutation develop gastric ECL-cell carcinoids in their 3rd or 4th decade. The adenocarcinoma may be classified as neuroendocrine with ECL-cell differentiation.

  17. A PCR blood test outperforms chromogranin A in carcinoid detection and is unaffected by proton pump inhibitors.

    PubMed

    Modlin, Irvin M; Aslanian, Harry; Bodei, Lisa; Drozdov, Ignat; Kidd, Mark

    2014-12-01

    A critical requirement in neuroendocrine tumor (NET) management is a blood biomarker test that is sensitive, specific and reproducible. We evaluated a PCR-based 51-transcript signature to detect tumors, compared it with chromogranin A (CgA) and examined the confounding effect of proton pump inhibitors (PPIs), which cause falsely elevated CgA levels. The multigene signature was evaluated in two groups. Group 1: 125 prospectively collected NETs: gastroenteropancreatic NETs (n=91, including 42 pancreatic and 40 small intestinal), carcinoids of unknown primary (n=18) and other sites (n=16). Group 2: prospectively collected non-NET patients receiving PPIs (>1 month; dyspepsia, n=19; GERD, n=6; and pancreatitis, n=4) and 50 controls. All samples were analyzed by PCR (marker genes) and ELISA (DAKO-CgA). Sensitivity comparisons included χ(2), non-parametric measurements, and receiver operating characteristic (ROC) curves. Group 1: 123 NETs were PCR-positive (98.4%) compared with 50 (40%) CgA-positive (χ(2)=97.3, P<10(-26)). Significant differences (P<0.001) were noted between pancreas: PCR 95% vs CgA 29.2% (P<10(-9)) and small intestine: 100 vs 58% (P<10(-4)). The multigene test was elevated in all grades (G1-G3), in both local and disseminated disease, and was not normalized by somatostatin analog therapy. It was also elevated in 97% of CgA normal NETs. Group 2: PPI administration increased CgA in 83% and CgA was elevated in 26% of controls. PCR values were not elevated in either group. PCR performance metrics were as follows: sensitivity 98.4%, specificity 100%, positive predictive value 100%, negative predictive value 97.8%, and the ROC-derived area under the curve (AUC) was 0.997. These were significantly better than CgA (all metrics <60%; AUC, 0.54; Z-statistic, 10.44, P<0.0001). A 51-panel multigene blood transcript analysis is significantly more sensitive than plasma CgA for NET detection and is unaffected by acid suppression therapy.

  18. Diagnostic challenges and management of a patient with acromegaly due to ectopic growth hormone-releasing hormone secretion from a bronchial carcinoid tumour

    PubMed Central

    Kyriakakis, Nikolaos; Trouillas, Jacqueline; Dang, Mary N; Lynch, Julie; Belchetz, Paul; Korbonits, Márta

    2017-01-01

    Summary A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically. A lung mass was discovered incidentally on a chest X-ray performed as part of a routine pre-assessment for spinal surgery 5 years following the initial presentation. This was confirmed to be a bronchial carcinoid tumour, which was strongly positive for growth hormone-releasing hormone (GHRH) and somatostatin receptor type 2 by immunohistochemistry. The re-examination of the pituitary specimens asserted the diagnosis of pituitary GH hyperplasia. Complete resolution of the patient’s acromegaly was achieved following right lower and middle lobectomy. Seventeen years following the successful resection of the bronchial carcinoid tumour the patient remains under annual endocrine follow-up for monitoring of the hypopituitarism he developed after the original interventions to his pituitary gland, while there has been no evidence of active acromegaly or recurrence of the carcinoid tumour. Ectopic acromegaly is extremely rare, accounting for <1% of all cases of acromegaly. Our case highlights the diagnostic challenges differentiating between ectopic acromegaly and acromegaly of pituitary origin and emphasises the importance of avoiding unnecessary pituitary surgery and radiotherapy. The role of laboratory investigations, imaging and histology as diagnostic tools is discussed. Learning points: Ectopic acromegaly is rare, accounting for less than 1% of all cases of acromegaly. Ectopic acromegaly is almost always due to extra-pituitary GHRH secretion

  19. EVALUATION OF LANREOTIDE DEPOT/AUTOGEL EFFICACY AND SAFETY AS A CARCINOID SYNDROME TREATMENT (ELECT): A RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL.

    PubMed

    Vinik, Aaron I; Wolin, Edward M; Liyanage, Nilani; Gomez-Panzani, Edda; Fisher, George A

    2016-09-01

    To evaluate the efficacy and safety of lanreotide depot/autogel 120 mg for the control of carcinoid syndrome (CS) symptoms in patients with neuroendocrine tumors (NETs). This was a 16-week, randomized, double-blind, phase 3 trial (Clinicaltrials.gov: NCT00774930). Patients with/without prior somatostatin analog (SSA) use were randomized to lanreotide depot/autogel 120 mg or placebo every 4 weeks, with access to short-acting octreotide as rescue medication. The primary endpoint was the percentage of days in which short-acting octreotide was used, which was assessed from daily diaries using an analysis of covariance including the stratification variables baseline short-acting octreotide use and frequency of diarrhea/flushing. The proportions of patients experiencing treatment success was a supportive analysis. Adverse events were recorded at all visits. A total of 115 patients were enrolled (lanreotide, n = 59; placebo, n = 56). The adjusted mean (95% confidence interval [CI]) percentage of days with rescue octreotide use (primary endpoint) was significantly lower in the lanreotide (33.7%; 95% CI, 25.0%-42.4%) versus the placebo group (48.5%; 95% CI, 39.6%-57.4%), representing an absolute difference of -14.8% (95% CI, -26.8% to -2.8%; P = .017). The odds ratio of full/partial treatment success (≤3 days short-acting octreotide use weeks 12 to 15) was significantly greater with lanreotide than placebo (2.4; 95% CI, 1.1-5.3; P = .036). No new safety concerns were identified, and lanreotide was well tolerated. Lanreotide depot/autogel is effective for the control of CS symptoms in patients (SSA-naïve or experienced) with NETs. AE = adverse event BMI = body mass index CS = carcinoid syndrome ELECT = Evaluating Lanreotide Efficacy and safety as a Carcinoid-syndrome Treatment HRQoL = health-related quality of life LTOLE = long-term open-label extension NET = neuroendocrine tumor OL = open label SSA = somatostatin analog.

  20. What is the role of lymph nodal metastases and lymphadenectomy in the surgical treatment and prognosis of thymic carcinomas and carcinoids?

    PubMed

    Viti, Andrea; Bertolaccini, Luca; Terzi, Alberto

    2014-12-01

    A best evidence topic in thoracic surgery was written according to a structured protocol. We looked at the clinical relevance of lymph node involvement and nodal (N) stage, in thymomas, thymic carcinomas and carcinoids. The possible role of lymphadenectomy in addition to thymectomy was also evaluated. A total of 605 papers were found, of which nine represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers were tabulated. In the Yamakawa-Masaoka classification, based on 226 patients, lymph nodes were classified as anterior mediastinal (N1), defined as nodes surrounding the thymus gland; intrathoracic (N2), all nodes within the thorax excluding N1; and extrathoracic nodes (N3). Kondo validated the Yamakawa-Masaoka classification in a multicentric cohort of 1320 patients. Thymomas presented nodal involvement in 1.8% of cases, carcinomas in 27% of cases, and carcinoids in 28% of cases. The role of nodal status in defining the stage was even more emphasized in the staging system developed by Tsuchiya for thymic carcinomas. In the Istituto Nazionale Tumori classification, thymomas with N1 or N2 were considered as locally advanced disease with a 95-month disease-free survival rate for locally advanced disease of 46.9 vs 98.6% for locally restricted disease (absence of nodal involvement). Weissferdt and Moran, on a series of 65 thymic carcinomas, underlined the clinical relevance of nodal involvement. Positive lymph nodes were associated with significantly worse survival (P = 0.01070). Okuma, in a series of 68 advanced stage thymic carcinomas, showed that curative-intent surgical treatment was related to prolonged survival (P = 0.03). In particular, IVb tumours due to node-only involvement had better survival when radical resection was achieved when compared with IVb due to distant metastases (P = 0.03). Sung et al. showed the

  1. Phase III study of pasireotide long-acting release in patients with metastatic neuroendocrine tumors and carcinoid symptoms refractory to available somatostatin analogues

    PubMed Central

    Wolin, Edward M; Jarzab, Barbara; Eriksson, Barbro; Walter, Thomas; Toumpanakis, Christos; Morse, Michael A; Tomassetti, Paola; Weber, Matthias M; Fogelman, David R; Ramage, John; Poon, Donald; Gadbaw, Brian; Li, Jiang; Pasieka, Janice L; Mahamat, Abakar; Swahn, Fredrik; Newell-Price, John; Mansoor, Wasat; Öberg, Kjell

    2015-01-01

    In a randomized, double-blind, Phase III study, we compared pasireotide long-acting release (pasireotide LAR) with octreotide long-acting repeatable (octreotide LAR) in managing carcinoid symptoms refractory to first-generation somatostatin analogues. Adults with carcinoid tumors of the digestive tract were randomly assigned (1:1) to receive pasireotide LAR (60 mg) or octreotide LAR (40 mg) every 28 days. Primary outcome was symptom control based on frequency of bowel movements and flushing episodes. Objective tumor response was a secondary outcome. Progression-free survival (PFS) was calculated in a post hoc analysis. Adverse events were recorded. At the time of a planned interim analysis, the data monitoring committee recommended halting the study because of a low predictive probability of showing superiority of pasireotide over octreotide for symptom control (n=43 pasireotide LAR, 20.9%; n=45 octreotide LAR, 26.7%; odds ratio, 0.73; 95% confidence interval [CI], 0.27–1.97; P=0.53). Tumor control rate at month 6 was 62.7% with pasireotide and 46.2% with octreotide (odds ratio, 1.96; 95% CI, 0.89–4.32; P=0.09). Median (95% CI) PFS was 11.8 months (11.0 – not reached) with pasireotide versus 6.8 months (5.6 – not reached) with octreotide (hazard ratio, 0.46; 95% CI, 0.20–0.98; P=0.045). The most frequent drug-related adverse events (pasireotide vs octreotide) included hyperglycemia (28.3% vs 5.3%), fatigue (11.3% vs 3.5%), and nausea (9.4% vs 0%). We conclude that, among patients with carcinoid symptoms refractory to available somatostatin analogues, similar proportions of patients receiving pasireotide LAR or octreotide LAR achieved symptom control at month 6. Pasireotide LAR showed a trend toward higher tumor control rate at month 6, although it was statistically not significant, and was associated with a longer PFS than octreotide LAR. PMID:26366058

  2. Diagnostic challenges and management of a patient with acromegaly due to ectopic growth hormone-releasing hormone secretion from a bronchial carcinoid tumour.

    PubMed

    Kyriakakis, Nikolaos; Trouillas, Jacqueline; Dang, Mary N; Lynch, Julie; Belchetz, Paul; Korbonits, Márta; Murray, Robert D

    2017-01-01

    A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically. A lung mass was discovered incidentally on a chest X-ray performed as part of a routine pre-assessment for spinal surgery 5 years following the initial presentation. This was confirmed to be a bronchial carcinoid tumour, which was strongly positive for growth hormone-releasing hormone (GHRH) and somatostatin receptor type 2 by immunohistochemistry. The re-examination of the pituitary specimens asserted the diagnosis of pituitary GH hyperplasia. Complete resolution of the patient's acromegaly was achieved following right lower and middle lobectomy. Seventeen years following the successful resection of the bronchial carcinoid tumour the patient remains under annual endocrine follow-up for monitoring of the hypopituitarism he developed after the original interventions to his pituitary gland, while there has been no evidence of active acromegaly or recurrence of the carcinoid tumour. Ectopic acromegaly is extremely rare, accounting for <1% of all cases of acromegaly. Our case highlights the diagnostic challenges differentiating between ectopic acromegaly and acromegaly of pituitary origin and emphasises the importance of avoiding unnecessary pituitary surgery and radiotherapy. The role of laboratory investigations, imaging and histology as diagnostic tools is discussed. Ectopic acromegaly is rare, accounting for less than 1% of all cases of acromegaly.Ectopic acromegaly is almost always due to extra-pituitary GHRH secretion, mainly from neuroendocrine

  3. Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases.

    PubMed

    Reid, Michelle D; Basturk, Olca; Shaib, Walid L; Xue, Yue; Balci, Serdar; Choi, Hye-Jeong; Akkas, Gizem; Memis, Bahar; Robinson, Brian S; El-Rayes, Bassel F; Staley, Charles A; Staley, Christopher A; Winer, Joshua H; Russell, Maria C; Knight, Jessica H; Goodman, Michael; Krasinskas, Alyssa M; Adsay, Volkan

    2016-10-01

    High-grade versions of appendiceal goblet cell carcinoids ('adenocarcinoma ex-goblet cell carcinoids') are poorly characterized. We herein document 77 examples. Tumors occurred predominantly in females (74%), mean age 55 years (29-84), most with disseminated abdominal (77% peritoneal, 58% gynecologic tract involvement) and stage IV (65%) disease. Many presented to gynecologic oncologists, and nine had a working diagnosis of ovarian carcinoma. Metastases to liver (n=3) and lung (n=1) were uncommon and none arose in adenomatous lesions. Tumors had various histologic patterns, in variable combinations, most of which were fairly specific, making them recognizable as appendiceal in origin, even at metastatic sites: I: Ordinary goblet cell carcinoid/crypt pattern (rounded, non-luminal acini with well-oriented goblet cells), in variable amounts in all cases. II: Poorly cohesive goblet cell pattern (diffusely infiltrative cords/single files of signet ring-like/goblet cells). III: Poorly cohesive non-mucinous cell (diffuse-infiltrative growth of non-mucinous cells). IV: Microglandular (rosette-like glandular) pattern without goblet cells. V: Mixed 'other' carcinoma foci (including ordinary intestinal/mucinous). VI: goblet cell carcinoid pattern with high-grade morphology (marked nuclear atypia). VII: Solid sheet-like pattern punctuated by goblet cells/microglandular units. Ordinary nested/trabecular ('carcinoid pattern') was very uncommon. In total, 33(52%) died of disease, with median overall survival 38 months and 5-year survival 32%. On multivariate analysis perineural invasion and younger age (<55) were independently associated with worse outcome while lymph-vascular invasion, stage, and nodal status trended toward, but failed to reach, statistical significance. Worse behavior in younger patients combined with female predilection and ovarian-affinity raise the possibility of hormone-assisted tumor progression. In conclusion, 'adenocarcinoma ex-goblet cell carcinoid' is

  4. Ewing sarcoma mimicking atypical carcinoid tumor: detection of unexpected genomic alterations demonstrates the use of next generation sequencing as a diagnostic tool.

    PubMed

    Doyle, Leona A; Wong, Kwok-Kin; Bueno, Raphael; Dal Cin, Paola; Fletcher, Jonathan A; Sholl, Lynette M; Kuo, Frank

    2014-01-01

    Increasingly, tumors are being analyzed for a variety of mutations and other genomic changes, with the goals of guiding personalized therapy and directing patients to appropriate clinical trials based on genotype, as well as identifying previously unknown genomic changes in different tumor types and thereby providing new insights into the pathogenesis of human cancers. Next generation sequencing is a powerful research tool now gaining traction in the clinic. In this report, we demonstrate the utility of next generation sequencing assays in providing diagnostic information when evaluating tumor specimens. This is illustrated by a case previously thought to represent an atypical carcinoid tumor, in which an EWSR1-ERG translocation was detected during next generation sequencing using a hybrid capture approach, leading to a revised diagnosis of Ewing sarcoma. The role of translocation detection in these assays is also discussed.

  5. Recurrent pneumonia due to bronchial carcinoid tumour in a young patient: the role of (111)in-pentetreotide in imaging studies.

    PubMed

    García-Talavera, Paloma; López-Pedreira, María Rosa; Udaondo, Marian; Matilla, José María; Villanueva, Juan Gabriel; Borrego, Henar

    2015-03-01

    Bronchial carcinoid tumours are an uncommon cause of recurrent pneumonia in young patients. Diagnosis is determined from imaging studies, bronchoscopy, and histological confirmation, and treatment is generally surgical. Two cases are reviewed in order to examine the value of (111)In-DTPA-Phe-octreotide ((111)In-pentetreotide) scintigraphy in the pre-surgical evaluation of these patients. After a suspicious area was observed in other tests (standard X-ray, CT), a neuroendocrine tumour was diagnosed using this technique and the presence of regional or distant disease was ruled out. Comparison with the less valuable (18)F-FDG PET (carried out in one of the cases) highlights the usefulness of SPECT-CT, which performs notably better in terms of the localization and characterisation of findings.

  6. Subcutaneous octreotide treatment of a growth hormone-releasing hormone-secreting bronchial carcinoid: superiority of continuous versus intermittent administration to control hormonal secretion.

    PubMed

    Lefebvre, S; De Paepe, L; Abs, R; Rahier, J; Selvais, P; Maiter, D

    1995-09-01

    Diagnosis of ectopic acromegaly was made in a 21-year-old female patient who 3 years before had undergone a right pneumectomy for a disseminated bronchial carcinoid. Plasma growth hormone-releasing hormone (GHRH) concentrations were markedly elevated (6440 ng/l; normal value < 100 ng/l), as were serum GH (187 micrograms/l; normal < 5 micrograms/l) and plasma insulin-like growth factor I (IGF-I) levels (6.7 U/ml; normal < 2 U/ml). Retrospective immunohistochemical examination of the carcinoid tumor was positive for GHRH and the tumoral content of GHRH was 2130 ng/g wet weight. Subcutaneous treatment with octreotide was begun and first resulted in a profound inhibition of GH hypersecretion, normalization of plasma IGF-I and only partial reduction of GHRH concentrations. However, the initial dose of 3 x 100 micrograms had to be increased gradually to 4 x 750 micrograms because of a progressive deterioration of the hormonal control. After 15 months of intermittent therapy, octreotide was administered by continuous sc infusion. This treatment improved compliance, allowed the daily dose of octreotide to be reduced to 1500 micrograms and normalized serum GH levels. A near-normalization of the plasma IGF-I concentrations was also obtained, whereas the suppression of plasma GHRH concentrations remained incomplete. Despite favorable evolution of the endocrine parameters, intramedullar metastases were diagnosed and required radiation therapy. This observation emphasizes the superiority of continuous over intermittent administration of octreotide in the treatment of ectopic acromegaly. It also shows that the somatostatin analog acts more at the pituitary level to inhibit GH secretion than at the site of the neuroendocrine tumor.

  7. Malignant ileocaecal serotonin-producing carcinoid tumours: the presence of a solid growth pattern and/or Ki67 index above 1% identifies patients with a poorer prognosis.

    PubMed

    Cunningham, Janet L; Grimelius, Lars; Sundin, Anders; Agarwal, Smriti; Janson, Eva T

    2007-01-01

    Patients with malignant serotonin-producing carcinoid tumours in the jejunum, ileum and caecum generally have long survival expectancy. In some patients, however, tumour progression is more rapid and there is a need to identify them at an early stage. The purpose of this study was to determine if histopathological characteristics and/or Ki67 and apoptotic indices are of prognostic value in cases of metastatic disease. Eighty-one patients with this tumour were included in the study; all had metastases and their survival range was 1-223 months. Five growth patterns were identified and described. For 57 patients whose tumour material was available, the Ki67 and apoptotic indices were calculated for ten randomly selected tumour areas and 'hot spots'. A Cox regression analysis was used to test if histopathology and/or Ki67 index >/=1% could identify patients whose survival might be shorter than anticipated. One of the histopathological growth patterns-the solid (non-organoid) cell pattern-was correlated to shorter survival in both primary tumours and metastases, when compared with the organoid growth patterns (hazard ratio 2.9 and 2.3, p/=1%, in both primary tumour and metastases, identified patients at increased risk of shorter survival (hazard ratio 5.4 and 2.5, pcarcinoids, two independent criteria, a solid growth pattern and Ki67 index >/=1%, can be used to identify patients with a poorer prognosis. This study also showed that Ki67 index <2% cannot, as previously suggested, be used to indicate a benign progression for this tumour category.

  8. The novel somatostatin receptor 2/dopamine type 2 receptor chimeric compound BIM-23A758 decreases the viability of human GOT1 midgut carcinoid cells.

    PubMed

    Zitzmann, Kathrin; Andersen, Sandra; Vlotides, George; Spöttl, Gerald; Zhang, Shengwen; Datta, Rakesh; Culler, Michael; Göke, Burkhard; Auernhammer, Christoph J

    2013-01-01

    The majority of neuroendocrine tumors (NETs) of the gastroenteropancreatic system coexpress somatostatin receptors (SSTRs) and dopamine type 2 receptors (D2R), thus providing a rationale for the use of novel SSTR2/D2R chimeric compounds in NET disease. Here we investigate the antitumor potential of the SSTR2/D2R chimeric compounds BIM-23A760 and BIM-23A758 in comparison to the selective SSTR2 agonist BIM-23023 and the selective D2R agonist BIM-53097 on human NET cell lines of heterogeneous origin. While having only minor effects on human pancreatic and bronchus carcinoid cells (BON1 and NCI-H727), BIM-23A758 induced significant antitumor effects in human midgut carcinoid cells (GOT1). These effects involved apoptosis induction as well as inhibition of mitogen-activated protein kinase and Akt signaling. Consistent with their antitumor response to BIM-23A758, GOT1 cells showed relatively high expression levels of SSTR2 and D2R mRNA. In particular, GOT1 cells highly express the short transcript variant of D2R. In contrast to BIM-23A758, the SSTR2/D2R chimeric compound BIM-23A760 as well as the individual SSTR2 and D2R agonistic compounds BIM-23023 and BIM-53097 induced no or only minor antitumor responses in the examined NET cell lines. Taken together, our findings suggest that the novel SSTR2/D2R chimeric compound BIM-23A758 might be a promising substance for the treatment of NETs highly expressing SSTR2 and D2R. In particular, a sufficient expression of the short transcript variant of DR2 might play a pivotal role for effective treatment.

  9. Lung Carcinoid Tumor: Surgery

    MedlinePlus

    ... the sleeve above and below the stain and sewing the cuff back onto the shortened sleeve. A ... because it requires a great deal of technical skill. Possible risks and side effects of lung surgery ...

  10. Carcinoid Cancer Foundation

    MedlinePlus

    ... a larger size). The Latest News Gallium 68 PET/CT Scanning for Neuroendocrine Tumors — Information and Locations The ... June 1, 2016 approval of the Gallium-68 PET/CT DOTATATE scan using NETSPOT ® heralded a new age ...

  11. Acromegaly and Cushing's syndrome due to ectopic production of GHRH and ACTH by a thymic carcinoid tumour: in vitro responses to GHRH and GHRP-6.

    PubMed

    Jansson, J O; Svensson, J; Bengtsson, B A; Frohman, L A; Ahlman, H; Wängberg, B; Nilsson, O; Nilsson, M

    1998-02-01

    A 50-year-old male presented with diabetes mellitus and Cushing's syndrome associated with a large mediastinal mass. The levels of serum cortisol were high (1500-1800 nmol/l) without diurnal variation. Plasma ACTH levels (200-250 ng/l) and urinary excretion of cortisol were also increased. The levels of these hormones did not change in response to stimulation with corticotrophin releasing hormone (CRH) or suppression with high doses of dexamethasone. The patient had an elevated baseline GH level (7.3 mU/l), and the levels of immunoreactive GH-releasing hormone (GHRH) in eight plasma samples were markedly increased (600-1500 ng/l). Circulating levels of IGF-1, chromogranin A and neuropeptide Y (NPY) were also increased. Computer-assisted tomography and octreotide scintigraphy revealed a large mediastinal tumour and metastases in the left supraclavicular fossa. During treatment with octreotide, the baseline GH level was decreased (to 4.4 mU/l), while the GH pulse height was unchanged. Surgical removal of most of the tumour tissue resulted in a further decrease in the baseline serum GH level to a value (1.6 mU/l) about 20% of that before treatment, while the pulse height and mean GH were affected to a lesser extent. Postoperatively, circulating levels of cortisol and IGF-1 decreased, and the patient exhibited clinical improvement. Histological examination showed a neuroendocrine tumour with characteristics consistent with a foregut carcinoid of thymic origin. Immunoreactive GHRH, ACTH and NPY, but not immunoreactive GH, were detected in 80-90% of the tumour cells and the three peptides appeared to be co-localized. In primary culture, cells from this tumour displayed calcium influx in response to GHRH or GH releasing peptide-6 (GHRP-6), while there were not such responses by cells from another carcinoid not producing GHRH, ACTH or NPY. These results demonstrate a rare case of ectopic production of GHRH, ACTH and NPY, and indicate that the tumour cells were responsive

  12. Reduced uncertainty as a diagnostic benefit: an initial assessment of somatostatic receptor scintigraphy's value in detecting distant metastases of carcinoid liver tumours.

    PubMed

    Woodward, R S; Schnitzler, M A; Kvols, L K

    1998-03-01

    This paper employs classical concepts of diminishing marginal utility to demonstrate that risk-aversion can increase the perceived value of diagnostic procedures and thus raise optimum diagnostic expenditures. The theory is applied to a model in the spirit of Phelps and Mushlin's initial technology assessments. The specific evaluation is the cost-effectiveness of somatostatin receptor scintigraphy used to detect distant metastases of carcinoid liver tumours in a patient otherwise eligible for surgical resection of the liver. Data for the model are taken from published sources and financial databases, when available, and otherwise from a senior clinician's experience (LKK). The quantitative results indicate that receptor scintigraphy may have two beneficial impacts to risk-neutral individuals. First, it may reduce the combined costs of therapy and treatment because the diagnostic procedure costs less than the expected savings generated by avoiding inappropriate surgeries. Second, it may improve the patient's expected health-status-adjusted life years (HSALY) because the information allows physicians to better match treatment to the cancer's stage. Finally the paper demonstrates that risk aversion, as embodied in classical diminishing marginal utility applied to health status, can increase the value of the diagnostic tests and can lead the patient to choose a less beneficial treatment. An illustrative risk-averse utility function changed the optimum treatment from surgery to chemotherapy and increased scintigraphy's benefit by 500%.

  13. Rare triad of periampullary carcinoid, duodenal gastrointestinal stromal tumor and plexiform neurofibroma at hepatic hilum in neurofibromatosis type 1: a case report.

    PubMed

    Abdessayed, Nihed; Gupta, Rahul; Mestiri, Sarra; Bdioui, Ahlem; Trimech, Mounir; Mokni, Moncef

    2017-08-29

    Neurofibromatosis type 1 is a relatively common inherited disorder. Patients with neurofibromatosis type 1 are at high risk of developing neurogenic, neuroendocrine and mesenchymal intra-abdominal tumors. Although coexistence of multiple tumors of different types is frequent in neurofibromatosis type 1, simultaneous occurrence of abdominal tumors of three types in very rare. A 66-year-old lady with neurofibromatosis type 1 presented with painless progressive jaundice for six months. Laboratory investigations revealed iron deficiency anemia and conjugated hyperbilirubinemia. Tumor markers were normal. Abdominal computed tomography showed a 3 × 2 cm heterogenous mass in the periampullary region with mild dilation of the common bile duct and another 2 × 1.7 cm mass in the fourth portion of the duodenum. Endoscopic biopsy confirmed the diagnosis of periampullary carcinoid. At surgery, multiple small nodules were detected at the hepatic hilum. Frozen section suggested them to be neurofibromas. Patient underwent pancreatoduodenectomy and had uneventful recovery with no recurrence at two months. Microscopic examination of the resected specimen confirmed presence of three tumors: periampullary well differentiated neuroendocrine tumor, gastrointestinal stromal tumor of the fourth part of duodenum and plexiform neurofibroma at the hepatic hilum. Patients of neurofibromatosis type 1 with abdominal symptoms should be treated with high index of clinical suspicion and thoroughly evaluated to rule out multiple tumors.

  14. Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

    PubMed Central

    Reid, Michelle D; Basturk, Olca; Shaib, Walid L; Xue, Yue; Balci, Serdar; Choi, Hye-Jeong; Akkas, Gizem; Memis, Bahar; Robinson, Brian S; El-Rayes, Bassel F; Staley, Charles A; Staley, Christopher A; Winer, Joshua H; Russell, Maria C; Knight, Jessica H; Goodman, Michael; Krasinskas, Alyssa M; Adsay, Volkan

    2016-01-01

    High-grade versions of appendiceal goblet cell carcinoids (‘adenocarcinoma ex-goblet cell carcinoids’) are poorly characterized. We herein document 77 examples. Tumors occurred predominantly in females (74%), mean age 55 years (29–84), most with disseminated abdominal (77% peritoneal, 58% gynecologic tract involvement) and stage IV (65%) disease. Many presented to gynecologic oncologists, and nine had a working diagnosis of ovarian carcinoma. Metastases to liver (n =3) and lung (n =1) were uncommon and none arose in adenomatous lesions. Tumors had various histologic patterns, in variable combinations, most of which were fairly specific, making them recognizable as appendiceal in origin, even at metastatic sites: I: Ordinary goblet cell carcinoid/crypt pattern (rounded, non-luminal acini with well-oriented goblet cells), in variable amounts in all cases. II: Poorly cohesive goblet cell pattern (diffusely infiltrative cords/single files of signet ring-like/goblet cells). III: Poorly cohesive non-mucinous cell (diffuse-infiltrative growth of non-mucinous cells). IV: Microglandular (rosette-like glandular) pattern without goblet cells. V: Mixed ‘other’ carcinoma foci (including ordinary intestinal/mucinous). VI: goblet cell carcinoid pattern with high-grade morphology (marked nuclear atypia). VII: Solid sheet-like pattern punctuated by goblet cells/microglandular units. Ordinary nested/trabecular (‘carcinoid pattern’) was very uncommon. In total, 33(52%) died of disease, with median overall survival 38 months and 5-year survival 32%. On multivariate analysis perineural invasion and younger age (<55) were independently associated with worse outcome while lymph-vascular invasion, stage, and nodal status trended toward, but failed to reach, statistical significance. Worse behavior in younger patients combined with female predilection and ovarian-affinity raise the possibility of hormone-assisted tumor progression. In conclusion, ‘adenocarcinoma ex

  15. Activation of Type 1 CRH receptor isoforms induces serotonin release from human carcinoid BON-1N cells: an enterochromaffin cell model.

    PubMed

    Wu, S Vincent; Yuan, Pu-Qing; Lai, Jim; Wong, Kelvin; Chen, Monica C; Ohning, Gordon V; Taché, Yvette

    2011-01-01

    CRH and 5-hydroxytryptamine (5-HT) are expressed in human colonic enterochromaffin (EC) cells, but their interactions at the cellular level remain largely unknown. The mechanistic and functional relationship between CRH and 5-HT systems in EC cells was investigated in a human carcinoid cloned BON cell line (BON-1N), widely used as an in vitro model of EC cell function. First, we identified multiple CRH(1) splice variants, including CRH(1a), CRH(1c), CRH(1f), and a novel form lacking exon 4, designated here as CRH(1i), in the BON-1N cells. The expression of CRH(1i) was also confirmed in human brain cortex, pituitary gland, and ileum. Immunocytochemistry and immunoblot analysis confirmed that BON-1N cells were CRH(1) and 5-HT positive. CRH, urocortin (Ucn)-1, and cortagine, a selective CRH(1) agonist, all increased intracellular cAMP, and this concentration-dependent response was inhibited by CRH(1)-selective antagonist NBI-35965. CRH and Ucn-1, but not Ucn-2, stimulated significant ERK1/2 phosphorylation. In transfected human embryonic kidney-293 cells, CRH(1i) isoforms produced a significant increase in pERK1/2 in response to CRH(1) agonists that was sensitive to NBI-35965. CRH and Ucn-1 stimulated 5-HT release that reached a maximal increase of 3.3- and 4-fold at 10(-8) m over the basal level, respectively. In addition, exposure to CRH for 24-h up-regulated tryptophan hydroxylase-1 mRNA levels in the BON-1N cells. These findings define the expression of EC cell-specific CRH(1) isoforms and activation of CRH(1)-dependent pathways leading to 5-HT release and synthesis; thus, providing functional evidence of a link exists between CRH and 5-HT systems, which have implications in stress-induced CRH(1) and 5-HT-mediated stimulation of lower intestinal function.

  16. Synergistic effects of erlotinib and everolimus on bronchial carcinoids and large-cell neuroendocrine carcinomas with activated EGFR/AKT/mTOR pathway.

    PubMed

    Bago-Horvath, Zsuzsanna; Sieghart, Wolfgang; Grusch, Michael; Lackner, Andreas; Hayden, Hubert; Pirker, Christine; Komina, Oxana; Węsierska-Gądek, Józefa; Haitel, Andrea; Filipits, Martin; Berger, Walter; Schmid, Katharina

    2012-01-01

    Epidermal growth factor receptor (EGFR) and mammalian target of rapamycin (mTOR) are crucial targets in cancer therapy. Combined inhibition of both targets yielded synergistic effects in vitro and in vivo in several cancer entities. However, the impact of EGFR and mTOR expression and combined inhibition in neuroendocrine lung tumors other than small-cell lung cancer remains unclear. Expression and activation of EGFR/AKT/mTOR pathway constituents were investigated in typical and atypical bronchial carcinoid (AC) tumors and large-cell neuroendocrine lung carcinomas (LCNEC) by immunohistochemistry in 110 tumor samples, and correlated with clinicopathological parameters and patient survival. Cytotoxicity of mTOR inhibitor everolimus and EGFR inhibitor erlotinib alone and in combination was assessed using growth inhibition assay in NCI-H720 AC and SHP-77 LCNEC cells. Cell cycle phase distribution was determined by FACS. Apoptosis-associated activation of caspase-3/7 was measured by Caspase-Glo® assay. Activity status of EGFR and mTOR pathway components was analyzed by immunoblotting. Activation of the EGFR/AKT/mTOR axis could be demonstrated in all entities and was significantly increased in higher grade tumors. Neoadjuvant chemotherapy correlated significantly with p-AKT expression and p-ERK loss. Erlotinib combined with everolimus exerted synergistic combination effects in AC and LCNEC cells by induction of apoptosis, while cell cycle phase distribution remained unaffected. These effects could be explained by synergistic downregulation of phospho-mTOR, phospho-p70S6 kinase and phospho-AKT expression by everolimus and erlotinib. Our study indicates that EGFR and mTOR are clinically important targets in bronchial neuroendocrine tumors, and further in vivo and clinical exploration of combined inhibition is warranted. Copyright © 2012 S. Karger AG, Basel.

  17. Long-term follow-up of a large series of patients with type 1 gastric carcinoid tumors: data from a multicenter study.

    PubMed

    Thomas, Dimitrios; Tsolakis, Apostolos V; Grozinsky-Glasberg, Simona; Fraenkel, Merav; Alexandraki, Krystallenia; Sougioultzis, Stavros; Gross, David J; Kaltsas, Gregory

    2013-02-01

    To study the clinical presentation, diagnostic approach, response to treatment, and the presence of other pathologies in patients with gastric carcinoid type 1 (GC 1) tumors. Retrospective analysis of 111 patients from four institutions and a mean follow-up of 76 months. The main indications for gastroscopy were upper gastrointestinal tract symptoms. The mean number of lesions, maximum tumoral diameter, and percentage of cells expressing Ki-67 labeling index were 3.6±3.8, 8±12.1 mm and 1.9±2.4% respectively. Serum gastrin and chromogranin A (CgA) levels were elevated in 100/101 and 85/90 patients respectively. Conventional imaging studies demonstrated pathology in 9/111 patients. Scintigraphy with radiolabeled octreotide was positive in 6/60 without revealing any additional lesions. From the 59 patients who had been followed-up without any intervention, five developed tumor progression. Thirty-two patients were treated with long-acting somatostatin analogs (SSAs), leading to a significant reduction of gastrin and CgA levels, number of visible tumors, and CgA immune-reactive tumor cells in 28, 19, 27, and 23 treated patients respectively. Antrectomy and/or gastrectomy were initially performed in 20 patients and a complete response was achieved in 13 patients. The most common comorbidities were vitamin B12 deficiency, thyroiditis, and parathyroid adenomas. Most GCs1 are grade 1 (82.7%) tumors presenting with stage I (73.9%) disease with no mortality after prolonged follow-up. Ocreoscan did not provide further information compared with conventional imaging techniques. Treatment with SSAs proved to be effective for the duration of administration.

  18. Can C-Arm Cone-Beam CT Detect a Micro-Embolic Effect After TheraSphere Radioembolization of Neuroendocrine and Carcinoid Liver Metastasis?

    PubMed Central

    Pellerin, Olivier; Lin, MingDe; Bhagat, Nikhil; Shao, Wenbo

    2013-01-01

    Abstract Rational and Objective Radioembolization with yttrium-90 microspheres is a therapy that is used for hepatic tumors. 20–30 μm microspheres loaded with Y90 are supposedly occluding tumor vessels at the capillary level. Then, these spheres deliver high-dose radiation to the tumor. However, this theoretical embolic effect has never been appreciated in imaging. Dual-Phase cone-beam computed tomography (DPCBCT) is a multi-phasic intra-procedural scan that uses only one contrast media injection to visualize early (feeding vessel) and delayed (capillary level) tumor enhancement. The purpose of this study was to determine whether there is a micro-embolic effect induced by TheraSpheres® (MDS Nordion, Ottawa, Ontario, Canada) at the capillary level by using DPCBCT imaging. Materials and Methods 14 patients with 72 carcinoid or neuroendocrine tumors were treated with radioembolization, and all underwent DPCBCT (Allura Xper, Philips Healthcare) imaging before and immediately after radioembolization with TheraSpheres®. Tumor enhancement was measured in each phase by drawing a region of interest within the tumors. Results 72 tumors were evaluated: average tumor density in the early arterial phase was 241 and 230 Hounsfield units (HU) (p<0.001) before and after radioembolization, respectively; the average density in the delayed arterial phase was 226 and 161 HU (p<0.001) before and after radioembolization, respectively. Average difference in tumor attenuation before and after radioembolization in early arterial and delayed phase was 11 HU and 64 HU (p<0.001), respectively. Conclusion The significant decrease in tumor enhancement in the DPCBCT delayed phase after TheraSpheres® injection indicates that there is an appreciable microembolic effect at the tumor capillary bed level. PMID:23484809

  19. Foraminotomia cervical posterior en el tratamiento de conflictos foraminales

    PubMed Central

    Campero, Álvaro; Barrera, Ramiro; Ajler, Pablo

    2012-01-01

    Introducción: La foraminomotima cervical posterior es un procedimiento utilizado para la descompresion radicular por via posterior y constituye una alternativa a la via clásica anterior. En este trabajo evaluamos nuestra serie de pacientes tratados por esta via. Método: Desde enero de 2008 a diciembre de 2011, 17 pacientes (18 foraminotomías) fueron operados por presentar cervicobraquialgia a causa de un conflicto foraminal, realizando un foraminotomía cervical posterior. Los pacientes fueron evaluados en el postoperatorio inmediato, al mes y a los 3 meses de la cirugía. Los parámetros para valorar los resultados fueron la Escala Análoga del Dolor (VAS), la Neck Disability Index y los criterios de Odom. Resultados: El dolor radicular por conflicto foraminal secundario a hernia de disco cervical fue el síntoma y la patología predominante. El nivel más afectado fue C5-C6. La resolución completa del dolor radicular se observó en casi todos los pacientes. La VAS preoperatoria en promedio fue de 8.8 (mínimo 8 – máximo 10), con una franca mejoría en todos los casos (0.4 en el último control). La media en la Neck Disability Index al inicio fue de 35.3 (mínimo 32 – máximo 45), con una evolución favorable en la evaluación final (0.6). Los Criterios de Odom para la evaluación de pacientes operados de columna cervical fueron satisfactorios con un promedio de 1.17. Se observaron complicaciones en 4 pacientes (23%), todas tuvieron una evolución favorable. No hubo infecciones, discitis ni empeoramiento de los síntomas preexistentes en ningún paciente. Conclusión: La foraminotomía cervical posterior es un procedimiento efectivo para el tratamiento del dolor radicular en los conflictos foraminales PMID:23596556

  20. El uso de la neuromodulación para el tratamiento del temblor

    PubMed Central

    Bendersky, Damián; Ajler, Pablo; Yampolsky, Claudio

    2014-01-01

    Introducción: El temblor puede ser un desorden incapacitante y el tratamiento de primera línea para estos pacientes es farmacológico. Sin embargo, este tratamiento puede llevar a una reducción satisfactoria del temblor en sólo el 50% de los pacientes con temblor esencial. La talamotomía era el tratamiento de elección para el temblor refractario al tratamiento médico hasta que comenzó a utilizarse la estimulación cerebral profunda (ECP) del núcleo ventral intermedio (Vim) del tálamo. En la actualidad, raramente se realiza la talamotomía. Métodos: Este artículo es una revisión no sistemática de las indicaciones, resultados, parámetros de programación y técnica quirúrgica de la ECP del Vim para el tratamiento del temblor. Resultados: Aunque los resultados clínicos son similares usando la talamotomía o la ECP del Vim, la primera causa más efectos adversos que la última. Además, la ECP puede ser usada bilateralmente, mientras que la talamotomía tiene un alto riesgo de causar disartria cuando se realiza de ambos lados. La ECP del Vim logró una adecuada mejoría del temblor en varias series de pacientes con temblor causado por temblor esencial, enfermedad de Parkinson o esclerosis múltiple. Además del Vim, hay otros blancos que están siendo usados por varios autores, tales como la zona incerta y las radiaciones prelemniscales. Conclusión: La ECP del Vim es un tratamiento útil para el temblor incapacitante refractario al tratamiento médico. Es esencial realizar una precisa selección de pacientes, así como utilizar una técnica quirúrgica correcta. Aún se desconoce el mejor blanco estereotáctico para el temblor, aunque el Vim es el más usado. PMID:25165613

  1. mTOR, p70S6K, AKT, and ERK1/2 levels predict sensitivity to mTOR and PI3K/mTOR inhibitors in human bronchial carcinoids.

    PubMed

    Gagliano, Teresa; Bellio, Mariaenrica; Gentilin, Erica; Molè, Daniela; Tagliati, Federico; Schiavon, Marco; Cavallesco, Narciso Giorgio; Andriolo, Luigi Gaetano; Ambrosio, Maria Rosaria; Rea, Federico; Degli Uberti, Ettore; Zatelli, Maria Chiara

    2013-08-01

    Bronchial carcinoids (BCs) are rare neuroendocrine tumors that are still orphans of medical treatment. Human BC primary cultures may display resistance to everolimus, an inhibitor of the mammalian target of rapamycin (mTOR), in terms of cell viability reduction. Our aim was to assess whether the novel dual phosphatidylinositol 3-kinase (PI3K)/mTOR inhibitor NVP-BEZ235 is effective in everolimus-resistant human BC tissues and cell lines. In addition, we searched for possible markers of the efficacy of mTOR inhibitors that may help in identifying the patients who may benefit from treatment with mTOR inhibitors, sparing them from ineffective therapy. We found that NVP-BEZ235 is twice as potent as everolimus in reducing cell viability and activating apoptosis in human BC tissues that display sensitivity to mTOR inhibitors, but is not effective in everolimus-resistant BC tissues and cell lines that bypass cyclin D1 downregulation and escape G0/G1 blockade. Rebound AKT activation was not observed in response to treatment with either mTOR inhibitor in the 'resistant' BC cells. In addition to total mTOR levels, putative markers of the sensitivity of BCs to mTOR inhibitors are represented by AKT, p70S6K (RPS6KB2), and ERK1/2 (MAPK3/1) protein levels. Finally, we validated these markers in an independent BC group. These data indicate that the dual PI3K/mTOR inhibitor NVP-BEZ235 is more potent than everolimus in reducing the proliferation of human BC cells. 'Resistant' cells display lower levels of mTOR, p70S6K, AKT, and ERK1/2, indicating that these proteins may be useful as predictive markers of resistance to mTOR and PI3K/mTOR inhibitors in human BCs.

  2. Carcinoid and neuroendocrine tumors: building on success.

    PubMed

    Kunz, Pamela L

    2015-06-01

    We have come a long way in our understanding and treatment of neuroendocrine tumors since the term "karzinoide" was coined in 1907. Neuroendocrine tumors are a group of biologically and clinically heterogeneous neoplasms that most commonly originate in the lungs, GI tract, and pancreas. The selection of initial and subsequent therapies requires careful consideration of both tumor and treatment characteristics. With recent advances, we now have more tools for the diagnosis and treatment of our patients. This comprehensive review article summarizes recent advances in the field of neuroendocrine tumors and places them into context for best management practices.

  3. Metastatic Midgut Carcinoid in the Myocardium.

    PubMed

    Bukowczan, Jakub; Lois, Konstantinos B; Skinner, Jane; Petrides, George; James, Robert Andrew; Perros, Petros

    2015-09-01

    Metastasis of neuroendocrine tumor to the myocardium is rare. We present a case of 64-year-old woman, who presented initially with abdominal pain and large adnexal mass. The image-guided biopsy showed low-grade neuroendocrine tumor with Ki67 less than 2% within the ovarian tissue. CT staging revealed bilateral adnexal masses, liver metastases, and primary lesion in the terminal ileum. Octreoscan showed marked tracer uptake within the lower esophagus not related to obvious mass on CT scan; the echocardiography confirmed the presence of a 2.7 cm LV/LA mass. In this case, close correlation between ECHO and the octreoscan obviated need for myocardial biopsy.

  4. Treatment of Gastrointestinal Carcinoid Tumors by Stage

    MedlinePlus

    ... partial gastrectomy) along with nearby lymph nodes. Small intestine Some small tumors in the duodenum (the first ... vessels and lymph nodes) for larger tumors. Large intestine (other than appendix and rectum) The usual treatment ...

  5. How Are Gastrointestinal Carcinoid Tumors Diagnosed?

    MedlinePlus

    ... tumors shrink. Written by References The American Cancer Society medical and editorial content team Our team is ... 2015 Last Revised: February 8, 2016 American Cancer Society medical information is copyrighted material. For reprint requests, ...

  6. What Is a Gastrointestinal Carcinoid Tumor?

    MedlinePlus

    ... starts in. Written by References The American Cancer Society medical and editorial content team Our team is ... 2015 Last Revised: February 8, 2016 American Cancer Society medical information is copyrighted material. For reprint requests, ...

  7. Can Gastrointestinal Carcinoid Tumors Be Found Early?

    MedlinePlus

    ... may also be found when parts of the gastrointestinal system are removed to treat other diseases. For example, a person with stomach pain or bleeding may have a test called an upper endoscopy to look for an ulcer. In this test, the doctor looks at the stomach ... Medical Review: February 26, 2015 ...

  8. Propiedades biomecánicas de la membrana limitante interna tras recibir tratamiento intravítreo con ocriplasmina.

    PubMed

    Vielmuth, Franziska; Schumann, Ricarda G; Spindler, Volker; Wolf, Armin; Scheler, Renate; Mayer, Wolfgang J; Henrich, Paul B; Haritoglou, Christos

    2017-01-01

    Objetivo: Evaluar la rigidez de la membrana limitante interna (MLI) humana y evaluar los posibles cambios de las propiedades mecánicas tras administrar una inyección intravítrea de ocriplasmina para tratar la tracción vitreomacular. Métodos: Este estudio se compone de una serie de casos intervencionales y comparativos de 12 muestras de MLI extraídas mediante cirugía y obtenidas de forma consecutiva de 9 ojos de 9 pacientes después de someterse sin éxito a vitreólisis farmacológica con ocriplasmina. Durante el mismo periodo de tiempo, 16 muestras de otros 13 ojos sin tratamiento con ocriplasmina se obtuvieron mediante vitrectomía y sirvieron como controles. Todos los pacientes presentaron agujeros maculares o tracción vitreomacular y se sometieron a vitrectomía con disección de la MLI tanto con tinción con azul brillante (AB) como sin ella. Todas las muestras se analizaron con un microscopio de fuerza atómica con imágenes de las regiones de 25 × 25 μm. En todas las muestras, se analizaron tanto la parte de la retina como la del vítreo de la MLI. Resultados: La microscopia de fuerza atómica no reveló diferencias significativas en cuanto a elasticidad de las muestras de MLI extraídas de ojos con o sin tratamiento con ocriplasmina. Las áreas onduladas de la parte de la retina presentaron una mayor rigidez que la parte del vítreo de la MLI. La cartografía topográfica tanto de la parte del vítreo como de la retina de la MLI no mostró ninguna alteración aparente de la morfología en ojos tratados con ocriplasmina en comparación con los ojos no tratados. La tinción con azul brillante conllevó un aumento de la rigidez tisular. Conclusiones: Las inyecciones intravítreas de ocriplasmina no varían las propiedades biomecánicas de la MLI humana. No existen pruebas de un posible efecto enzimático que interfiera con la rigidez de esta membrana basal. © 2017 S. Karger AG, Basel.

  9. [Prevalence of gastrointestinal disorders in adults with common variable immunodeficiency at Specialty Hospital Dr. Bernardo Sepulveda].

    PubMed

    Rodríguez-Negrete, Elda Victoria; Mayoral-Zavala, Arturo; Rodríguez-Mireles, Karen Alicia; Díaz de León-Salazar, Oscar Edmundo; Hernández-Mondragón, Oscar; Gómez-Jiménez, Luz María; Moreno-Alcántar, R; González-Virla, Baldomero

    2015-01-01

    Antecedentes: la incidencia de la inmunodeficiencia común variable (IDCV) es de 1 por cada 15,000 a 117,000 casos, sin predominio de género. La incidencia de manifestaciones gastrointestinales en estos pacientes es de 20 a 60% y pueden ser la primera y única manifestación clínica de IDCV. En México existe escasa información en relación con el tipo y frecuencia de alteraciones gastrointestinales que padecen los pacientes adultos con IDCV. Objetivo: determinar la prevalencia de alteraciones gastrointestinales en pacientes adultos con inmunodeficiencia común variable. Material y método: estudio descriptivo, observacional y transversal en el que participaron pacientes con inmunodeficiencia común variable de la Clínica de Inmunodeficiencias del Servicio de Alergia e Inmunología Clínica del Hospital de Especialidades, Centro Médico Nacional Siglo XXI. A todos los pacientes se les aplicó un cuestionario de síntomas gastrointestinales y se les realizaron estudios de laboratorio, gabinete, endoscopia y prueba de aliento para determinar sobrepoblación bacteriana. Resultados: evaluamos 17 pacientes, 8 hombres y 9 mujeres, con edad promedio de 36 años y diagnóstico definitivo de inmunodeficiencia común variable de acuerdo con criterios internacionales. El 59% refirió dolor abdominal, 53% distensión abdominal y 17.6% estreñimiento. El 47% tenía diarrea crónica, en dos de ellos (11.8%) acompañada de pujo rectal. Las enfermedades gastrointestinales de esta población fueron: 18% diarrea crónica, enfermedad celiaca y sobrepoblación bacteriana, 24% trastorno funcional digestivo, 12% estreñimiento, 6% dispepsia. Sólo un paciente (6%) no tenía síntomas gastrointestinales. Conclusión: la prevalencia de las enfermedades gastrointestinales en pacientes adultos con inmunodeficiencia común variable fue de 94%, sin predominio de género. Debido a la frecuencia de manifestaciones gastrointestinales, es importante realizar protocolos de estudio al respecto

  10. Comparación de la efectividad de ranibizumab intravítreo para el tratamiento del edema macular diabético en ojos vitrectomizados y no vitrectomizados.

    PubMed

    Koyanagi, Yoshito; Yoshida, Shigeo; Kobayashi, Yoshiyuki; Kubo, Yuki; Yamaguchi, Muneo; Nakama, Takahito; Nakao, Shintaro; Ikeda, Yasuhiro; Ohshima, Yuji; Ishibashi, Tatsuro; Sonoda, Kohhei

    2017-07-11

    Objetivo: Comparar la efectividad de ranibizumab intravítreo (RIV) para el tratamiento del edema macular diabético (EMD) en ojos con y sin vitrectomía previa. Procedimientos: Evaluamos de manera prospectiva la mejor agudeza visual corregida (MAVC) y el grosor macular central (GMC) tras el tratamiento con RIV durante 6 meses. Resultados: No se observaron diferencias significativas en la MAVC o GMC inicial en ninguno de los dos grupos. En el grupo no vitrectomizado (n = 15), los cambios medios en la MAVC y GMC hasta el sexto mes de tratamiento con respecto al valor inicial resultaron significativos (p < 0,01). En el grupo vitrectomizado (n = 10), se observó una mejora más lenta, y la mejora media en la MAVC no resultó significativa (p = 0,5), aunque la media en la disminución del GMC sí que lo fue (p < 0,05). No se observaron diferencias significativas en los cambios medios en la MAVC y el GMC entre ambos grupos a los 6 meses del tratamiento. Conclusiones: La diferencia en la efectividad de RIV entre ambos grupos no resultó significativa. Ranibizumab intravítreo puede ser una opción de tratamiento incluso en pacientes vitrectomizados con EMD. © 2017 S. Karger AG, Basel.

  11. Extensión del Formalismo de Orbitales de Defecto Cuántico al tratamiento del efecto Stark (SQDO).

    NASA Astrophysics Data System (ADS)

    Menéndez, J. M.; Martín, I.; Velasco, A. M.

    El estudio experimental de las interacciones de átomos Rydberg altamente excitados con campos eléctricos ha experimentado un creciente interés durante las dos últimas décadas debido, en gran medida, al desarrollo de nuevas técnicas para crear y estudiar átomos Rydberg en el laboratorio. Acompañando a estas nuevas técnicas experimentales, es necesario el desarrollo de modelos teóricos que nos permitan contrastar sus medidas y conocer mejor los fundamentos de los mismos. Desde el punto de vista teórico el conocimiento del desdoblamiento de los niveles energéticos de un átomo en función de la magnitud del campo eléctrico aplicado (lo que se conoce como mapa Stark) es el mejor punto de partida para la descripción del sistema y un prerrequisito fundamental para el cálculo de distintas propiedades atómicas en presencia del campo eléctrico tales como intensidades de transición, umbrales de ionización de campo eléctrico, tiempos de vida, posición y anchura de cruces evitados, etc. En este trabajo presentamos la adaptación del método de orbitales de defecto cuántico [1,2,3] al tratamiento del efecto Stark (SQDO) [4] y su aplicación al cálculo de los desdoblamientos energéticos y fuerzas de oscilador de estados Rydberg en los átomos de Li, Na y K. El propósito de este estudio es, por un lado, desarrollar métodos fiables para la determinación de propiedades atómicas en presencia de campos eléctricos y, por otro, mostrar la fiabilidad de las funciones de onda QDO en la descripción del efecto Stark en sistemas atómicos.

  12. Nuevas estrategias de gestión, tratamiento y valorización de los efluentes organicos pecuarios: Experiencias en USDA. (Management strategies for organic livestock effluents,innovative treatment and valorization)

    USDA-ARS?s Scientific Manuscript database

    En la actualidad el impacto potencial de los residuos ganaderos en el medio-ambiente representa uno de los desafíos más grandes de la agricultura. Las tecnologías de tratamiento pueden tener un importante papel en el manejo de los residuos ganaderos dando más flexibilidad en los programas de la apli...

  13. Prognostic factors in multiple myeloma: definition of risk groups in 410 previously untreated patients: a Grupo Argentino de Tratamiento de la Leucemia Aguda study.

    PubMed

    Corrado, C; Santarelli, M T; Pavlovsky, S; Pizzolato, M

    1989-12-01

    Four hundred ten previously untreated multiple myeloma patients entered onto two consecutive Grupo Argentino de Tratamiento de la Leucemia Aguda (GATLA) protocols were analyzed to identify significant prognostic factors influencing survival. The univariate analysis selected the following variables: performance status, renal function, percentage of bone marrow plasma cells at diagnosis, hemoglobin, and age. A multivariate analysis showed that performance status, renal function, percentage of bone marrow plasma cells, hemoglobin, and age were the best predictive variables for survival. A score was assigned to each patient according to these variables, which led to their classification in three groups: good, intermediate, and poor risk, with a probability of survival of 26% and 10% at 96 months, and 5% at 56 months, and median survival of 60, 37, and 14 months, respectively (P = .0000). In our patient population, this model proved to be superior to the Durie-Salmon staging system in defining prognostic risk groups, and separating patients with significantly different risks within each Durie-Salmon stage.

  14. Intussusception secondary to a carcinoid tumor in an adult patient☆

    PubMed Central

    Wiener-Carrillo, Isidoro; González-Alvarado, Carlos; Cervantes-Valladolid, Mario; Echaverry-Navarrete, Denis; Zubieta-O’Farrill, Gregorio; Gudiño-Chávez, Andrés

    2014-01-01

    INTRODUCTION Intussusception in adult patients represents 5% of all intussusceptions and 1–5% of bowel obstructions in adults. In contrast to pediatric patients, 90% of the time, in adults, it's caused by well-established pathologic mechanisms, such as carcinoma, polyps, diverticula, Meckel diverticula, stenosis, or benign neoplasms. Small intestine intussusceptions are more frequent, but colonic intussusceptions are caused 50% of the time by malignant neoplasms, especially adenocarcinoma. PRESENTATION OF CASE We present a 70-year-old woman, with no relevant familial history, who presented with a 3-day symptomatology consisting of epigastric, colic, diffuse, abdominal pain of moderate intensity, which progressed till reaching a severe intensity, also referring abdominal distension, nausea, and gastrointestinal-content vomits. DISCUSSION In adult patients, the exact mechanism of intussusception is unknown in 8–20% of the cases, however, secondary intussusception can occur with any lesion of the intestinal wall or any irritant factor in its lumen that alters normal peristaltic activity and that could serve as a trigger to start an intussusception of one bowel segment over another the most common site is the small intestine. CONCLUSION Intussusception represents an unusual problem in adult patients; it requires a high clinical suspicion, mainly as a differential diagnosis in patients with intestinal obstruction, and it clinically presents as a subacute or chronic illness. CT represents the most useful diagnostic tool. An attempt to perform reduction procedures in small intestine intussusceptions can be done, however, in ileocolic or colonic intussusceptions, a formal resection of the segment is recommended. PMID:24727207

  15. What's New in Gastrointestinal Carcinoid Tumors Research and Treatment?

    MedlinePlus

    ... approach is still only available in the United States as a part of a clinical trial . Written by References The American Cancer Society medical and editorial content team Our team is made up of doctors and master’s-prepared nurses with deep knowledge of cancer care as well as journalists, ...

  16. Tratamiento Quirúrgico de los Meningiomas del Foramen Óptico, Técnicay Resultados de una Serie de 18 Pacientes

    PubMed Central

    Goldschmidt, Ezequiel; Ajler, Pablo; Campero, Álvaro; Landriel, Federico; Sposito, Maximiliano; Carrizo, Antonio

    2014-01-01

    Introducción: los meningiomas del foramen óptico producen un rápido deterioro de la función visual aún cuando su tamaño es pequeño, por eso su diagnóstico y manejo difiere del resto de los meningiomas clinoideos. El propósito de este estudio es presentar la técnica y los resultados de nuestro manejo quirúrgico de meningiomas foraminales (MF). Pacientes y Métodos: se llevó a cabo una revisión de las historias clínicas de 47 pacientes con meningiomas primarios intraorbitarios. Se realizaron 52 cirugías en los pacientes con MF. Se empleó una craneotomía fronto-orbitaria, seguida de una descompresión extradural del canal óptico, resección del componente intraorbitario y exploración intradural del nervio óptico. Resultados: de los 12 pacientes con MF que presentaban la visión conservada, la agudeza visual fue preservada en 7 casos, mejoró en 2, y empeoró en 3. En 18 pacientes, el principal síntoma fue exoftalmos y en 35 pacientes ceguera unilateral. Ocurrieron 6 recurrencias, 2 a 10 años después de la resección quirúrgica. Cinco de ellos fueron reoperados. Se indicó radioterapia después de la recurrencia en 3 pacientes. Conclusión: el manejo de los MF continúa siendo controvertido y frecuentemente se propone un tratamiento conservador. Basados en nuestros hallazgos de frecuente extensión intracraneal, proponemos realizar una resección total o subtotal del tumor, preservando el nervio óptico en pacientes con visión prequirúrgica conservada. PMID:25165616

  17. [Characteristics and mortality following primary percutaneous coronary intervention for acute myocardial infarction in Spain. Results from the TRIANA 1 (TRatamiento del Infarto Agudo de miocardio eN Ancianos) Registry].

    PubMed

    Cequier, Angel; Bueno, Héctor; Augé, Josep M; Bardají, Alfredo; Fernández-Ortiz, Antonio; Heras, Magda

    2005-04-01

    Although more elderly patients will experience acute myocardial infarction (AMI) in coming years, the best reperfusion strategy in these patients remains unknown. The Spanish TRIANA (TRatamiento del Infarto Agudo de miocardio eN Ancianos) registry was set up to determine the feasibility of performing a randomized study of percutaneous coronary intervention (PCI) versus thrombolysis in AMI patients aged > or =75 years. The TRIANA 1 subregistry included consecutive patients of all ages with ST-segment-elevation AMI (< or =12 h from onset) who underwent PCI in selected hospitals. In total, 459 TRIANA-1 patients were included at 25 hospitals over 3 months. Some 11% had cardiogenic shock. PCI was performed as rescue therapy in 24% and, in 15% because thrombolysis was contraindicated. After PCI, 83% had TIMI grade-3 flow without significant residual stenosis. Mortality at 1 month was 10.8%. Independent predictors of mortality identified by multivariate analysis were: cardiogenic shock at admission (OR, 7.2, 95% CI, 2.2-23.3), age (OR, 1.05 per year, 95% CI, 1.005-1.100), maximum creatine kinase MB fraction (OR, 1.01, 95% CI, 1.004-1.270) and post-PCI TIMI grade <3 (OR, 2.8, 95% CI, 1.0-8.3). Of 104 participants aged > or =75 years (mortality, 24%), 58 (55.7%) fulfilled criteria for inclusion in a randomized study. 1) The TRIANA 1 subregistry probably reflects the reality of PCI for AMI in Spain. 2) Mortality at 1 month was associated with classic predictive factors. 3) Some 50% of patients > or =75 years old who underwent PCI could be included in a randomized study. These findings indicate that randomized study to determine the best reperfusion strategy in elderly AMI patients is feasible.

  18. PubMed

    García Almeida, Jose M; Lupiáñez Pérez, Yolanda; Blanco Naveira, Mercedes; Ruiz Nava, Josefina; Medina, José Antonio; Cornejo Pareja, Isabel; Gómez Pérez, Ana; Molina Vega, María; López-Medina, José A; Tinahones Madueño, Francisco

    2017-06-05

    Introducción: en pacientes con riesgo nutricional, la Sociedad Europea de Clínica y Metabolismo (ESPEN) y Parenteral recomienda suplementos nutricionales durante el tratamiento oncológico para prevenir la pérdida de peso involuntaria.Objetivos: nuestro objetivo es conocer el cumplimiento, la aceptabilidad y la tolerancia de un suplemento hiperproteico, hipercalórico, rico en omega 3 en pacientes oncológicos.Métodos: estudio unicéntrico, observacional y prospectivo en pacientes oncológicos con un suplemento nutricional hiperproteico, hipercalórico, rico en omega 3 y de bajo volumen. Fueron incluidos 30 pacientes con desnutrición o en riesgo de desnutrición. La suplementación duró seis días. Se evaluaron el cumplimiento (envases utilizado), la aceptabilidad (escala Madrid), las variables antropométricas y los acontecimientos adversos (AA) gastrointestinales.Resultados: el 70% fueron hombres, con una edad media de 60 años (rango: 32 a 79) y con neoplasias de pulmón (43,3%), ORL (26,7%) y mama (13,3%), en estadio III-IV (56,7%), tratados con radioterapia (93,3%), quimioterapia (60%) y cirugía (16,7%). El producto fue aceptado por todos los pacientes. Se observó un cumplimiento del 100%. En dos pacientes (6,7%) se observaron AA gastrointestinales (grado II) relacionados con el suplemento; ambos sujetos presentaban patologías gastrointestinales previas. La mediana del peso, índice de masa corporal (IMC) y proteínas ingeridas aumentó durante la suplementación (0,2 kg, 0,1 kg/m2 y 6,2 g). No se observaron diferencias respecto a la ingesta de calorías, lípidos y carbohidratos. Conclusión: la elevada aceptación y cumplimiento del suplemento nutricional específico se asoció con la mejora nutricional de los pacientes oncológicos, pues revirtió la pérdida de peso, sin presentar problemas gastrointestinales severos ni producir desplazamiento de la ingesta.

  19. [Pulmonary carcinoid: analysis of a single institutional experience and prognostic factors].

    PubMed

    Correia, Silvia da Silva; Pinto, Carlos; Bernardo, João

    2014-01-01

    IntroduçÉo: Os tumores carcinóides pulmonares sÉo tumores raros com origem nas células neuro-endócrinas do pulmÉo. Classificam-se de acordo com os critérios da OMS em carcinóides típicos ou atípicos. Quando comparados com outros tipos de neoplasia pulmonar, os tumores carcinóides apresentam melhor prognóstico.Objetivos: CaracterizaçÉo dos doentes com diagnóstico histológico de tumor carcinóide observados numa instituiçÉo. Análise dos fatores que influenciaram o prognóstico.Material e Métodos: Análise retrospetiva incluindo todos os doentes com diagnóstico histológico de tumor carcinóide pulmonar durante um período de 11 anos numa instituiçÉo. Os tumores foram classificados em típicos e atípicos de acordo com a classificaçÉo da OrganizaçÉo Mundial de Saúde de 2004. O estadiamento foi feito com base na classificaçÉo TNM de 2009 para o carcinoma do pulmÉo de nÉo pequenas células: T (Tumor); N (Ganglionar); M (Metástase).Resultados: Foram incluídos 59 doentes: 53 carcinóides típicos e seis carcinóides atípicos. Destes, 90% foram submetidos a cirurgia. O follow-up médio foi de 57 meses. A mortalidade operatória foi de 2% (n = 1) tratando-se de cirurgia paliativa para um doente em estádio IV. Em 49 doentes nÉo se verificou envolvimento ganglionar (N0), um doente apresentava doença N1, oito doença N2 e um doente doença N3. A sobrevivência global aos cinco anos foi de 79,2%: 80,2% nos carcinóides típicos e 66,7% nos carcinóides atípicos (p < 0,05). Nos doentes T1, a sobrevivência foi de 88,1% e de 58,2% nos T2-T4 (p < 0,01). Nos doentes N0 a sobrevivência aos cinco anos de 89,7% e de 36% para os doentes N1-N3 (p < 0,001). Os doentes com doença M0 apresentaram uma sobrevivência aos cinco anos de 85,9% sendo de 0% nos doentes M1 (p < 0,01). Dos 11 doentes que necessitaram de quimioterapia adjuvante, 45,4% eram carcinóides atípicos.DiscussÉo: Na nossa série, a cirurgia no tumor carcinóide pulmonar demonstrou-se segura, com uma baixa taxa de complicações no pós-operatório. Observou-se pior prognóstico em doentes com tumor carcinóide atípico, tumores com mais de 3 cm, com envolvimento ganglionar ou presença de metástase. A sobrevivência aos cinco anos nos tumores carcinóides típicos foi excelente (80,2%), correspondente à encontrada na literatura. No tumor carcinóide atípico, a sobrevivência aos cinco anos foi de 66,7% também concordante com os dados obtidos em estudos anteriores.Conclusões: Os tumores carcinóides pulmonares sÉo na maioria carcinóides típicos com uma excelente sobrevivência a longoprazo. A cirurgia de ressecçÉo é o tratamento de eleiçÉo nestes doentes. Os fatores relacionados com um pior prognóstico foram o subtipo histológico (carcinóides típicos versus carcinóides atípicos), o tamanho do tumor, o envolvimento ganglionar e a presença de metástases.

  20. Chromogranin-reactive endocrine cells in argyrophilic carcinomas ("carcinoids") and normal tissue of the breast.

    PubMed Central

    Bussolati, G.; Gugliotta, P.; Sapino, A.; Eusebi, V.; Lloyd, R. V.

    1985-01-01

    Breast carcinomas, either positive or negative with the Grimelius' silver procedure, benign fibroadenomas, duct papillomas, and areas of histologically normal breast tissue were tested immunocytochemically with the mouse monoclonal antibody LK2H10 directed against human chromogranin. This is regarded as a general stain for polypeptide-hormone-producing cells and tumors. In 3 of the 9 cases of argyrophilic carcinoma, but in none of 12 ductal infiltrating carcinomas, chromogranin-positive cells were found: the number of reactive cells was very low in 1 case, while in the other 2 carcinomas about 50% of the argyrophilic cells appeared stained. In areas of histologically normal breast tissue, rare argyrophilic chromogranin-positive cells were detected. This study is the first reported evidence concerning the presence of endocrinelike cells probably belonging to the diffuse neuroendocrine system in the normal mammary parenchyma. Our data are consistent with the endocrine nature of at least some of the breast argyrophilic carcinomas. Images Figure 1 Figure 2 Figures 3 and 4 Figure 5 Figure 6 PMID:4025508

  1. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

    PubMed Central

    Ahmed, A; Ardill, J; Bax, N; Breen, D J; Caplin, M E; Corrie, P; Davar, J; Davies, A H; Lewington, V; Meyer, T; Newell-Price, J; Poston, G; Reed, N; Rockall, A; Steward, W; Thakker, R V; Toubanakis, C; Valle, J; Verbeke, C; Grossman, A B

    2011-01-01

    These guidelines update previous guidance published in 2005. They have been revised by a group who are members of the UK and Ireland Neuroendocrine Tumour Society with endorsement from the clinical committees of the British Society of Gastroenterology, the Society for Endocrinology, the Association of Surgeons of Great Britain and Ireland (and its Surgical Specialty Associations), the British Society of Gastrointestinal and Abdominal Radiology and others. The authorship represents leaders of the various groups in the UK and Ireland Neuroendocrine Tumour Society, but a large amount of work has been carried out by other specialists, many of whom attended a guidelines conference in May 2009. We have attempted to represent this work in the acknowledgements section. Over the past few years, there have been advances in the management of neuroendocrine tumours, which have included clearer characterisation, more specific and therapeutically relevant diagnosis, and improved treatments. However, there remain few randomised trials in the field and the disease is uncommon, hence all evidence must be considered weak in comparison with other more common cancers. PMID:22052063

  2. Intractable hypoglycaemia in a patient with advanced carcinoid syndrome successfully treated with hepatic embolization.

    PubMed

    Kyriacou, Angelos; Mansoor, Was; Lawrance, Jeremy; Trainer, Peter J

    2016-01-01

    A male patient presented at the age of 54 years with metastatic pancreatic neuroendocrine tumour (NET). He was managed with interferon and multiple courses of MIBG therapy which controlled his disease for about seven years. He then developed symptomatic hypoglycaemia which resolved with the introduction of somatostatin analogue treatment and further therapeutic MIBG. However, three years later he was admitted to hospital with severe and intractable hypoglycaemia, which persisted despite treatment with dietary manipulation, diazoxide, long-acting octreotide injections, intravenous infusion of dextrose and octreotide and everolimus. Bland hepatic embolization was attempted as a last resort and resulted in prompt and dramatic improvement of his condition with no hypoglycaemia for five months. We recommend that hepatic embolization should be considered in patients with advanced and metastatic NETs accompanied by refractory hypoglycaemia, with the aim of symptomatic relief and palliation, and possibly some survival benefit.

  3. Perforated Meckel's diverticulum containing a carcinoid tumor successfully treated by the laparoscopic approach: Case report

    PubMed Central

    Curbelo-Peña, Yuhamy; Dardano-Berriel, Juan; Guedes-De la Puente, Xavier; Saladich-Cubero, Maria; Stickar, Tomas; De Caralt-Mestres, Enric

    2016-01-01

    Mekel's diverticulum is a gastrointestinal malformation. Occurs in one of every 40 patients. It is usually asymptomatic whereas complications can be developed in 2% to 4%. The report is based on a 41-year old male, who attended to emergency, complaining of right lower quadrant abdominal pain. Blood tests showed high level of inflammatory markers. With acute appendicitis as presumptive diagnosis, laparoscopy was performed. The intraoperative findings were: a perforated Mekel's diverticulum with normal cecal appendix. Mechanical diverticular resection was made. The patient was successfully recovered from surgery. Histopathology examination showed: Meckel's diverticulum perforated with acute inflammation and neuroendocrine tumor (G1) pT1. Mekel's diverticulum is rarely affected by inflammatory complications and just few cases are associated with tumors. However, has ever been described before, coexisting both situations, being our patient the first reported with this exceptional clinical presentation, and treated successfully by laparoscopic approach. PMID:27251847

  4. Ectopic Adrenocorticotropic Hormone-Secreting Bronchial Carcinoid Diagnosed by Balloon-Occluded Pulmonary Arterial Sampling.

    PubMed

    Yotsukura, Masaya; Kohno, Mitsutomo; Asakura, Keisuke; Kamiyama, Ikuo; Ohtsuka, Takashi; Hayashi, Yuichiro; Kurihara, Isao; Nakatsuka, Seishi; Asamura, Hisao

    2016-05-01

    We present the case of a 50-year-old man with Cushing syndrome caused by an ectopic adrenocorticotropic hormone (ACTH)-secreting tumor. A small nodule was located in close association with the lateral segmental branch of the pulmonary artery in the left upper lobe. Blood samples were obtained from various branches of the pulmonary artery by balloon-occluded retrograde sampling for the measurement of location-specific serum ACTH levels. After confirmation that the pulmonary nodule was responsible for the increased ACTH secretion, lobectomy was performed. This report demonstrates the usefulness of balloon-occluded retrograde pulmonary arterial sampling for the preoperative diagnosis of an ACTH-producing tumor whose diagnosis is difficult to confirm.

  5. Double transmanubrial approach and sternotomy for resection of a giant thymic carcinoid tumor.

    PubMed

    Spaggiari, L; Pastorino, U

    2001-08-01

    The transmanubrial approach allows excellent unilateral exposure of the thoracic outlet. However, selected patients may require a bilateral cervicomediastinal exposure to completely resect the neoplasm. We report the use of a "double" transmanubrial approach for the resection of a giant mediastinal mass requiring bilateral vascular dissection and superior vena cava system resection and replacement.

  6. [Adherence and toxicity to tyrosine kinase inhibitor therapy in chronic myeloid leukemia].

    PubMed

    González Rosa, V; Gutiérrez Nicolás, F; Gavira Moreno, R; Viña Romero, M M; Moreno Carvajal, M T; Gázquez Pérez, R

    2013-01-01

    Objetivo: Analizar la adherencia y la toxicidad del tratamiento con inhibidores de tirosinquinasa (TKIs) en pacientes diagnosticados de Leucemia Mieloide Crónica (LMC). Método: Estudio observacional retrospectivo de 18 meses de duración (enero 2011-junio 2012) en el que se incluyeron todos los pacientes diagnosticados de LMC en un hospital de se - gundo nivel (550 camas) en tratamiento con imatinib, dasatinib o nilotinib. Las variables recogidas fueron sexo, edad de diagnóstico, años de tratamiento y reacciones adversas. La adherencia se valoró mediante un sistema combinado basado en el autocuestionario SMAQ y el registro de dispensaciones. Resultados: Se incluyeron un total de 25 pacientes. El 92,0% experimentaron reacciones adversas a imatinib; 83,3% a dasatinib y 66,7% a nilotinib. La adherencia media fue de 71,3%. Se identificaron como posibles parámetros de falta de adherencia el sexo femenino (55,6% vs. 66,7%, p = 0,586), mayores de 50 años (55,6% vs. 83,3%, p = 0,125), más de cuatro años de duración de tratamiento (70,0% vs. 57,1%, p = 0,521) y la presencia de determinados efectos adversos (trastornos gastrointestinales y dolor musculoesquelético). Conclusiones: Casi un tercio de los pacientes en tratamiento fueron considerados no adherentes. A pesar de que el tamaño muestral no nos ha permitido establecer relaciones estadísticamente significativos entre la adherencia y las variables analizadas, la relevancia clínica de estos resultados muestran la importancia de realizar futuros estudios con poblaciones mayores que confirmen las tendencias establecidas en este estudio.

  7. Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors

    ClinicalTrials.gov

    2014-11-14

    Gastrinoma; Glucagonoma; Insulinoma; Metastatic Gastrointestinal Carcinoid Tumor; Neuroendocrine Tumor; Pancreatic Polypeptide Tumor; Recurrent Gastrointestinal Carcinoid Tumor; Recurrent Islet Cell Carcinoma; Somatostatinoma; WDHA Syndrome

  8. [The metoclopramide effect on enteral nutrition tolerance and mechanical ventilation associated pneumonia in neuro critically ill patients].

    PubMed

    Acosta-Escribano, Jose; Almanza López, Susana; Plumed Martín, Lidia; García Martinez, Miguel Angel; Tajadura Manjarín, Nuria

    2014-06-01

    Introducción: El uso de procinéticos en el paciente crítico con nutrición enteral, tienen como objetivo el reducir el aumento del residuo gástrico (RG). Analizamos su eficacia en la mejoría del aporte enteral y sobre la reducción en la incidencia complicaciones gastrointestinales (CGI) y neumonía, en pacientes críticos, con lesión neurológica Objetivos: Medir los efectos en la administración metoclopramida (MCG) durante los primeros cinco días con nutrición enteral, versus control (GC), sobre el volumen de dieta enteral administrada, el número de complicaciones gastrointestinales y la incidencia de neumonía asociada a ventilación mecánica (NAVM); en enfermos neurocríticos de etiología traumática y vascular. Métodos: De los 150 pacientes NC ingresados de forma consecutiva, 109 fueron aleatorizados en dos grupos: 58 MCG y 51 GC. Los objetivos primarios fueron: nutricionales: el volumen de dieta administrada (VDA), el volumen eficaz (VEM), el número de complicaciones gastrointestinales (CGI) y la tasa de suspensión temporal y definitiva de la dieta. Infecciosos: incidencia de neumonía asociada a ventilación mecánica (NAVM). Fueron objetivos secundarios: la duración de la ventilación mecánica, la estancia en UCI y hospitalaria, la secuela neurológica grave al alta y la mortalidad a los 30 días. Resultados: No se observaron diferencias en los parámetros de gravedad entre grupos al ingreso. Un incremento significativo fue observado en el análisis global y a los cinco días (p < 0,03) del VEM en el grupo de MCG. Los valores del VDA global y durante las dos fases de estudio, el número de CGI y el número de suspensiones parciales y definitivas de la dieta o el número de NAVM fueron similares en ambos grupos, no significativos. Tampoco se observaron diferencias en los diferentes objetivos secundarios Conclusión: El uso de metoclopramida en el enfermo neurocrítico, no es eficaz en la disminución de las CGI, en las dosis y tiempo de

  9. [Clinical applications of the use of probiotics in pediatrics].

    PubMed

    Álvarez-Calatayud, Guillermo; Pérez-Moreno, Jimena; Tolín, Mar; Sánchez, César

    2013-01-01

    Introducción: El empleo de probióticos supone un novedoso avance en el campo de la Pediatría puesto que pueden ser útiles en la prevención y tratamiento de múltiples patologías gastrointestinales, constituyendo un elemento más en nuestro arsenal terapéutico. Objetivo: En este artículo se presenta una revisión actualizada de la literatura científica sobre el uso de los probióticos en Pediatría, principalmente en problemas gastrointestinales con alteración en la microbiota intestinal describiéndose las principales aplicaciones del empleo de los probióticos y prebióticos en la infancia y repasando las líneas de investigación futuras. Resultados y conclusiones: A pesar de existir suficiente evidencia científica en varias patologías, la utilización de probióticos no está del todo incorporado a la práctica clínica habitual de los pediatras. Se emplea en el contexto de las enfermedades gastrointestinales (diarrea aguda infecciosa, la diarrea asociada a antibióticos, sobredesarrollo bacteriano) y, más recientemente, en procesos inflamatorios crónicos como la enfermedad inflamatoria intestinal o en trastornos funcionales como el cólico del lactante o el estreñimiento. También se ha valorado su efecto beneficioso en alteraciones extraintestinales, tales como la alergia (dermatitis atópica) o los efectos sobre las mucosas respiratorias o urogenitales y, en los últimos años, en la prevención de patología del recién nacido pretérmino y en la infección por H. pylori. Además existen varias líneas de investigación abiertas en la suplementación alimentaria con probióticos y prebióticos. Cada cepa prebiótica debe ser estudiada individualmente y extensamente para determinar su eficacia y seguridad en todas aquellas situaciones en que su empleo puede ser aconsejable.

  10. [Fiber-type indication among different pathologies].

    PubMed

    Sánchez Almaraz, Rosalía; Martín Fuentes, María; Palma Milla, Samara; López Plaza, Bricia; Bermejo López, Laura M; Gómez Candela, Carmen

    2015-06-01

    Introducción: la fibra engloba los carbohidratos no digeridos ni absorbidos en el tubo digestivo, llegando intactos al colon. Se clasifica en soluble e insoluble, con propiedades fisiológicas distintas. La fibra se ha empleado frecuentemente para tratar y prevenir diversas patologías gastrointestinales. También se ha estudiado su papel en la fisiopatología de enfermedades como la diabetes, la dislipemia, la hipertensión arterial y la obesidad. Además se ha relacionado su consumo con la prevención de ciertos tumores, en especial del cáncer colorrectal, así como con el aumento de la excreción de nitrógeno en heces. Objetivos: analizar la evidencia del papel que la fibra puede tener en el tratamiento y prevención de distintas enfermedades, así como el tipo de fibra más adecuado en cada una. Métodos: revisión no sistemática en Medline y Pubmed, y posterior aplicación de los criterios de inclusión y exclusión. Resultados: diferentes tipos de fibra pueden ser útiles en el tratamiento de enfermedades gastrointestinales, como el estreñimiento, la diarrea, el síndrome de intestino irritable, la colitis ulcerosa en remisión o el síndrome de intestino corto. Los pacientes con diabetes, obesidad, hiperlipidemia, hipertensión y enfermedad cardiovascular también pueden beneficiarse del consumo principalmente de fibra soluble. La fibra alimentaria ha demostrado prevenir el cáncer de colon y otros tumores. En pacientes con encefalopatía hepática o insuficiencia renal, la fibra fermentable ha demostrado beneficios. Conclusiones: la fibra tiene un papel importante en la prevención y tratamiento de múltiples enfermedades; sin embargo, son necesarios más estudios de calidad para poder realizar recomendaciones más específicas.

  11. Correlación entre síntomas gastrointestinales funcionales y la histopatología de la mucosa gástrica, incluyendo la infección por Helicobacter pylori, en Lima, Perú.

    PubMed

    McDonald, Katharine; Shopinski, Samuel; Wilkinson, Ariana; Meza, Claudia; Cok, Jaime; Bussalleu, Alejandro; Valdivieso, Manuel

    2015-01-01

    This study aims to investigate the relationship between functional gastrointestinal disorders and histopathology characteristics, including H. pylori infection, of gastric mucosa, at Cayetano Heredia National Hospital, Lima-Peru, in 2013. 112 patients were interviewed prospectively between June and July 2013 in the gastroenterology service. Dyspepsia, irritable bowel syndrome, and postprandial distress syndrome were characterized using the Rome III Survey. Pathology results were determined by gastric biopsies obtained by endoscopy. Of the patients interviewed, biopsy results were obtained for 101. 22.8% had atrophy, 24.8% had intestinal metaplasia, 57.4% presented with H pylori. Using chi-square analysis, no statistically significant relationship could be identified between clinical presentation and biopsy results.

  12. Vitamin D and vitamin B12 deficiencies are common in patients with midgut carcinoid (SI-NET).

    PubMed

    Lind, A; Wängberg, B; Ellegård, L

    2016-09-01

    Patients with small intestinal neuroendocrine tumours (SI-NET) often have diarrhoea from hormonal overproduction, surgery and medical treatment, leading to malabsorption of bile salts, fats, vitamin B12 and fat-souble vitamins. This could lead to malnutrition. We assessed nutritional status in 50 consecutive out patients with disseminated SI-NET, 25 patients in each cohort. The first cohort was descriptive and the second cohort supplemented with vitamin D, B12 and calcium. Vitamin D deficiency was defined as <50 nmol/l. All patients were assessed by clinical chemistry and dual-energy X-ray absorptiometry (DXA) and interviewed about weight changes, appetite, gastrointestinal disorders, sunhabits and the use of supplements. In the first cohort, 29% of the patients were severely and 17% moderately vitamin D deficient. In patients without prior substitution, 32% had subnormal vitamin B12 levels. Seventy-six percent had low bone density. In the second cohort with vitamin and mineral supplementation, none had severe vitamin D deficiency, but 28% had moderate deficiency. No patient had subnormal vitamin B12 levels. Sixty percent had low bone density. The serum levels of vitamin D and B12 were higher and parathyroid hormone (PTH) lower in the second cohort compared with the first cohort (P⩽0,022). Vitamin D and PTH were negatively correlated, r=-30, P=⩽0.036. Low serum levels of vitamin D and vitamin B12, and low bone density are common in patients with disseminated SI-NET. Supplementation of vitamin D, B12 and calcium resulted in higher serum levels of vitamins, lower PTH levels and diminished severe vitamin D deficiency and is thus recommended as standard care.

  13. Primary mature cystic teratoma with carcinoid mimicking an adrenal tumor: Report of a rare association and review of literature.

    PubMed

    Narla, Swetha Lakshmi; Jacob, Sheba; Kurian, Ann; Parameswaran, Ashok

    2016-01-01

    Teratomas are neoplasms comprising of tissues derived from more than one germ layer. Most common sites are gonads, sacrococcygeal, mediastinal, retroperitoneal, and pineal region. Here, we report a case of 2 -year-old previously healthy female toddlerwho presented with fever and right upper quadrant abdominal pain. Magnetic resonance imaging (MRI) of the abdomen revealed a right suprarenal nonenhancing cystic mass. Focal calcification was seen in the inferomedial aspect of the cyst. Complete surgical resection of the right adrenal gland was done. The patient had an uneventful postoperative course and was free of recurrence at 8 months follow-up. Primary retroperitoneal teratomas of the adrenal gland are very uncommon (4% of all primary teratomas) and can mimic a primary adrenal tumor, hence should be included in the differential diagnosis of adrenal masses.

  14. Desarrollo y validación de una nueva tecnología, basada en arginina al 1.5%, un compuesto de calcio insoluble y fluoruro, para el uso diario en la prevención y tratamiento de la caries dental.

    PubMed

    Cummins, D

    2013-10-22

    este artículo discute brevemente la prevalencia de caries, la naturaleza multifactorial de su etiología, el riesgo de caries y el papel y eficacia del fluoruro. Resalta también la investigación sobre el metabolismo bacteriano, que ha aportado conocimientos sobre la defensa natural oral contra la caries y la base para el desarrollo de una nueva tecnología para la prevención diaria y el tratamiento de la caries. Por último, se resume la evidencia que respalda que la tecnología complementa y mejora la eficacia anti-caries de la crema dental con fluoruro. los datos globales muestran que a pesar de la exitosa introducción del fluoruro, la caries dental es una enfermedad prevalente. La experiencia de caries depende del balance entre el consumo de azúcares, la higiene oral y el uso del fluoruro. Hay tres conceptos científicos que son fundamentales en las nuevas mediciones para detectar, tratar y monitorear la caries: (1) la caries dental es un proceso dinámico, (2) la caries dental es un proceso continuo de etapas que van desde reversible (pre-clínica) hasta irreversible (lesiones clínicamente detectables), y (3) el proceso de la caries es un balance de factores patológicos y protectores que pueden modularse para el manejo de la caries. El fluoruro funciona como factor protector al detener y revertir el proceso de la caries, pero el fluoruro no previene los factores patológicos que inician el proceso. Se ha identificado una tecnología novedosa, basada en arginina y un compuesto insoluble de calcio, que está dirigida a la placa dental para prevenir la iniciación del proceso de caries al reducir los factores patológicos. Como los mecanismos de acción de la arginina y el fluoruro son altamente complementarios, se ha desarrollado un nuevo dentífrico que combina la arginina y el fluoruro, y se ha probado clínicamente que brinda una prevención superior contra la caries. Copyright © 2013 Elsevier Ltd. All rights reserved.

  15. Como Lo Hago Yo: Tratamiento Quirurgico Del Mielomeningocele

    PubMed Central

    Portillo, Santiago

    2014-01-01

    En Argentina hay plan de fortificación con ácido fólico. Diagnostico prenatal no siempre es correcto. Cierre según técnica. Cerramos músculo. No favorecemos corpectomía temprana en casos de cifosis. Suturamos la plaqueta. Cerramos el plano muscular. Hidrocefalia: Válvula de derivación, generalmente dentro de los dos primeros meses. Ventriculostomía no está indicada. Chiari II. Laminectomia cervical alta. Siringomielia: Derivación desde la cavidad al peritoneo. PMID:24791219

  16. [Diphallia: a case report].

    PubMed

    Rossete-Cervantes, Héctor Enrique; Villegas-Muñoz, Alvaro

    2016-01-01

    Introducción: la difalia es una alteración anatómica poco frecuente que se presenta en uno de cada cinco millones de nacimientos. Su etiología se desconoce y su apariencia varía desde un pene accesorio pequeño hasta la duplicación completa y se asocia con otras malformaciones congénitas urogenitales, gastrointestinales, cardiacas y musculoesqueléticas. Se han diseñado varias clasificaciones de acuerdo con las características anatómicas y el estudio de esta alteración se complementa con ecografía e imagen de resonancia magnética. El tratamiento debe ser temprano y se individualiza con el objetivo de lograr un resultado estético y funcional que sea satisfactorio. Caso clínico: reportamos el hallazgo incidental de difalia, sin otras malformaciones anatómicas asociadas, en un adulto de 83 años que fue hospitalizado y falleció por traumatismo craneoencefálico severo. Conclusión: el caso que presentamos es relevante por lo poco frecuente de esta alteración, la ausencia de otras malformaciones anatómicas asociadas y la edad a la que se detectó.

  17. [Use of intravenous immunoglobulin in pregnancy. Report of a patient with common variable immunodeficiency].

    PubMed

    Cambray-Gutiérrez, Julio César; García-Ramírez, Ulises Noel; Del Rivero-Hernández, Leonel Gerardo; López-Pérez, Patricia; Chávez-García, Aurora

    2016-01-01

    Antecedentes: La inmunodeficiencia común variable es la inmunodeficiencia primaria más diagnosticada en los adultos; se caracteriza por infecciones sinopulmonares y gastrointestinales de repetición y mayor incidencia de procesos autoinmunes y malignidad. Numerosos pacientes inician con las manifestaciones clínicas durante la edad reproductiva. Caso clínico: Mujer de 34 años de edad con 12 semanas de gestación, en quien se diagnosticó inmunodeficiencia común variable después de cuadros recurrentes de rinosinusitis, faringoadmidalitis y neumonías. Durante el segundo trimestre se prescribió 0.6 g/kg de inmunoglobulina intravenosa cada 21 días; la paciente solo presentó un episodio de faringoamigdalitis, con adecuada respuesta al tratamiento con antibióticos. Durante el tercer trimestre se ajustó la dosis a cada 14 días. La paciente concluyó el embarazo a término sin complicaciones, con producto sin malformaciones y con peso y talla adecuados. Conclusiones: La administración de inmunoglobulina es el principal tratamiento para controlar la inmunodeficiencia común variable. Si bien la dosis inicial recomendada es de 400-800 mg/kg en forma intravenosa cada 3 a 4 semanas, no existe un consenso sobre la dosis que debe emplearse en la mujer que cursa con embarazo. La recomendación es realizar controles de niveles séricos antes de la infusión para determinarla y ajustarla.

  18. [Hematopoietic stem cells transplant in patients with common variable immunodeficiency. Is a therapeutic option?

    PubMed

    Cambray-Gutiérrez, Julio César; Herrera-Sánchez, Diana Andrea; López-Pérez, Patricia; Chávez-García, Aurora; Yamazaki-Nakashimada, Marco Antonio

    2017-01-01

    Antecedentes: Los pacientes con inmunodeficiencia común variable presentan mayor incidencia de infecciones sinopulmonares y gastrointestinales, así como de enfermedades linfoproliferativas y autoinmunes. El tratamiento de elección es el reemplazo con gammaglobulina humana. El trasplante de células progenitoras hematopoyéticas es una modalidad terapéutica no convencional. Caso clínico: Mujer de 26 años de edad sin antecedentes heredofamiliares de inmunodeficiencias primarias ni consanguinidad, con procesos repetidos de otitis, sinusitis, gastroenteritis y bronquitis desde la infancia. En la adolescencia fue diagnosticada con inmunodeficiencia común variable; se le prescribió gammaglobulina intravenosa, antimicrobianos de amplio espectro y macrólidos. A los 22 años se le realizó trasplante de células progenitoras hematopoyéticas por continuar con infecciones severas. A los 4 meses del trasplante se le diagnosticó hipotiroidismo e insuficiencia ovárica. Durante los siguientes 3 años no presentó infecciones, pero a los 25 años manifestó púrpura trombocitopénica inmune, que persistía al momento de este informe con enfermedad de Raynaud e infecciones reincidentes de vías respiratorias altas. Es tratada con gammaglobulina intravenosa y profilaxis con claritromicina, sin esteroides ni danazol. Conclusiones: Dada la alta tasa de morbimortalidad asociada y falla en la reconstitución inmunológica, el trasplante de células progenitoras hematopoyéticas deberá ser cuidadosamente evaluado en pacientes con infecciones sin respuesta al tratamiento o con enfermedades linfoproliferativas.

  19. Effectiveness and safety of pemetrexed for non-small cell lung cancer in the Andalusian Public Health System.

    PubMed

    Pérez-Moreno, María Antonia; Cotrina-Luque, Jesús; Galván-Banqueri, Mercedes; Flores-Moreno, Sandra; Bautista-Paloma, Francisco Javier; Calleja-Hernández, Miguel Ángel

    2016-11-01

    Objetivo: Evaluar la efectividad y el perfil de seguridad del pemetrexed en pacientes con cáncer de pulmón no microcítico (CPNM) localmente avanzado o metastásico en la práctica clínica real en Andalucía (una región española con 8,5 millones de habitantes según los datos del censo de 2014). Métodos: Se realizó un estudio retrospectivo multicéntrico observacional, incluyendo aquellos pacientes adultos con CPNM localmente avanzado/metastásico que hubiesen recibido pemetrexed en cualquier hospital del Sistema Sanitario Público de Andalucía durante el último trimestre de 2011. Se revisaron las características basales de los pacientes, los datos relativos al diagnóstico y al tratamiento, las variables de efectividad (en términos de respuesta al tratamiento con pemetrexed y supervivencia global) y las principales reacciones adversas detectadas. Resultados: Se incluyeron un total de 172 pacientes procedentes de 17 hospitales (77,33% hombres), con una mediana de edad de 63 años (rango: 34 y 83). La histología predominante fue el adenocarcinoma (84,30%) y el 85,20% fueron diagnosticados de cáncer de pulmón en estadio IV. El 78,49% habían sido fumadores en algún momento de sus vidas. La mediana de supervivencia global desde el inicio del pemetrexed fue de 9 meses (IC del 95%, 4,1-13,9). La progresión de la enfermedad fue la respuesta al tratamiento más frecuente (33,14%) y solo un paciente tuvo una respuesta completa. La presencia de enfermedad estable se asoció con una mayor probabilidad de supervivencia. Las principales reacciones adversas detectadas fueron astenia; reacciones hematológicas, gastrointestinales y dermatológicas o toxicidad mucosa. Ninguno de los pacientes interrumpió el tratamiento por toxicidad grave. Conclusiones: El pemetrexed resultó bastante efectivo en el CPNM cuando fue utilizado en la práctica clínica real, con una mayor supervivencia en histología no escamosa y en los pacientes con mejor puntuación en la escala

  20. MIBG scintiscan

    MedlinePlus

    Adrenal medullary imaging; Meta-iodobenzylguanidine scintiscan; Pheochromocytoma - MIBG; Neuroblastoma - MIBG; Carcinoid MIBG ... answer. It is also used to help diagnose neuroblastoma and can be used for carcinoid tumors.

  1. Rectal Carcinoid Tumor With Liver Metastases Treated by Local Excision and Orthotopic Liver Transplant With Long-term Follow-up.

    PubMed

    Vennarecci, Giovanni; Mascianà, Gianluca; de Werra, Edoardo; Guglielmo, Nicola; Levi Sandri, Giovanni Battista; Coluzzi, Mariagrazia; Ettorre, Giuseppe Maria

    2017-03-28

    In patients affected by unresectable liver metastases from neuroendocrine tumor, liver transplant represents currently the only realistic chance for cure. The first attempt to establish selection criteria for liver transplant in patients affected by neuroendocrine tumor liver metastases was made by Mazzaferro and associates in 2007. We report the case of a 46-year-old man who came to our institution in 2006 with right upper quadrant abdominal pain. Diagnosis of rectal neuroendocrine tumor with bilobar liver nodules was made; the patient underwent transanal local resection. A liver biopsy confirmed the metastatic nature of the hepatic lesion, showing a low-grade neuroendocrine tumor (G1, proliferation index Ki-67 <2%). The patient underwent 2 sessions of transarterial chemoembolization that resulted in stable disease. Afterward, the patient underwent a liver transplant, using the piggyback technique without a venous-venous bypass. His postoperative course was uneventful. The patient has been disease-free for 3 years. Posttransplant treatment has played a key role in increasing the overall survival of the patient and assuring him a good quality of life. He died 9 years (102 mo) after liver transplant.

  2. [GASTROSTOMY POSITIVELY AFFECTS NUTRITIONAL STATUS AND DIMINISHES HOSPITAL DAYS IN PATIENTS WITH INBORN ERRORS OF METABOLISM].

    PubMed

    Guillén-López, Sara; Vela-Amieva, Marcela; Juárez-Cruz, Merit Valeria; González-Zamora, José Francisco; Monroy-Santoyo, Susana; Belmont-Martínez, Leticia

    2015-07-01

    Introducción: el tratamiento nutricional de los pacientes con errores innatos del metabolismo (EIM) implica el uso permanente de fórmulas modificadas en aminoácidos cuyas características organolépticas pueden dificultar su aceptación por vía oral. Estos pacientes pueden tener alteraciones gastrointestinales y requieren el uso constante de medicamentos, lo cual complica la adherencia al tratamiento, comprometiéndose con ello su estado nutricional y el control de la enfermedad. La gastrostomía es una alternativa para facilitar la alimentación y el tratamiento, pero existen controversias sobre su uso. Objetivo: comparar el estado nutricional y la duración de las hospitalizaciones antes y después de la realización de la gastrostomía en un grupo de pacientes con EIM. Métodos: análisis retrospectivo de datos antropométricos, número de internamientos por descompensación metabólica y su duración en pacientes pediátricos con EIM antes y después de la gastrostomía. Resultados: se analizaron 16 niños; 40% con defectos del propionato, 25% con alteraciones del ciclo de la urea y 35% con otros EIM. Después de la gastrostomía, la proporción de pacientes eutróficos aumentó del 6 al 56% y la desnutrición disminuyó del 94 al 44%. Después de la gastrostomía, la duración de los periodos hospitalarios disminuyó significativamente de 425 a 131 días (p = 0.011); el número de internamientos disminuyó de 33 antes de la intervención a 17, sin embargo, esta diferencia no tuvo significación estadística. Conclusión: en esta muestra, la gastrostomía mejoró el estado nutricional en 56% de los pacientes con EIM, y redujo significativamente los días de hospitalización por descompensación metabólica.

  3. [Simplified laparoscopic gastric bypass. Initial experience].

    PubMed

    Hernández-Miguelena, Luis; Maldonado-Vázquez, Angélica; Cortes-Romano, Pablo; Ríos-Cruz, Daniel; Marín-Domínguez, Raúl; Castillo-González, Armando

    2014-01-01

    Antecedentes: la cirugía de la obesidad comprende diversos procedimientos gastrointestinales. El bypass gástrico en Y de Roux es el prototipo de los procedimientos mixtos y el más practicado en el mundo en sus diversas variedades. Una técnica similar y novedosa es la adoptada por Cardoso-Ramos y Galvao denominada "bypass simplificado" que rápidamente se aceptó por la mayor facilidad y resultados muy parecidos a la técnica convencional. Objetivo: describir los resultados a un año del bypass gástrico simplificado para el tratamiento de la obesidad mórbida. Material y métodos: estudio retrospectivo y descriptivo de todos los pacientes a quienes se realizó bypass gástrico de enero de 2008 a julio de 2012, en la clínica de obesidad de un hospital privado de la Ciudad de México. Resultados: se estudiaron 90 pacientes con diagnóstico de obesidad mórbida, con límites de edad de 18 y 65 años, operados para bypass gástrico simplificado. En 10% de los pacientes hubo complicaciones, las más frecuentes fueron: hemorragia y hernia interna. Durante el periodo de estudio la mortalidad fue de 0%. La pérdida de peso promedio a los 12 meses fue de 72.7%. Conclusión: el bypass gástrico simplificado laparoscópico es una cirugía segura, con buenos resultados a mediano plazo, y con una pérdida del exceso de peso adecuada en 71% de los casos.

  4. Tratamiento canonico del problema de Poincaré. Movimiento del polo.

    NASA Astrophysics Data System (ADS)

    Romero, P.; Sevilla, M. J.

    The rotational motion for a rigid Earth model with a homogeneous liquid core has been obtained using Hamilton's equations. From the canonical equations for the precessional und nutational motions in an inertial frame, the corresponding equations in an Earth fixed frame are deduced. The linearized equations obtained for polar motion and liquid core motion are equivalent to the Moritz's equations (1980).

  5. Tratamiento formal de imágenes astronómicas con PSF espacialmente variable

    NASA Astrophysics Data System (ADS)

    Sánchez, B. O.; Domínguez, M. J.; Lares, M.

    2017-10-01

    We present a python implementation of a method for PSF determination in the context of optimal subtraction of astronomical images. We introduce an expansion of the spatially variant point spread function (PSF) in terms of the Karhunen Loève basis. The advantage of this approach is that the basis is able to naturally adapt to the data, instead of imposing a fixed ad-hoc analytic form. Simulated image reconstruction was analyzed, by using the measured PSF, with good agreement in terms of sky background level between the reconstructed and original images. The technique is simple enough to be implemented on more sophisticated image subtraction methods, since it improves its results without extra computational cost in a spatially variant PSF environment.

  6. Tratamiento de mostos utilizando el filtro anaerobio (treatment of must using an anaerobic filter). Master thesis

    SciTech Connect

    Negron, R.M.

    1982-12-01

    This study reports on the feasibility of treating the slops from rum distilleries with an anaerobic filter composed of an oxygen free reactor filled with a plastic filtering media, commercially known as 'Actifil' with a diameter of 5/8 inches (16mm). This filter is similar in appearance to an aerobic biofilter (trickling filter). The main difference between the two systems is that on the anaerobic filter the flow is upward so that the filter medium is always completely submerged.

  7. Medicinal ethnobotany in Huacareta (Chuquisaca, Bolivia)

    PubMed Central

    2012-01-01

    used as a base for subsequent work related to traditional medicine and its contribution to allopathic medicine in San Pablo de Huacareta. Resumen Introducción El objetivo del presente estudio fue documentar los tipos de enfermedades tratadas mediante el uso de plantas medicinales, sus aplicaciones principales y también tener un reporte de las enfermedades mayormente atendidas en el Hospital de San Pablo de Huacareta (Chuquisaca, Bolivia). Métodos Se realizaron encuestas semiestructuradas a 10 informantes locales anotando los usos atribuidos a sus plantas medicinales, se agruparon las plantas por categorías de enfermedades tratadas en la medicina tradicional. Se obtuvieron reportes de casos tratados en el Hospital de Huacareta para poder relacionar el tratamiento de enfermedades recurrentes en la zona entre la medicina tradicional y la medicina occidental. Resultados Se reportaron 91 especies nativas y exóticas, además de un espécimen indeterminado exótico que intervienen en un total de 258 aplicaciones medicinales, las cuales son empleadas en un total de 13 categorías de enfermedades. Los desórdenes gastrointestinales (55%) son mayormente tratados mediante plantas medicinales, seguidas de las afecciones al sistema esqueleto-muscular (25%) y enfermedades dermatológicas (24%). La información del Hospital indica que las enfermedades más frecuentes son Infecciones Respiratorias Agudas (47%) y Enfermedades Diarreicas Agudas (37%). Los remedios vegetales se emplean en forma de infusiones y cocciones principalmente. Se emplean mayormente plantas nativas, también se introdujo en la farmacopea médica el uso de plantas exóticas al lugar. Conclusiones El tratamiento de trastornos gastrointestinales constituye el objetivo primordial de la etnobotánica médica de los habitantes de Huacareta, las enfermedades del sistema respiratorio, son mayormente tratadas en el Hospital. Observando los datos del libro de consultas del Hospital, se puede inferir que los des

  8. Endoscopic Placement of Metal Stents in Treating Patients With Cancer- Related Duodenal Obstruction

    ClinicalTrials.gov

    2012-05-31

    Colorectal Cancer; Constipation, Impaction, and Bowel Obstruction; Extrahepatic Bile Duct Cancer; Gastric Cancer; Gastrointestinal Carcinoid Tumor; Gastrointestinal Stromal Tumor; Pancreatic Cancer; Quality of Life; Small Intestine Cancer

  9. Oxaliplatin Plus Irinotecan in Treating Patients With Metastatic Gastrointestinal Cancer

    ClinicalTrials.gov

    2013-06-24

    Anal Cancer; Colorectal Cancer; Esophageal Cancer; Extrahepatic Bile Duct Cancer; Gallbladder Cancer; Gastric Cancer; Gastrointestinal Carcinoid Tumor; Liver Cancer; Pancreatic Cancer; Small Intestine Cancer

  10. Autonomic Dysreflexia-Like Syndrome in a T12 Paraplegic During Thoracic Spine Surgery

    DTIC Science & Technology

    2010-11-01

    pheochromocytoma , carcinoid syndrome, or thyroid storm. This presentation differs from autonomic dysreflexia because the spinal cord lesion was well below T6...instability. The differential diagnosis of isolated, abrupt intraopera- tive hypertension includes pheochromocytoma , carcinoid syndrome, thyroid storm...pressure, even after cessation of surgical stimulation. This suggests an underlying, per- sistent physiologic cause for the sustained and resistant

  11. Diffuse Neuroendocrine Cell Hyperplasia: Report of Two Cases

    PubMed Central

    Cansız Ersöz, Cevriye; Cangır, Ayten Kayı; Dizbay Sak, Serpil

    2016-01-01

    Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterised by a proliferation of neuroendocrine cells within the lung. It is believed that a minority of the patients with DIPNECH can develop carcinoid tumors. Here, we report two new cases of DIPNECH with coexisting carcinoid tumors. PMID:27293939

  12. California Cancer Registry Enhancement for Breast Cancer Research.

    DTIC Science & Technology

    1997-10-01

    QUERY SYSTEM March 6, 1997 FEMALE BREAST CANCER STAGE GROUPS Site = C500-509 (740-749); Sex = 2 Histology = 8000-8231, 8244-8573 (excludes carcinoids ... carcinoids , sarcomas, lymphomas) Dxdate > = 1995 74 DEFINITION PDQ CRITERIA FOR Group TNM = 4th Edition CONDITIONS APPROPRIATE RX 12 STAGE III A

  13. Hacia una adaptación cultural para el tratamiento de trastornos alimentarios en latinos en Estados Unidos

    PubMed Central

    Reyes-Rodríguez, Mae Lynn; Bulik, Cynthia M.

    2011-01-01

    Eating disorders affect all ethnic and socioeconomic groups. However, evidence based treatments for eating disorders have been developed and tested exclusively on Caucasian populations. With the purpose to develop a culturally sensitive framework for the eating disorders treatment in Latinos/as, the objectives of this work were: 1) identify and describe some of the relevant cultural elements for eating disorders in the Latino population and, 2) to draft a culturally sensitive intervention model for eating disorders in Latino population in the United States. Providing culturally sensitive treatments for Latinos with psychiatric disorders is essential to reverse public health disparities. PMID:22003472

  14. Innovative manure treatments in the USA – state of the art (Tratamientos Innovadores de estiercoles en USA - estado del arte)

    USDA-ARS?s Scientific Manuscript database

    Currently, the potential impact of manure on the environment represents one of the world agriculture’s major challenges. Treatment technologies can play an important role in the management of livestock manure by providing a more flexible approach to land application and acreage limitations and by so...

  15. Hacia una adaptación cultural para el tratamiento de trastornos alimentarios en latinos en Estados Unidos.

    PubMed

    Reyes-Rodríguez, Mae Lynn; Bulik, Cynthia M

    2010-01-01

    Eating disorders affect all ethnic and socioeconomic groups. However, evidence based treatments for eating disorders have been developed and tested exclusively on Caucasian populations. With the purpose to develop a culturally sensitive framework for the eating disorders treatment in Latinos/as, the objectives of this work were: 1) identify and describe some of the relevant cultural elements for eating disorders in the Latino population and, 2) to draft a culturally sensitive intervention model for eating disorders in Latino population in the United States. Providing culturally sensitive treatments for Latinos with psychiatric disorders is essential to reverse public health disparities.

  16. A Medical Center Network for Optimized Lung Cancer Biospecimen Banking

    DTIC Science & Technology

    2013-10-01

    None S0054 Carcinoid Tumor: Typical Stage IB Y N .143 100 99 0 1 4 .128 1 4 .23 1 No - Quit Smoking 10 None None None S0055 Adenocarcinoma: Mucinous...None None S0145 Carcinoid Tumor: Typical Stage IA Y N 0.011 100 100 0 0.141 No - Quit Smoking 20 None None None S0146 Adenocarcinoma Stage Y N 0.587...Smoking 0 None None None S0154 Adenocarcinoma Stage IA Y N 0.201 45 95 0 0.192 No - Quit Smoking 8 None None None S0155 Carcinoid Tumor: Typical

  17. Gastroscopic follow up of pernicious anaemia patients.

    PubMed Central

    Sjöblom, S M; Sipponen, P; Järvinen, H

    1993-01-01

    To assess the value of gastroscopic cancer surveillance of patients with pernicious anaemia, 56 patients were re-endoscoped and biopsied after three years. In addition, changes in the density of fundic mucosal endocrine cells were evaluated morphometrically. Two cases (3.6%) of early gastric cancer and two cases of small gastric carcinoid tumours (3.6%) were detected in addition to the five carcinoids that had been found at the initial endoscopic screening. Nodular argyrophil cell hyperplasia and morphometric density of argyrophil cells were not stable phenomena: nodular hyperplasias regressed in five patients, remained similar in six, and progressed to a small carcinoid tumour in one. Serum gastrin concentrations did not correlate well with changes in the endocrine cell density. Regular endoscopic surveillance for gastric cancer may be beneficial and realistic in young patients with pernicious anaemia while the importance of fundic endocrine cell hyperplasia and that of small gastric carcinoids need further study. PMID:8432447

  18. Telotristat

    MedlinePlus

    ... by carcinoid tumors (slow-growing tumors that release natural substances that can cause symptoms like diarrhea) in ... works by blocking the formation of a certain natural substance in the body that is released by ...

  19. [Endocrine tumors of the gastrointestinal and pancreatic systems. Multiple endocrine adenoma from another viewpoint].

    PubMed

    Klempa, I; Helmstädter, V; Feurle, G; Röttger, P

    1980-05-01

    The 24 endocrine pancreatic tumors and 14 carcinoids were examined immunohistochemically for cholecystokinin, insulin, gastrin, GIP, glucagon, sercretin, VIP, motilin, neurotensin, pancreatic polypeptide (PP), somatostatin, and ACTH. In 12 tumors of the pancreas more than one peptide-containing cell type was observed. The clinical symptoms showed hypersecretion of only one of the hormones, however. The midgut carcinoids (jejunum, appendix) represented the classical view of the carcinoid as an argentaffin cell tumor secreting 5-hydroxytryptamine. Tumors originating in the foregut (bronchus, stomach, duodenum) and hindgut carcinoids (rectum) were nonargentaffine, containing and secreting various polypeptide hormones. We conclude that light microscopic immunohistochemical methods are useful in distinguishing endocrine from nonendocrine tumors and multihormonal syndromes (MEA) in the classification of predominant hormone-secreting tumors.

  20. Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer

    ClinicalTrials.gov

    2014-07-01

    Brain and Central Nervous System Tumors; Childhood Langerhans Cell Histiocytosis; Gastrointestinal Carcinoid Tumor; Head and Neck Cancer; Intraocular Melanoma; Islet Cell Tumor; Kidney Cancer; Lung Cancer; Melanoma (Skin); Neoplastic Syndrome; Neuroendocrine Carcinoma of the Skin; Pheochromocytoma

  1. Irinotecan, Fluorouracil, and Leucovorin in Treating Patients With Advanced Gastrointestinal Cancer

    ClinicalTrials.gov

    2016-04-19

    Anal Cancer; Carcinoma of the Appendix; Colorectal Cancer; Esophageal Cancer; Extrahepatic Bile Duct Cancer; Gallbladder Cancer; Gastric Cancer; Gastrointestinal Carcinoid Tumor; Gastrointestinal Stromal Tumor; Liver Cancer; Pancreatic Cancer; Small Intestine Cancer

  2. A Case of Paraneoplastic Cushing Syndrome Presenting as Hyperglycemic Hyperosmolar Nonketotic Syndrome.

    PubMed

    Brzezniak, Christina E; Vietor, Nicole; Hogan, Patricia E; Oronsky, Bryan; Thilagar, Bennett; Ray, Carolyn M; Caroen, Scott; Lybeck, Michelle; Oronsky, Neil; Carter, Corey A

    2017-01-01

    Carcinoid tumors are neuroendocrine tumors that mainly arise in the gastrointestinal tract, lungs, and bronchi. Bronchopulmonary carcinoids have been associated with Cushing syndrome, which results from ectopic adrenocorticotrophic hormone (ACTH) secretion. We report the case of a 65-year-old man, a colonel in the US Air Force, with metastatic bronchopulmonary carcinoid tumors treated on a clinical trial who was hospitalized for complaints of increasing thirst, polydipsia, polyuria, weakness, and visual changes. Decompensated hyperglycemia suggested a diagnosis of hyperglycemic hyperosmolar nonketotic syndrome (HHNS). Additional findings, which included hypokalemia, hypernatremia, hypertension, metabolic alkalosis, moon facies, and striae, raised a red flag for an ectopic ACTH syndrome. Elevated ACTH levels confirmed Cushing syndrome. Treatment with a fluid replacement and insulin drip resulted in immediate symptomatic improvement. Cushing syndrome should be considered in carcinoid patients with physical stigmata such as moon facies and striae. HHNS may be the presenting clinical feature in patients with impaired glucose metabolism.

  3. A rectal neuroendocrine neoplasm.

    PubMed

    Varas Lorenzo, Modesto J; Muñoz Agel, Fernando

    2017-08-01

    The incidence of gastric and rectal carcinoids is increasing. This is probably due to endoscopic screening. The prognosis is primarily dependent upon tumor size, aggressiveness (pathology, Ki-67), metastatic disease and stage. However, neuroendocrine carcinoma usually behaves as an adenocarcinoma.

  4. Palonosetron Hydrochloride in Preventing Nausea and Vomiting Caused by Radiation Therapy in Patients With Primary Abdominal Cancer

    ClinicalTrials.gov

    2016-12-07

    Anal Cancer; Carcinoma of the Appendix; Colorectal Cancer; Extrahepatic Bile Duct Cancer; Gallbladder Cancer; Gastric Cancer; Gastrointestinal Carcinoid Tumor; Liver Cancer; Nausea and Vomiting; Pancreatic Cancer; Primary Peritoneal Cavity Cancer; Small Intestine Cancer

  5. Octreotide Injection

    MedlinePlus

    ... hormone (a natural substance) produced by people with acromegaly (condition in which the body produces too much ... Octreotide long-acting injection is used to control acromegaly, carcinoid tumors, and VIP-omas in people who ...

  6. Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors

    ClinicalTrials.gov

    2017-03-21

    Gastrinoma; Glucagonoma; Insulinoma; Metastatic Gastrointestinal Carcinoid Tumor; Metastatic Pheochromocytoma; Pancreatic Polypeptide Tumor; Recurrent Gastrointestinal Carcinoid Tumor; Recurrent Islet Cell Carcinoma; Recurrent Melanoma; Recurrent Neuroendocrine Carcinoma of the Skin; Recurrent Non-small Cell Lung Cancer; Recurrent Pheochromocytoma; Recurrent Renal Cell Cancer; Somatostatinoma; Stage III Neuroendocrine Carcinoma of the Skin; Stage IV Melanoma; Stage IV Non-small Cell Lung Cancer; Stage IV Renal Cell Cancer; Thyroid Gland Medullary Carcinoma; Unspecified Adult Solid Tumor, Protocol Specific

  7. Divergent cyclin B1 expression and Rb/p16/cyclin D1 pathway aberrations among pulmonary neuroendocrine tumors.

    PubMed

    Igarashi, Toru; Jiang, Shi-Xu; Kameya, Toru; Asamura, Hisao; Sato, Yuichi; Nagai, Kanji; Okayasu, Isao

    2004-10-01

    A total of 111 pulmonary neuroendocrine tumors comprising 13 typical carcinoids, five atypical carcinoids, 44 large-cell neuroendocrine carcinomas and 49 small-cell carcinomas were immunohistochemically studied for dysregulated cyclin B1 expression and disruption of the Rb/p16/cyclin D1 pathway (Rb pathway), and the results were correlated with tumor proliferation activity and clinical outcome. Overexpression of cyclins B1 and D1, respectively, was detected in no and 15% typical carcinoids, 20 and 20% atypical carcinoids, 84 and 32% large-cell neuroendocrine carcinomas, 84 and 10% small-cell carcinomas. Loss of Rb and p16 expression, respectively, was observed in no and 14% typical carcinoids, no and 40% atypical carcinoids, 49 and 18% large-cell neuroendocrine carcinomas, 84 and 8% small-cell carcinomas. In summary, 29% typical carcinoids, 20% atypical carcinoids, 78% large-cell neuroendocrine carcinomas and 93% small-cell carcinomas had Rb pathway aberrations. Rb pathway aberration was mostly attributed to Rb loss in small-cell carcinomas, while p16 loss and/or cyclin D1 overexpression besides Rb loss also played an important role in large-cell neuroendocrine carcinomas, while cyclin D1 overexpression was the only cause of Rb pathway aberration in carcinoid tumors. Thus, both cyclin B1-associated G2/M arrest and Rb-mediated G1 arrest are consistently compromised in high-grade large-cell neuroendocrine carcinoma and small-cell carcinoma, but are generally intact or occasionally altered in carcinoid tumor; the mechanisms involved in Rb pathway aberration among the tumor categories are different, reflecting a genetic divergence among the individual tumor categories. Cyclin B1 expression closely correlated with the Ki-67 labeling index either in the individual tumor categories or overall tumors (P < 0.0001, r = 0.742), suggesting that cyclin B1 is one of the key factors regulating cell proliferation in pulmonary neuroendocrine tumors. Neither cyclins B1 and D1, Rb, p

  8. Los pronombres de cortesia: su tratamiento en espanol y en otros idiomas, El adverbio (Pronouns of Courtesy: Their Treatment in Spanish and Other Languages, the Adverbs)

    ERIC Educational Resources Information Center

    Criado de Val, Manuel

    1973-01-01

    Compares use of pronouns and adverbs in Spanish, French, Italian, Portuguese, English, German, Rumanian, and Slavic languages. Excerpted from the book Fisonomia del y de las lenguas modernas'' ( Features of Modern Languages''). (DS)

  9. Los pronombres de cortesia: su tratamiento en espanol y en otros idiomas, El adverbio (Pronouns of Courtesy: Their Treatment in Spanish and Other Languages, the Adverbs)

    ERIC Educational Resources Information Center

    Criado de Val, Manuel

    1973-01-01

    Compares use of pronouns and adverbs in Spanish, French, Italian, Portuguese, English, German, Rumanian, and Slavic languages. Excerpted from the book Fisonomia del y de las lenguas modernas'' ( Features of Modern Languages''). (DS)

  10. Testing road surface treatments to reduce erosion in forest roads in Honduras [Tratamientos de la superficie de rodadura para reducir la erosion en caminos forestales en Honduras

    USGS Publications Warehouse

    Rivera, Samuel; Kershner, Jeffrey L.; Keller, Gordon R.

    2009-01-01

    Testing road surface treatments to reduce erosion in forest roads in Honduras. Cien. Inv. Agr. 36(3):425-432. Using forest roads produces more erosion and sedimentation than any other forest or agricultural activity. This study evaluated soil losses from a forest road in central Honduras over two consecutive years. We divided a 400-m segment of road into 8 experimental units, each 50 m in length. Four units were treated with Best Management Practices (BMPs) and four were left untreated. The BMP treatments included reshaping the road prism, installing culverts and reshaping of road ditches, compacting 20-cm layers of the road tread, crowning the road surface (3% slope, double drainage), longitudinal sloping (less than 12%), and adding a 10-cm layer of gravel (crush size = 0.63 cm). Soil movement was measured daily during the rainy seasons. The highest soil loss occurred in the control road, around 500 m3 km-1 per year, while the road treated with BMP lost approximately 225 m3km-1 per year. These results show that road surface erosion can be reduced up to 50% with the implementation of surface treatments.

  11. Somatostatin receptor subtypes, octreotide scintigraphy, and clinical response to octreotide treatment in patients with neuroendocrine tumors.

    PubMed

    Kölby, L; Wängberg, B; Ahlman, H; Tisell, L E; Fjälling, M; Forssell-Aronsson, E; Nilsson, O

    1998-07-01

    Several types of neuroendocrine tumor express high numbers of somatostatin receptors (sstr). We have compared the expression of sstr subtypes with the outcome of octreotide scintigraphy in patients with carcinoids and medullary thyroid carcinoma (MTC) in comparison with Hürthle cell tumors. The effect of sstr activation (octreotide treatment) on tumor markers was also studied in patients with disseminated carcinoid tumors. Six patients with carcinoid tumors (four midgut and two foregut), and three patients with thyroid tumors (one MTC, one Hürthle cell carcinoma, and one Hürthle cell adenoma) were studied. Octreotide scintigraphy visualized tumor sites in all nine patients. Macroscopic tumor was verified at these sites at subsequent surgical exploration. Using Northern blotting and subtype-specific riboprobes, sstr could be detected in all tumors examined. All five sstr subtypes were detected in most of the carcinoid tumors. All six carcinoids expressed sstr2. This was in contrast to the findings for the thyroid tumors analyzed, which also expressed several sstr subtypes but in some cases lacked expression of sstr2. This was also the case for normal thyroid tissue. Clinically, octreotide treatment of the patients with midgut carcinoid tumors resulted in palliation of hormonal symptoms accompanied by a significant reduction of urinary 5-HIAA levels (28-71%). These results indicate that carcinoid tumors frequently express all five sstr subtypes. The thyroid tumors also expressed multiple sstr but could lack expression of sstr2. Nevertheless, these tumors were visualized by octreotide scintigraphy, indicating that sstr2 expression is not a prerequisite for tumor imaging.

  12. Gastric neuroendocrine tumors: prevalence in Europe, USA, and Japan, and rationale for treatment with a gastrin/CCK2 receptor antagonist.

    PubMed

    Boyce, Malcolm; Thomsen, Liv

    2015-05-01

    Gastric carcinoids (neuroendocrine tumors) arise from enterochromaffin-like cells in the gastric mucosa. Most are caused by hypergastrinemia. The objectives were to determine if their prevalence in Europe, USA and Japan meets the criteria for an orphan disease and to justify treatment with a gastrin/CCK2 receptor antagonist. We obtained data from European and USA cancer registries, and searched PubMed. Prevalence per 10,000 population obtained from cancer registries was: median 0.32 (range 0.09-0.92) for Europe; and 0.17 for the USA, equivalent to 4812 for the whole population. A PubMed search for gastric carcinoids yielded prevalence for Japan only, which was 0.05 per 10,000 population, equivalent to 665 for the entire population. A further search for gastric carcinoids in patients with pernicious anemia (PA) or autoimmune chronic atrophic gastritis (CAG), two presentations of about 80% of gastric carcinoids, produced prevalence rates of 5.2-11%. Prevalence of PA itself was 0.12-1.9%. Data on CAG epidemiology were sparse. Prevalence of gastric carcinoids varied widely. All sources probably underestimate prevalence. However, prevalence was below the limits required for recognition by drug regulatory authorities as an orphan disease: 5 per 10,000 population of Europe; 200,000 for the whole population of the USA; and 50,000 for the whole population of Japan. Because gastric carcinoids are an orphan disease, and nonclinical and healthy volunteer studies support treatment with netazepide, a gastrin/CCK2 antagonist, netazepide has been designated an orphan medicinal product in Europe and the USA for development as targeted treatment for gastric carcinoids.

  13. Massive parallel sequencing and digital gene expression analysis reveals potential mechanisms to overcome therapy resistance in pulmonary neuroendocrine tumors.

    PubMed

    Walter, Robert Fred Henry; Vollbrecht, Claudia; Christoph, Daniel; Werner, Robert; Schmeller, Jan; Flom, Elena; Trakada, Georgia; Rapti, Aggeliki; Adamidis, Vasilis; Hohenforst-Schmidt, Wolfgang; Kollmeier, Jens; Mairinger, Thomas; Wohlschlaeger, Jeremias; Zarogoulidis, Paul; Porpodis, Konstantinos; Schmidt, Kurt Werner; Mairinger, Fabian Dominik

    2016-01-01

    Background: Lung cancer is the leading cause of cancer-related deaths worldwide. 25% show neuroendocrine differentiation (typical/atypical carcinoids, large-/small-cell neuroendocrine carcinomas). Carcinoids present with long survival rates, but metastatic carcinoids correlate with decreased survival and are commonly insensitive to standard chemotherapy or radiation. Therefore, novel therapeutic strategies are urgently needed. Material and methods: 70 representative tumor specimens were used for next-generation sequencing analysis of 14 genes related to therapy response. Additionally, mRNA-expression profiles of 60 matching samples were determined for 13 selected drug targets by using the NanoString nCounter technology. Results: A number of features known to sensitize tumors for different targeted therapies could be identified, which hopefully improve the clinical management of this subgroup of lung neoplasias. In particular, EGFR expression was observed in the investigated tumors in a noteworthy manner. Additionally, MDM2 was strongly expressed in the majority of all samples whereas the expression of its physiological inhibitor, CDKN2A, was nearly absent in all low-grade tumors. TP53 showed a high frequency of variants in high-grade tumors but mutations were rare in carcinoids. Conclusion: Based on our results, therapeutic approaches with MDM2-inhibitors and monoclonal anti-EGFR antibodies may be promising in pulmonary carcinoid tumors.

  14. Massive parallel sequencing and digital gene expression analysis reveals potential mechanisms to overcome therapy resistance in pulmonary neuroendocrine tumors

    PubMed Central

    Walter, Robert Fred Henry; Vollbrecht, Claudia; Christoph, Daniel; Werner, Robert; Schmeller, Jan; Flom, Elena; Trakada, Georgia; Rapti, Aggeliki; Adamidis, Vasilis; Hohenforst-Schmidt, Wolfgang; Kollmeier, Jens; Mairinger, Thomas; Wohlschlaeger, Jeremias; Zarogoulidis, Paul; Porpodis, Konstantinos; Schmidt, Kurt Werner; Mairinger, Fabian Dominik

    2016-01-01

    Background: Lung cancer is the leading cause of cancer-related deaths worldwide. 25% show neuroendocrine differentiation (typical/atypical carcinoids, large-/small-cell neuroendocrine carcinomas). Carcinoids present with long survival rates, but metastatic carcinoids correlate with decreased survival and are commonly insensitive to standard chemotherapy or radiation. Therefore, novel therapeutic strategies are urgently needed. Material and methods: 70 representative tumor specimens were used for next-generation sequencing analysis of 14 genes related to therapy response. Additionally, mRNA-expression profiles of 60 matching samples were determined for 13 selected drug targets by using the NanoString nCounter technology. Results: A number of features known to sensitize tumors for different targeted therapies could be identified, which hopefully improve the clinical management of this subgroup of lung neoplasias. In particular, EGFR expression was observed in the investigated tumors in a noteworthy manner. Additionally, MDM2 was strongly expressed in the majority of all samples whereas the expression of its physiological inhibitor, CDKN2A, was nearly absent in all low-grade tumors. TP53 showed a high frequency of variants in high-grade tumors but mutations were rare in carcinoids. Conclusion: Based on our results, therapeutic approaches with MDM2-inhibitors and monoclonal anti-EGFR antibodies may be promising in pulmonary carcinoid tumors. PMID:27994651

  15. Overexpression of wild-type PKD2 leads to increased proliferation and invasion of BON endocrine cells

    SciTech Connect

    Jackson, Lindsey N.; Li Jing; Chen, L. Andy; Townsend, Courtney M.; Evers, B. Mark . E-mail: mevers@utmb.edu

    2006-09-29

    Carcinoid tumors are rare neuroendocrine tumors with a predilection for the gastrointestinal tract. Protein kinase D (PKD), a novel serine/threonine protein kinase, has been implicated in the regulation of transport processes in certain cell types. We have reported an important role for PKD in stimulated peptide secretion from a human (BON) carcinoid cell line; however, the role of PKD isoforms, including PKD2, in the proliferation and invasion of carcinoid tumors remains unclear. In the present study, we found that overexpression of PKD2 by stable transfection of BON cells with PKD2-wild type (PKD2{sub WT}) significantly increased proliferation and invasion compared to cells transfected with PKD2-kinase dead (PKD2{sub KD}) or pcDNA3 (control). Similarly, inhibition of PKD2 activity with small interfering RNA (siRNA) significantly decreased proliferation and invasion compared to cells transfected with non-targeting control (NTC) siRNA. These data support an important role for PKD2 in carcinoid tumor progression. Targeted inhibition of the PKD family may prove to be a novel treatment option for patients with carcinoid tumors.

  16. Neuroendocrine Tumors of the Kidney: A Single Institution Experience

    PubMed Central

    Teegavarapu, Purnima Sravanti; Rao, Priya; Matrana, Marc; Cauley, Diana H.; Wood, Christopher G; Tannir, Nizar M.

    2014-01-01

    Background Renal neuroendocrine tumors (NET), comprising carcinoid tumors and small cell carcinomas, are a rare group of neoplasms. The rarity of these tumors poses a diagnostic and therapeutic challenge. Our purpose was to characterize the cases treated at a tertiary cancer center and to evaluate patients' outcomes with the available treatment modalities. Methods This is a retrospective study of patients with renal NET seen at The University of Texas MD Anderson Cancer Center between January 1, 2001, and January 1, 2011. Patient and tumor data were analyzed by descriptive statistical methods. Results Three cases of carcinoid tumors and six cases of small cell carcinoma were identified. The median age at diagnosis was 53 years for patients with carcinoid and 65 years for patients with small cell carcinoma. The most common presenting symptoms were back pain, flank pain, and hematuria. The morphological appearance of the tumor cells and their immunohistochemical reactivity for neuroendocrine markers and cytokeratin helped establish the diagnosis. Nephrectomy was the mainstay of treatment for carcinoid tumors, yielding good long-term results, even in the presence of metastases. Surgery and chemotherapy were utilized for small cell carcinoma of the kidney. The median overall survival for patients with small cell carcinoma of the kidney was 17.3 months. Conclusion Renal carcinoid tumors are indolent and are associated with prolonged survival, while small cell carcinomas of the kidney are aggressive tumors with relatively short overall survival. Although palliative in nature, cytotoxic chemotherapy is the mainstay of therapy and is best given before surgery. PMID:25088468

  17. CD200 Expression in Neuroendocrine Neoplasms.

    PubMed

    Love, Jason E; Thompson, Kimberly; Kilgore, Mark R; Westerhoff, Maria; Murphy, Claire E; Papanicolau-Sengos, Antonios; McCormick, Kinsey A; Shankaran, Veena; Vandeven, Natalie; Miller, Faith; Blom, Astrid; Nghiem, Paul T; Kussick, Steven J

    2017-09-01

    CD200 expression has been well studied in hematopoietic malignancies; however, CD200 expression has not been well-characterized in neuroendocrine neoplasms. We examined CD200 expression in 391 neuroendocrine neoplasms from various anatomic sites. Tissue blocks containing pulmonary small cell carcinoma, pulmonary carcinoid, large cell neuroendocrine carcinoma, pancreatic neuroendocrine tumor, gastrointestinal carcinoid, and Merkel cell carcinoma were evaluated for CD200 expression by immunohistochemistry. A set of nonneuroendocrine carcinomas was stained for comparison. CD200 was expressed in 87% of the neuroendocrine neoplasms studied, including 60 of 72 (83%) pulmonary small cell carcinomas, 15 of 22 (68%) pulmonary carcinoids, three of four (75%) pulmonary large cell neuroendocrine carcinomas, 125 of 146 (86%) Merkel cell carcinomas, 79 of 83 (95%) gastrointestinal luminal carcinoids, and 56 of 60 (93%) pancreatic neuroendocrine tumors. Thirty-two of 157 (20%) nonneuroendocrine carcinomas expressed CD200. In gastrointestinal carcinoid and pancreatic neuroendocrine neoplasms, CD200 negativity correlated with higher grade. CD200 is a relatively sensitive marker of neuroendocrine neoplasms and represents a potential therapeutic target in these difficult-to-treat malignancies.

  18. Distribution and Characteristics of Pulmonary Neuroendocrine Tumors: Single Institution Experience in Lebanon.

    PubMed

    Kesrouani, Carole; Ghorra, Claude; Rassy, Marc; Kourie, Hampig Raphael; Kattan, Joseph

    2016-01-01

    Neuroendocrine tumors represent 20% of primary lung neoplasms in some registries. According to the WHO classification of 2004, reconsidered for 2015, these lung tumors are divided into 4 groups: typical and atypical carcinoid, small cell and large cell neuroendocrine carcinomas. We report in this paper, for the first time in Lebanon, the distribution and the population characteristics of these tumors. This descriptive retrospective study concerned all the pulmonary neuroendocrine tumors (NET) with their characteristics diagnosed in Hotel Dieu de France in Beirut, Lebanon from 2001 to 2012, with attention to features like age, gender and subgroup. Of 194 patients with pulmonary NET, 12.4% were typical carcinoid tumors, 3.6% atypical carcinoid, 66.5% small cell lung cancer, 7.7% combined small cell carcinomas and 9.8% large cell neuroendocrine tumors. The mean ages of patients were respectively 51.2 years in typical carcinoid, 64 years in atypical carcinoid, 64.2 years in small cell lung cancers, 67.2 in combined small cell lung cancer and 66.9 in large cells neuroendocrine tumors. The M/F sex ratios were respectively 0.3, 1.3, 1.4, 2.7 and 2.2. The characteristics of lung neuroendocrine tumors in our Lebanese institution are comparable to those reported in the literature.

  19. Array comparative genomic hybridization-based characterization of genetic alterations in pulmonary neuroendocrine tumors.

    PubMed

    Voortman, Johannes; Lee, Jih-Hsiang; Killian, Jonathan Keith; Suuriniemi, Miia; Wang, Yonghong; Lucchi, Marco; Smith, William I; Meltzer, Paul; Wang, Yisong; Giaccone, Giuseppe

    2010-07-20

    The goal of this study was to characterize and classify pulmonary neuroendocrine tumors based on array comparative genomic hybridization (aCGH). Using aCGH, we performed karyotype analysis of 33 small cell lung cancer (SCLC) tumors, 13 SCLC cell lines, 19 bronchial carcinoids, and 9 gastrointestinal carcinoids. In contrast to the relatively conserved karyotypes of carcinoid tumors, the karyotypes of SCLC tumors and cell lines were highly aberrant. High copy number (CN) gains were detected in SCLC tumors and cell lines in cytogenetic bands encoding JAK2, FGFR1, and MYC family members. In some of those samples, the CN of these genes exceeded 100, suggesting that they could represent driver alterations and potential drug targets in subgroups of SCLC patients. In SCLC tumors, as well as bronchial carcinoids and carcinoids of gastrointestinal origin, recurrent CN alterations were observed in 203 genes, including the RB1 gene and 59 microRNAs of which 51 locate in the DLK1-DIO3 domain. These findings suggest the existence of partially shared CN alterations in these tumor types. In contrast, CN alterations of the TP53 gene and the MYC family members were predominantly observed in SCLC. Furthermore, we demonstrated that the aCGH profile of SCLC cell lines highly resembles that of clinical SCLC specimens. Finally, by analyzing potential drug targets, we provide a genomics-based rationale for targeting the AKT-mTOR and apoptosis pathways in SCLC.

  20. Immunotherapy for gastric premalignant lesions and cancer.

    PubMed

    Zorzetto, Valerio; Maddalo, Gemma; Basso, Daniela; Farinati, Fabio

    2012-06-01

    Chronic atrophic gastritis, a precancerous change for gastric cancer, shows a loss of appropriate glands, Helicobacter pylori infection and autoimmune gastritis being the two main etiologic factors. While H. pylori eradication is the mandatory treatment for the former, no etiologic treatment is available for the latter, in which a Th1-type response, modulated by Tregs and Th17 cells, is involved. H. pylori-related atrophic gastritis is a risk factor for gastric adenocarcinoma, while autoimmune atrophic gastritis is also linked to a substantial risk of gastric type I carcinoid, related to the chronic stimulus exerted by hypergastrinemia on enterochromaffin-like cells. Several studies have been published on gastric cancer treatment through an active specific immunotherapy, aimed at improving the immunoregulatory response and increasing the circulating tumor-specific T cells. No study on immunotherapy of carcinoids is available but, in our experience, the administration of an antigastrin 17 vaccine induced carcinoid regression in two out of three patients treated.

  1. Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis.

    PubMed

    Dominguez-Comesaña, Elias; Tome-Espiñeiro, Catherine; Ulla-Rocha, Jose L; Lorenzo-Lorenzo, Isabel; Lede-Fernandez, Angel; Portela-Serra, Jose L

    2011-09-01

    Carcinoids of the ampulla of Vater are infrequent tumors of which a quarter of cases have been detected in patients with type I neurofibromatosis. This hereditary disease is also associated with gastrointestinal stromal tumors (GIST). However, the coincidence of these three entities together have only been formerly detected in five cases. A 53 year-old female patient, diagnosed with type I neurofibromatosis, with a malignant carcinoid of ampulla of Vater and multiple gastrointestinal stromal tumors in the duodenum and jejunum, was treated with total pancreatectomy and the excision of her intestinal tumors. Five-years on, a follow-up showed the patient to be well, and free from tumor recurrence. The coexistence of an ampullary carcinoid tumor, GIST and neurofibramatosis is very rare. Radical curative surgical resection is a good treatment option, but the optimal management of this is not yet well established.

  2. [Pulmonary neuroendocrine tumors and preneoplasic lesions].

    PubMed

    Rouquette Lassalle, Isabelle

    2016-01-01

    In the recently published 2015 World Health Organization (WHO) classification of tumors of the lungs, all neuroendocrine tumors of the lungs are presented for the first time in one single chapter. In this classification, high-grade small cell lung cancer (SCLC) and large cell neuroendocrine carcinoma (LCNEC) are differentiated from intermediate grade atypical carcinoids (AC) and low-grade typical carcinoids as well as from preinvasive lesion diffuse neuroendocrine hyperplasia DIPNECH. In the 2004 WHO classification, SCLC and carcinoids each had a separate chapter and LCNEC was listed in the chapter on large cell carcinoma of the lungs. The new WHO classification also gives some recommendations for the diagnosis on small biopsies. This review describes morphological, immunohistochemical, and genomic characteristic of these tumors according to the new classification.

  3. The pituitary V3 vasopressin receptor and the corticotroph phenotype in ectopic ACTH syndrome.

    PubMed

    de Keyzer, Y; Lenne, F; Auzan, C; Jégou, S; René, P; Vaudry, H; Kuhn, J M; Luton, J P; Clauser, E; Bertagna, X

    1996-03-01

    Ectopic ACTH secretion occurs in highly differentiated and rather indolent tumors like bronchial carcinoids or, in contrast, in various types of aggressive and poorly differentiated neuroendocrine tumors. We explored this phenomenon using the recently cloned human pituitary V3 vasopressin receptor as an alternate molecular marker of the corticotroph phenotype. Expression of V3 receptor, corticotrophin releasing hormone (CRH) receptor, and proopiomelanocortin (POMC) genes was examined in tumors of pituitary and nonpituitary origin. A comparative RT-PCR approach revealed signals for both V3 receptor and CHR receptor mRNAs in 17 of 18 ACTH-secreting pituitary adenomas, and 6 of 6 normal pituitaries; in six growth hormone- or prolactin-secreting adenomas, a very faint V3 receptor signal was observed in three cases, and CRH receptor signal was undetected in all. Six of eight bronchial carcinoids responsible for the ectopic ACTH syndrome had both POMC and V3 receptor signals as high as those in ACTH-secreting pituitary adenomas; in contrast, no POMC signal and only a very faint V3 receptor signal were detected in six of eight nonsecreting bronchial carcinoids. Northern blot analysis showed V3 receptor mRNA of identical size in ACTH-secreting bronchial carcinoids and pituitary tumors. Other types of nonpituitary tumors responsible for ectopic ACTH syndrome presented much lower levels of both POMC and V3 receptor gene expression than those found in ACTH-secreting bronchial carcinoids. In contrast with the V3 receptor, CRH receptor mRNA was detected in the majority of neuroendocrine tumors irrespective of their POMC status. These results show that expression of the V3 receptor gene participates in the corticotroph phenotype. Its striking association with ACTH-secreting bronchial carcinoids defines a subset of nonpituitary tumors in which ectopic POMC gene expression is but one aspect of a wider process of corticotroph cell differentiation, and opens new possibilities of

  4. [Anorectal neuroendocrine carcinoma: observations on a case and review of the literature].

    PubMed

    Messinetti, S; Giacomelli, L; Drudi, F M; Innocenzi, D; Porcelli, C; Fabrizio, G; Finizio, R; Manno, A; Granai, A V

    1994-01-01

    Carcinoid tumours of the anorectum are rare (0.7% of malignant rectal tumours). Because of this rarity several aspects of the management of these tumours remain controversial. Diagnosis may be delayed because of failure to recognize their morphological characteristics and histological appearance may not reflect their biological behaviour. Immunocytochemistry for neuroendocrine-cells are essential to identify different types of carcinoid tumours and to do differential diagnosis from other malignant tumours. All that allow an exact therapeutic approach to these tumours. The tumours less than cm 1 in diameter can be safely treated by local excision; the tumours more than cm 1 in diameter are treated by radical surgery (AAP).

  5. An 111In-Pentetreotide Positive Sclerosing Pneumocytoma.

    PubMed

    Savelli, Giordano; Bnà, Claudio; Zambelli, Claudia; Illuminati, Sonia; Bonello, Luke

    2017-04-01

    A 43-year-old woman had an incidental lung mass identified on shoulder x-ray performed for pain. Contrast-enhanced CT showed a 38-mm mass in the medial segment of the right middle lobe, with features suggestive of carcinoid tumor. A In-pentetreotide scan showed intense uptake; furthermore, fine needle aspiration biopsy yielded neuroendocrine cells confirming the carcinoid hypothesis. However, definitive surgical histology showed a sclerosing pneumocytoma. This could potentially suggest that such rare tumors, with metastatic potential, could respond to somatostatin analogue treatment.

  6. [Trends in prognostic factors for neuroendocrine lung tumors].

    PubMed

    García-Yuste, Mariano; Molins, Laureano; Matilla, José M; González-Aragoneses, Federico; López-Pujol, Javier; Ramos, Guillermo; de la Torre, Mercedes

    2007-10-01

    The aim of this study was to analyze trends in a variety of prognostic factors for neuroendocrine lung carcinomas through analysis of 2 groups of surgically treated patients. Group A contained the first 361 patients, treated between 1980 and 1997. That group was analyzed retrospectively and contained 261 patients with typical carcinoid tumors, 43 with atypical carcinoid tumors, 22 with large-cell neuroendocrine carcinoma, and 35 with small-cell neuroendocrine carcinoma. Group B contained 404 patients enrolled prospectively between 1998 and 2002: 308 with typical carcinoid tumors, 49 with atypical carcinoid tumors, 18 with large-cell neuroendocrine carcinoma, and 29 with small-cell neuroendocrine carcinoma. The following clinical variables were considered: sex, mean age, tumor site, tumor size, lymph node involvement, stage, metastasis, and local recurrence. The 1997 TNM classification was used for staging of lung cancer and survival analysis was performed along with assessment of factors influencing survival. Statistical analysis of the data involved univariate and multivariate analysis. In both groups, significant differences were observed between patients with typical and atypical carcinoid tumors in terms of mean age, tumor size, node involvement, and recurrence. In group A, female sex, node involvement, and recurrence differed between patients with atypical carcinoid tumors and those with large-cell neuroendocrine carcinoma; the same was true for group B, with the exception of lymph node involvement. Node involvement differed between patients with small-cell versus large-cell neuroendocrine carcinoma in group A but not group B. Both groups displayed significant differences in overall survival and survival of patients with lymph node involvement between patients with typical and atypical carcinoid tumors and between patients with atypical carcinoid tumors and those with large-cell neuroendocrine carcinoma; no differences were observed between patients with large

  7. Development of Antidepressants as Novel Agents To Treat Small Cell Lung Cancer

    DTIC Science & Technology

    2014-08-01

    indicates that Merkel cell carcinoma, midgut carcinoid tumors, pheochromocytoma, and neuroblastoma tumor cells also express several of the main GPCR...pheochromocytomas, and 88 neuroblastomas . B and C, MTT viability assays of human pancreatic adenocarcinoma (PDAC), mouse pancreatic neuroendocrine tumors...PNET), human neuroblastoma (NB), human Merkel cell carcinoma (MCC), human large cell adenocarcinoma (LCLC), and neuroendocrine large cell lung carcinoma

  8. T-Cell Gene Therapy to Eradicate Disseminated Breast Cancers

    DTIC Science & Technology

    2011-05-01

    diagnosis and possible pathogenesis. Leuk Lymphoma. 2003;44(12):2103-2108. 109. Ladoyanni E, Nambi R. Psoriasis exacerbated by interferon-alpha in a...aggravation of psoriasis 51,108-111 IFN-α Cutaneous T cell lymphoma Psoriasis 112 IFN-α Mid-gut carcinoid tumors Systemic lupus erythmatosis

  9. Identification of deregulation of apoptosis and cell cycle in neuroendocrine tumors of the lung via NanoString nCounter expression analysis

    PubMed Central

    Walter, Robert Fred Henry; Werner, Robert; Ting, Saskia; Vollbrecht, Claudia; Theegarten, Dirk; Christoph, Daniel Christian; Schmid, Kurt Werner; Wohlschlaeger, Jeremias; Mairinger, Fabian Dominik

    2015-01-01

    Background Neuroendocrine tumors of the lung comprise typical (TC) and atypical carcinoids (AC), large-cell neuroendocrine cancer (LCNEC) and small-cell lung cancer (SCLC). Cell cycle and apoptosis are key pathways of multicellular homeostasis and deregulation of these pathways is associated with cancerogenesis. Materials and Methods Sixty representative FFPE-specimens (16 TC, 13 AC, 16 LCNEC and 15 SCLC) were used for mRNA expression analysis using the NanoString technique. Eight genes related to apoptosis and ten genes regulating key points of cell cycle were investigated. Results ASCL1, BCL2, CASP8, CCNE1, CDK1, CDK2, CDKN1A and CDKN2A showed lower expression in carcinoids compared to carcinomas. In contrast, CCNE1 and CDK6 showed elevated expression in carcinoids compared to carcinomas. The calculated BCL2/BAX ratio showed increasing values from TC to SCLC. Between SCLC and LCNEC CDK2, CDKN1B, CDKN2A and PNN expression was significantly different with higher expression in SCLC. Conclusion Carcinoids have increased CDK4/6 and CCND1 expression controlling RB1 phosphorylation via this signaling cascade. CDK2 and CCNE1 were increased in carcinomas showing that these use the opposite way to control RB1. BAX and BCL2 are antagonists in regulating apoptosis. BCL2 expression increased over BAX expression with increasing malignancy of the tumor from TC to SCLC. PMID:26008974

  10. Gastrin: from pathophysiology to cancer prevention and treatment.

    PubMed

    Maddalo, Gemma; Spolverato, Ylenia; Rugge, Massimo; Farinati, Fabio

    2014-07-01

    Gastrin has been identified as the principal effector of gastric secretion, but several studies have demonstrated its role as a biomarker of cancer risk and as a growth factor for colorectal, stomach, liver, and pancreatic cancer. Hypergastrinemia characterizes autoimmune gastritis, with body and fundic gland atrophy and increased risk for both gastric adenocarcinoma and neuroendocrine tumors. Gastric type I carcinoids develop in the context of autoimmune gastritis because of the stimulus exerted by gastrin on enterochromaffin-like cells and remain gastrin-sensitive for long durations because the removal of hypergastrinemia leads to tumor regression. The treatment of gastric carcinoid is still open to debate, but when the disease frequently relapses, or is multicentric or infiltrating, surgery is advocated or, in the alternative, a costly and long-lasting treatment with long-acting somatostatin analogues is prescribed. A technology allowing the preparation of an immunogen eliciting an immune system response with generation of antibodies against G17 has been developed. This vaccine has been tested in patients with colorectal, pancreatic or advanced gastric cancer. The vaccine has also been used in the treatment of gastric type I carcinoids, and the administration of G17DT in patients harboring these lesions leads to carcinoid regression. Antigastrin vaccination in the treatment of gastrointestinal cancer obviously needs validation, but this immunotherapy may well represent a simple, inexpensive, and active 'adjuvant' treatment.

  11. Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma

    ClinicalTrials.gov

    2016-07-14

    Gastrin-Producing Neuroendocrine Tumor; Lung Carcinoid Tumor; Metastatic Digestive System Neuroendocrine Tumor G1; Pancreatic Glucagonoma; Pancreatic Insulinoma; Pancreatic Polypeptide Tumor; Paraganglioma; Recurrent Digestive System Neuroendocrine Tumor G1; Recurrent Merkel Cell Carcinoma; Recurrent Pancreatic Neuroendocrine Carcinoma; Regional Digestive System Neuroendocrine Tumor G1; Somatostatin-Producing Neuroendocrine Tumor; Stage III Merkel Cell Carcinoma; Stage IV Merkel Cell Carcinoma; Thyroid Gland Medullary Carcinoma

  12. Trebananib And Temsirolimus in Treating Patients With Solid Tumors That Are Metastatic or Cannot Be Removed by Surgery

    ClinicalTrials.gov

    2015-10-05

    Adult Solid Neoplasm; Lung Carcinoid Tumor; Recurrent Digestive System Neuroendocrine Tumor G1; Recurrent Renal Cell Carcinoma; Recurrent Uterine Corpus Sarcoma; Stage III Renal Cell Cancer; Stage IIIB Uterine Sarcoma; Stage IIIC Uterine Sarcoma; Stage IV Renal Cell Cancer; Stage IVA Uterine Sarcoma; Stage IVB Uterine Sarcoma

  13. Pazopanib Hydrochloride in Treating Patients With Advanced Neuroendocrine Cancer

    ClinicalTrials.gov

    2015-10-15

    Gastrin-Producing Neuroendocrine Tumor; Lung Carcinoid Tumor; Metastatic Digestive System Neuroendocrine Tumor G1; Multiple Endocrine Neoplasia Type 1; Pancreatic Glucagonoma; Pancreatic Insulinoma; Pancreatic Polypeptide Tumor; Recurrent Digestive System Neuroendocrine Tumor G1; Recurrent Pancreatic Neuroendocrine Carcinoma; Regional Digestive System Neuroendocrine Tumor G1; Somatostatin-Producing Neuroendocrine Tumor

  14. Cancer and the heart

    SciTech Connect

    Kapoor, A.S.

    1986-01-01

    This book contains 28 chapters. Some of the titles are: Computed tomography of neoplastic disease of the pericardium; Radiation therapy and the heart; Valvular involvement in cancer; Smoking, lung cancer, and coronary heart disease; Carcinoid heart disease; Cardiac amyloidosis; and Anemia of cancer and its cardiac effects.

  15. Transformation of prostatic adenocarcinoma to well-differentiated neuroendocrine tumor after hormonal treatment.

    PubMed

    Gilani, Syed; Guo, Charles C; Li-Ning, Elsa M; Pettaway, Curtis; Troncoso, Patricia

    2017-06-01

    Carcinoid tumor of the prostate is extremely rare. Here we report a unique case of prostate cancer that underwent complete transformation from conventional adenocarcinoma to carcinoid-like tumor shortly after androgen-deprivation treatment (ADT). The patient was a 59-year-old man who presented with lower urinary tract symptoms. His biopsy specimen demonstrated a high-grade prostatic adenocarcinoma with mixed acinar and ductal features. After ADT for 6months, the patient underwent radical prostatectomy. The post-ADT tumor showed monotonous neoplastic cells with fine granular chromatin forming rosette-like structures, resembling a carcinoid tumor. No residual conventional adenocarcinoma was present. On immunostain, the tumor cells were diffusely positive for synaptophysin and chromogranin and negative for prostate-specific antigen and prostein. Thus, the carcinoid-like tumor represented complete transformation from prostatic adenocarcinoma to well-differentiated neuroendocrine tumor after ADT. This unique case highlights the important role of ADT in neuroendocrine differentiation of prostate cancer. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. The Physiology of Growth Hormone-Releasing Hormone (GHRH) in Breast Cancer

    DTIC Science & Technology

    2003-06-01

    production of growth hormone-releasing factor by carcinoid and pancreatic islet tumors associated with acromegaly . Prog Clin Biol Res 1981; 74:259-271. (16...promotion of apop- cause of acromegaly . More recently, expression has been tosis. These results indicate that disruption of enaog- demonstrated in tumors

  17. Corticotropin-releasing hormone, proopiomelanocortin, and glucocorticoid receptor gene expression in adrenocorticotropin-producing tumors in vitro.

    PubMed Central

    Suda, T; Tozawa, F; Dobashi, I; Horiba, N; Ohmori, N; Yamakado, M; Yamada, M; Demura, H

    1993-01-01

    To differentiate between ectopic ACTH syndrome and Cushing's disease, gene expression of corticotropin-releasing hormone (CRH), proopiomelanocortin (POMC), and glucocorticoid receptor was examined in 10 pituitary adenomas (Cushing's disease) and in 10 ectopic ACTH-producing tumors. CRH increased plasma ACTH levels in all patients with Cushing's disease and in five patients with ectopic ACTH syndrome whose tumors contained CRH and CRH mRNA. In five CRH nonresponders, CRH was not detected in tumors that contained no CRH mRNA or that contained only long-size CRH mRNA. Dexamethasone (Dex) decreased plasma ACTH levels in all patients with Cushing's disease and in three patients with ectopic ACTH-producing bronchial carcinoid. These tumors contained glucocorticoid receptor mRNA. CRH increased and Dex decreased ACTH release and POMC mRNA levels in pituitary adenoma and bronchial carcinoid cells. PMA increased POMC mRNA levels only in carcinoid cells. These results reveal characteristics of ectopic ACTH-producing tumors: long-size CRH mRNA and PMA-induced POMC gene expression. In addition, there are two ectopic ACTH syndrome subtypes: tumors containing ACTH with CRH (CRH responder) and tumors without CRH. Dex decreases ACTH release and POMC mRNA levels in some bronchial carcinoids. Therefore, CRH and Dex tests have limited usefulness in differentiating between Cushing's disease and ectopic ACTH syndrome. Images PMID:8254033

  18. Deficiencies in fat-soluble vitamins in long-term users of somatostatin analogue.

    PubMed

    Fiebrich, H-B; Van Den Berg, G; Kema, I P; Links, T P; Kleibeuker, J H; Van Beek, A P; Walenkamp, A M E; Sluiter, W J; De Vries, E G E

    2010-12-01

    Somatostatin analogues are administered to control hormone hypersecretion in acromegaly and carcinoid patients. Somatostatin analogues can increase fat in the stools, which can lead to loss of fat-soluble vitamins. The effect of long-term somatostatin analogue use on vitamin levels remains unknown. To investigate the prevalence of fat-soluble vitamin deficiencies in long-term somatostatin analogue users. All acromegaly and carcinoid patients using somatostatin analogues for ≥ 18 months visiting the University Medical Center Groningen between December 2008 and April 2009 were eligible. Vitamin levels of fat-soluble vitamins in blood, clinical and vitamin-dependent laboratory parameters were collected. In all, 19 acromegaly and 35 carcinoid patients were included. Twelve patients experienced steatorrhoea; two carcinoid patients experienced night blindness. Forty-two (78%) were deficient for one or more vitamins, and 32% (n = 17) had multiple deficiencies. Deficiencies for vitamin A, D, E, K1 and E in erythrocytes occurred in 6%, 28%, 15%, 63% and 58% of the patients. Prevalence of vitamin D, E and K1 deficiencies was similar in both patient groups. Treatment duration did not influence vitamin levels. The length of intestinal resection and age correlated negatively with vitamin A levels. Fat-soluble vitamin deficiencies are frequent during long-term somatostatin analogue treatment. Therefore, fat-soluble vitamins should be monitored in these patients. © 2010 Blackwell Publishing Ltd.

  19. Tumeur stromale rectale: à propos d'une observation

    PubMed Central

    Rejab, Haitham; Kridis, Wala Ben; Ben Ameur, Hazem; Feki, Jihene; Frikha, Mounir; Beyrouti, Mohamed Issam

    2014-01-01

    Les tumeurs stromales gastro-intestinales sont des tumeurs mésenchymateuses peu fréquentes. Elles sont localisées préférentiellement eu niveau de l'estomac. La localisation rectale reste rare. A un nouveau cas de tumeur stromale du rectum ainsi qu'une bref revue de la littérature, on se propose d’étudier les particularités cliniques, radiologiques et thérapeutiques de cette entité rare. PMID:25120863

  20. Co-Targeting the PI3K and RAS Pathways for the Treatment of Neuroendocrine Tumors

    PubMed Central

    Valentino, Joseph D.; Li, Jing; Zaytseva, Yekaterina Y.; Mustain, W. Conan; Elliott, Victoria A.; Kim, Ji Tae; Harris, Jennifer W.; Campbell, Katherine; Weiss, Heidi; Wang, Chi; Song, Jun; Anthony, Lowell; Townsend, Courtney M.; Evers, B. Mark

    2014-01-01

    Background The precise involvement of the PI3K/mTOR and RAS/MEK pathways in carcinoid tumors is not well defined. Therefore, the purpose of our study was to evaluate the role these pathways play in carcinoid cell proliferation, apoptosis, and secretion and to determine the effects of combined treatment on carcinoid tumor inhibition. Methods The human neuroendocrine cell lines BON (pancreatic carcinoid), NCI-H727 (lung carcinoid), and QGP-1 (somatostatinoma) were treated with either the pan-PI3K inhibitor, BKM120, or the dual PI3K-mTOR inhibitor, BEZ235, alone or in combination with the MEK inhibitor, PD0325901; proliferation, apoptosis, and protein expression were assessed. Peptide secretion was evaluated in BON and QGP-1 cells. The anti-proliferative effect of BEZ235, alone or combined with PD0325901, was then tested in vivo. Results Both BKM120 and BEZ235 decreased proliferation and increased apoptosis; combination with PD0325901 significantly enhanced the antineoplastic effects of either treatment alone. In contrast, neurotensin (NT) peptide secretion was markedly stimulated with BKM120 treatment, but not BEZ235. The combination of BEZ235 + PD0325901 significantly inhibited the growth of BON xenografts without systemic toxicity. Conclusions Both BKM120 and BEZ235 effectively inhibited NET cell proliferation and stimulated apoptosis. However, inhibition of the PI3K pathway alone with BKM120 significantly stimulated NT peptide secretion; this did not occur with the dual inhibition of both PI3K and mTOR using BEZ235 suggesting that this would be a more effective treatment regimen for NETs. Moreover, the combination of BEZ235 and the MEK inhibitor PD0325901 was a safe and more effective therapy in vivo compared with single agents alone. PMID:24443523