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Sample records for primary retroperitoneal teratoma

  1. 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.

  2. Retroperitoneal teratoma in a skunk (Mephitis mephitis).

    PubMed

    Munday, John S; Fairchild, Steve E; Brown, Cathy A

    2004-09-01

    A 4-yr-old striped skunk (Mephitis mephitis) developed an 11- x 7- x 7-cm retroperitoneal mass caudal to the left kidney. The mass was surgically excised and, on the basis of histology and immunohistochemistry, diagnosed as a benign teratoma. Six months later, a second histologically similar neoplasm was excised from the same location and was considered to represent local tumor recurrence. The skunk died 16 mo later without recurrence. Complete excision of the tumor appeared curative in this case and supported the benign diagnosis. An extragonadal teratoma should be considered as a differential diagnosis for an abdominal mass in a skunk.

  3. Primary retroperitoneal mature cystic teratoma with focal enteric type adenocarcinoma in a post-partum woman: report of a case with literature review.

    PubMed

    Hong, Wei; Dumoff, Kimberly L; Torigian, Drew A; Bing, Zhanyong

    2013-02-11

    Teratomas are characterized by containing tissue from all three germinal cell layers. Occasionally, somatic type malignancies develop within a mature cystic teratoma. We reported here a rare case of enteric type adenocarcinoma, with associated dysplastic epithelial precursor lesion, arising within a mature cystic teratoma in the retroperitoneum of a 30-year-old woman status post vaginal delivery 11 weeks earlier. The mass is 17.5 cm and cystic. A polypoid mass component measuring 4.7×4.2×2.5 cm was located inside the cystic component. Microscopically, the majority of the specimen was a mature cystic teratoma with all three germinal cell layers. The polypoid mass component was an adenocarcinoma with an adjacent dysplastic epithelial precursor lesion. The adenocarcinoma was diffusely positive for CK20 and CDX-2, and focally positive for CD7, indicating enteric differentiation. A brief review of retroperitoneal mature cystic teratomas with associated somatic type malignancy was performed.

  4. Primary medulla oblongata teratomas.

    PubMed

    Li, Da; Hao, Shu-Yu; Wu, Zhen; Zhang, Li-Wei; Zhang, Jun-Ting

    2014-09-01

    Medulla oblongata teratomas are rare. The authors report 2 new cases of teratomas that occurred exclusively in the medulla oblongata. The first case was in a 9-year-old boy who presented with a 6-month history of neck pain and repeated paroxysmal vomiting. Based on preoperative radiographic findings, the initial diagnosis was of an intraaxial medulla oblongata hemangioblastoma. Intraoperatively, the cystic component of the tumor was gray, gelatinous, and soft in consistency. The solid component was light pink, rubbery, and nodular in appearance, with an identifiable boundary. The lesion was completely removed. Histopathological investigation revealed a mature teratoma. Postoperatively, the patient was supported with ventilator assistance and received a tracheotomy, but died of intracranial infection. The second case was in a 10-year-old boy with intermittent headache for 1 month. Radiographs revealed an exophytic cystic and solid lesion with dorsal involvement of the medulla oblongata. The lesion was predominantly solid, pinkish gray, tenacious, and moderately vascularized, with clearly delineated surgical dissection planes. The histopathological examination confirmed a diagnosis of immature teratoma. Total resection was achieved, followed by postoperative chemotherapy. He was alive without recurrence of the lesion or symptoms at 59 months after surgery. Resection of medulla oblongata teratoma is challenging, with inherent surgical risks that are contingent on the tumor growth pattern. Teratomas should be considered in the differential diagnosis of brainstem lesions. Chemotherapy has been suggested for immature teratomas. Long-term follow-up and larger studies of teratomas in unusual locations are required to improve practitioners' understanding of this disease's treatment and outcomes.

  5. CT characteristics of primary retroperitoneal neoplasms in children.

    PubMed

    Xu, Yufeng; Wang, Jichen; Peng, Yun; Zeng, Jinjin

    2010-09-01

    Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.

  6. A Retroperitoneal Isolated Enteric Duplication Cyst Mimicking a Teratoma: A Case Report and Literature Review

    PubMed Central

    Momosaka, Daichi; Ushijima, Yasuhiro; Asayama, Yoshiki; Ishigami, Kousei; Takayama, Yukihisa; Okamoto, Daisuke; Fujita, Nobuhiro; Ikeda, Tetsuo; Uchida, Keiichiro; Sugimoto, Masaaki; Kohashi, Kenichi; Honda, Hiroshi

    2016-01-01

    Enteric duplication cysts lacking anatomic association with the gastrointestinal tract are called isolated enteric duplication cysts (IEDCs). We present an atypical case of a retroperitoneal IEDC with a tortuous tubular complex shape that enfolded the surrounding retroperitoneal fat and mimicked a retroperitoneal teratoma. Multiplanar reconstruction images should be used to evaluate such a lesion correctly. A tortuous tubular complex shape could be a key finding to differentiate from other retroperitoneal cysts. PMID:28083153

  7. Primary renal teratoma: a rare entity

    PubMed Central

    2013-01-01

    Abstract Teratomas are neoplasms that arise from pluripotent cells and can differentiate along one or more embryonic germ lines. Renal teratoma is an exceedingly rare condition. Teratomas commonly arise in the gonads, sacrococcygeal region, pineal gland, and retroperitoneum. They present mainly as an abdominal mass with few other symptoms. Majority of the tumors are benign, situated on the left side and para renal, occasional lesions are bilateral. If diagnosed early, they are amenable to curative excision. Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retroperitoneal teratomas secondarily invading the kidney. The differentiation between these two neoplasms in the kidney is often problematic. We present a case of intrarenal immature teratoma in a six-month-old baby girl. Virtual slides The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1746249869599954. PMID:23800134

  8. The Case of an Elderly Male Patient with Unknown Primary Mucinous Adenocarcinoma within Presacral Teratoma (Teratoma with Malignant Transformation)

    PubMed Central

    Cokmert, Suna; Koca, Emine; Bulut, Naki; Gul, Suha; Yilmaz, Nevin

    2015-01-01

    Teratomas are rarely seen in adults, and presacral region is an area where they rarely settle in. Similarly, only about 1% of teratomas show malignant transformation. Malignant transformation is often associated with the area where teratoma settles in. Malignant transformation of mediastinal teratomas is more frequent than the ones located in retroperitoneal area and gonad. They most commonly show rhabdomyosarcoma, primitive neuroectodermal tumor, enteric adenocarcinoma, and leukemia transformation. In teratomas showing malignant transformation, the clinical course is aggressive; and survival of patients with metastatic disease is very low. The primary treatment of teratomas with malignant transformations is surgical. Effect of radiotherapy and chemotherapy is not clear in patients, to whom surgical operation cannot be applied, or those who are with residual tumor, even if surgical operation can be applied to them, or those who are at metastatic stage. In this paper, we presented a 76-year-old male patient due to the histologic diagnosis of mucinous adenocarcinoma within teratoma, in whom approximately 7 cm presacral mass was found during the radiographic examination made by the reason of low back pain and pelvic pain. PMID:25874143

  9. Primary mature cystıc teratoma mimickıng an adrenal mass in an adult male patient.

    PubMed

    Okulu, Emrah; Ener, Kemal; Aldemir, Mustafa; Isik, Evren; Irkkan, Cigdem; Kayigil, Onder

    2014-02-01

    Teratomas are bizarre neoplasms derived from embryonic tissues that are typically found only in the gonadal and sacrococcygeal regions of adults. Primary retroperitoneal teratomas are rare and present challenging management options. We report a case of a unilateral primary retroperitoneal mature cystic teratoma mimicking an adrenal mass in a 54-year-old male patient. Complete resection of the adrenal mass was performed by the flank approach by using the 11th rib resection. Because of the risk of malignancy, follow-up radiographic studies were performed to ensure the oncologic efficacy of resection. The patient has been free of recurrence for longer than 12 months.

  10. Primary retroperitoneal acinar cell cystadenoma.

    PubMed

    Pesci, Anna; Castelli, Paola; Facci, Enrico; Romano, Luigi; Zamboni, Giuseppe

    2012-03-01

    In this report, we describe a case of hitherto unreported primary retroperitoneal acinar cell cystadenoma that morphologically and immunophenotypically resembled pancreatic acinar cell cystadenoma. Pancreatic acinar cell cystadenoma is a very uncommon benign lesion characterized by acinar cell differentiation, the evidence of pancreatic exocrine enzyme production, and the absence of cellular atypia. Our case occurred in a 55-year-old woman presenting a 10-cm multilocular cystic lesion in the retroperitoneum thought to be a mucinous cystic neoplasm. At laparotomy, the cystic mass, which showed no connection with any organ, was completely resected with a clinical diagnosis of cystic lymphangioma. The diagnosis of retroperitoneal acinar cell cystadenoma was based on the recognition of morphological acinar differentiation, the immunohistochemical demonstration of the acinar marker trypsin, and the absence of cellular atypia. These peculiar features can be used in the differential diagnosis with all the other cystic lesions of the retroperitoneum.

  11. Primary retroperitoneal mucinous cystadenoma with sarcoma-like mural nodule

    PubMed Central

    Bakker, R. F. R.; Stoot, J. H. M. B.; Blok, P.

    2007-01-01

    Primary retroperitoneal cystadenomas are extremely rare. This is the first report in literature to describe a primary retroperitoneal cystadenoma with a sarcoma-like mural nodule. A 45-year-old woman complained of a left-sided abdominal mass. A computed tomography scan revealed a cystic mass with a mural nodule, which seemed to originate from the tail of the pancreas. At laparotomy the cyst was not adhered to the pancreas but localized retroperitoneally. Histologic examination showed a mucinous cystadenoma with only foci of borderline malignancy with a mural “sarcoma-like” nodule. In view of the surgical and histopathological findings, the mucinous cystadenoma was regarded as primary retroperitoneal. This case demonstrates that in the era of radiological preoperative refinement, pathological diagnosis remains of utmost importance, especially for rare cases. PMID:17690906

  12. Primary retroperitoneal Merkel cell carcinoma: Case report and literature review

    PubMed Central

    Quiroz-Sandoval, Osvaldo A.; Cuellar-Hubbe, Mario; Lino-Silva, Leonardo S.; Salcedo-Hernández, Rosa A.; López-Basave, Horacio N.; Padilla-Rosciano, Alejandro E.; León-Takahashi, Alberto M.; Herrera-Gómez, Ángel

    2015-01-01

    Background Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine carcinoma that affects elderly patients and typically arises in sun-exposed skin. The disease is very rare and only few cases present with no apparent skin lesion. In the retroperitoneum there are only two cases reported in the literature. Case presentation We report a case of a 54-year-old Mexican male with MCC, which presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed a MCC. The patient underwent preoperative chemotherapy followed by a laparotomy and the mass was successfully excised. Discussion There are two possible explanations for what occurred in our patient. The most plausible theory is the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin. The less probable theory is the non-described “regression” phenomena of a cutaneous MCC, but we are not found a primary skin lesion. Conclusion Preoperative chemotherapy and excision of the primary tumor is the surgical treatment of choice for retroperitoneal MCC. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional and unconventional patients with MCC. PMID:26708276

  13. Primary retroperitoneal malignant melanoma: A case report

    PubMed Central

    LIU, GUO-BING; WU, GUANG-YAO; GHIMIRE, PRASANNA; ZHANG, ZAI-PENG

    2011-01-01

    Primary malignant melanoma occurring at an extra cutaneous site is rare. A case of primary malignant melanoma located in the retroperitoneum of an 18-year-old female is presented in this study. Histopathological examination of the tissue biopsies at laparotomy with immunohistochemical stains confirmed a diagnosis of malignant melanoma. Further extensive clinical and radiological investigations proved the retroperitoneum to be the primary site. PMID:22848275

  14. 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.

  15. 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

  16. Retroperitoneal and intrahepatic metastasis from primary clear cell carcinoma of the liver

    PubMed Central

    Xiong, Junjie; He, Du; Hu, Weiming; Liu, Xubao

    2017-01-01

    Abstract Background: Hepatocellular carcinoma (HCC) is a major cause of cancer-related mortality worldwide and the incidence is increasing as a result of growing hepatitis B and C virus infections. Primary clear cell carcinoma of the liver (PCCCL) is a rare subgroup of primary HCC, which has low metastatic potential and infrequently reported in literature. Retroperitoneal and intrahepatic metastasis of PCCCL has not been reported previously. Case Summary: Here, we present a 55-year-old male with retroperitoneal and intrahepatic metastasis of PCCCL who is managed with surgical method and transcatheter arterial chemoembolization (TACE) at our institution. When the patient is followed up in 16 months after surgery and TACE, he is alive without any extrahepatic metastasis and abnomal liver function. Conclusion: We concluded that surgical resection of retroperitoneal metastasis and TACE of the intrahepatic tumors provided an appropriate strategy for the patient with unresectable PCCCL accompanied with extra-hepatic metastasis. PMID:28328858

  17. Signet ring cell-type adenocarcinoma arising in a mature teratoma of the testis

    PubMed Central

    HA, HONG KOO; LEE, WAN; LEE, SANG DON; LEE, JEONG ZOO; CHUNG, MOON KEE

    2010-01-01

    A 48-year-old male who presented with an enlarged right scrotum was diagnosed with malignant transformation of testicular teratoma. Physical examination revealed a right scrotal mass of hard consistency with no inguinal lymphadenopathy. Since prepuberty, his right testis had been larger than the left one, with no pain or tenderness. Computed tomography and bone scan revealed retroperitoneal lymphadenopathy and multiple bone metastases. Right orchiectomy was performed immediately, and a pathological examination revealed a mature teratoma associated with adenocarcinoma, showing signet ring cell differentiation. Cisplatin-based combination chemotherapy was administered; however, the metastatic lesions progressed, and the patient succumbed to the disease after 15 months. Only a few cases of primary malignant transformation of teratoma in the testis have been reported, and this is the first case report of primary malignant transformation of teratoma in the testis with signet ring cell-type differentiation. PMID:22966298

  18. Primary retroperitoneal transitional cell carcinoma presenting as a dumb-bell tumour.

    PubMed

    Basu, S; Ansari, M; Gupta, S; Kumar, A

    2009-11-01

    We report a retroperitoneal transitional cell carcinoma arising from the primitive urogenital remnants of a 56-year-old married Indian woman. She presented with a huge cystic mass in the hypogastrium and right iliac fossa, which extended into the right thigh as a massive dumb-bell tumour. On exploration, it was found not to be arising from any known retroperitoneal structure. The mass was excised, and the histopathology confirmed transitional cell carcinoma with positive margins. Though she received postoperative chemotherapy with cyclophosphamide, adriamycin and cisplatin, she developed extensive local recurrence and hepatic secondaries, and succumbed to the disease after ten months of follow-up. We highlight the rarity of the disease, its atypical presentation as a cystic dumb-bell lump, its diagnostic challenges and aggressive behaviour, and review the literature on primary retroperitoneal transitional cell carcinomas.

  19. Immature embryoid teratoma of the gall bladder: case of a primary primitive neoplasm.

    PubMed

    Naim, Mohammed

    2009-01-01

    This study presents diagnostic histopathological features of a primary embryoid teratomatous neoplasm in the gall bladder of a 60-year-old woman, and also discusses its pathogenesis. Sections revealed immature and typical embryoid bodies and dispersed syncytial trophoblasts along with mature and immature neuroectodermal and mesodermal elements. The residual endothelial lining of the gall bladder showed in situ and proliferative changes characteristic of an endodermal tumour. Ultrasonographic and magnetic resonance imaging findings of the rest of the abdominal and pelvic organs appeared normal. Results show that the mature adult gall bladder is susceptible to primary neoplasms of a primitive nature ranging from immature embryoma and teratoma to neuroectodermal tumour. Such primitive neoplasms in adult organs where benign neuroectodermal elements are present may evolve from a constituent cell of an adult organ acquired during embryogenesis from a morula that differentiated into trophoblastic (outer) and embryoblastic (inner) cells, and existing dormant cells at rest until reactivation during oncogenesis.

  20. [Incidence of primary malignant lesions in clinically benign teratoma: on the problem of adequate surgical procedure].

    PubMed

    Kindermann, G; Jung, E M; Maassen, V; Bise, K

    1996-08-01

    The Problem of an Adequate Surgical Approach: Frequency of malignant teratomas is, according to the literature, 2%-10%. Examining 194 own cases (1983-1993) it was 1.5%. We found one squamous cell carcinoma (0.5%). Additionally we found 2 immature teratomas (1%). We point out the different biological behaviour of malignant mature teratomas and immature teratomas. We agree with the majority of authors that the method of choice is the intact removal of all teratomas without iatrogen rupture or contamination of the abdominal cavity by contents of the teratoma. This adequate surgical procedure can and should be performed by laparotomy or laparoscopy with endobag. The often practised method of cutting open the cyst during laparoscopy, sucking off the contents or cutting the teratoma into pieces, has been proven to lead to implantation and worsening the prognosis in case of a malignant teratoma. Even the rinsing of the abdominal cavity, usually carried out with this method, could not compensate always for the disadvantage of this "dirty" endoscopical method compared with usual oncological standards. This is pointed out by case reports in the literature and the first analysis of a German survey with early-follow-up of 192 laparoscopically managed ovarian malignancies [11a]. The principle of intact removal of every teratoma should again be kept in mind.

  1. Primary Retroperitoneal Melanoma Presented in a Rare Extracutaneous Site for Malignant Melanoma.

    PubMed

    Alsharedi, Mohamed; Zgheib, Nadim Bou; Khelfa, Yousef; Raufi, Ali; Elmsherghi, Nabiha; Lebowicz, Yehuda

    2016-09-05

    Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature.

  2. Primary retroperitoneal myxoid/round cell liposarcoma is a nonexisting disease: an immunohistochemical and molecular biological analysis.

    PubMed

    de Vreeze, Ronald S A; de Jong, Daphne; Tielen, Ivon H G; Ruijter, Henrique J; Nederlof, Petra M; Haas, Rick L; van Coevorden, Frits

    2009-02-01

    Almost all primary retroperitoneal liposarcomas can be classified as well-/dedifferentiated liposarcoma. Rarely, however, primary retroperitoneal liposarcoma is classified as myxoid/round cell liposarcoma, based on the presence of myxoid areas and vascular crow's feet pattern, which has resulted in a debate on the classification of liposarcoma in the retroperitoneum. Genetically, myxoid/round cell liposarcoma and well-/dedifferentiated liposarcoma are different diseases. Myxoid/round cell liposarcoma is characterized by a translocation causing FUS-CHOP or EWSR1-CHOP fusion, whereas well-/dedifferentiated liposarcoma is characterized by an amplification of the 12q13-15 region, including MDM2 and CDK4 genes. As myxoid/round cell liposarcoma is highly radio- and chemosensitive, differentiation between subtypes is important to optimize treatment. We studied whether primary retroperitoneal liposarcomas diagnosed as myxoid/round cell liposarcoma represent molecularly true myxoid/round cell liposarcoma or are histopathological mimics and represent well-/dedifferentiated liposarcoma. Primary retroperitoneal myxoid/round cell liposarcoma (n=16) were compared to primary extremity myxoid/round cell liposarcoma (n=20). Histopathological and immunohistochemical features were studied. Amplification status of the 12q13-15 region was studied using a multiplex ligation-dependent probe amplification analysis, and FUS-CHOP or EWS-CHOP translocations were studied using RT-PCR. In primary retroperitoneal myxoid/round cell liposarcoma, MDM2 and CDK4 staining was both positive in 12 of 15 cases. In primary extremity myxoid/round cell liposarcoma, MDM2 was negative in 18/20 and CDK4 was negative in all cases. Multiplex ligation-dependent probe amplification showed the amplification of 12q13-15 region in 16/16 primary retroperitoneal myxoid/round cell liposarcomas and in 1/20 primary extremity myxoid/round cell liposarcomas. Translocation was present in all (18/18) primary extremity myxoid

  3. Retroperitoneal inflammation

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/001255.htm Retroperitoneal inflammation To use the sharing features on this page, please enable JavaScript. Retroperitoneal inflammation is swelling that occurs in the retroperitoneal space. ...

  4. Cross-sectional Imaging Features of Primary Retroperitoneal Tumors and Their Subsequent Treatment

    PubMed Central

    Acar, Turker; Harman, Mustafa; Guneyli, Serkan; Gemici, Kazim; Efe, Duran; Guler, Ibrahim; Yildiz, Melda

    2015-01-01

    Basically malignant tumors in the retroperitoneal region arise from a heterogeneous group of tissues: mesodermal, neurogenic, germ cell, and lymphoid. Although rare, benign tumors and cystic masses can be also encountered in retroperitoneal space. Developments in computed tomography (CT) and magnetic resonance imaging (MRI) have contributed to both diagnosis and staging of the retroperitoneal tumors. High spatial resolution and superiority in calcification make CT indispensable; on the other hand, MRI has a better soft-tissue contrast resolution which is essential for the assessment of vascular invasion and tissue characterization. The aim of this article is to review the CT and MRI features of retroperitoneal tumors and their subsequent management. PMID:25973288

  5. Mutations of the KIT gene and loss of heterozygosity of the PTEN region in a primary malignant melanoma arising from a mature cystic teratoma of the ovary.

    PubMed

    Tate, Genshu; Tajiri, Takuma; Suzuki, Takao; Mitsuya, Toshiyuki

    2009-04-01

    A tumor suppressor gene at 10q23.3, designated PTEN, encoding a dual-specificity phosphatase with lipid and protein phosphatase activity, has been shown to play a pivotal role in the pathogenesis of a variety of human cancers. A frequent loss of heterozygosity (LOH) at 10q is found in melanoma; however, little is known about the role of PTEN in the pathogenesis of a primary malignant melanoma derived from ovarian mature cystic teratoma, which is an extremely rare melanoma. This study examined the genetic alterations involved in the mitogen-activated protein kinase and phosphatidylinositol 3 kinase pathways in an ovarian malignant melanoma. A LOH analysis revealed hemizygous deletion around and in the PTEN gene not only in the ovarian melanoma but also in a mature cystic teratoma. Another case of ovarian mature cystic teratomas in the absence of melanoma also showed allelic loss of the PTEN region. To date, mutations of BRAF, NRAS, and KIT genes have been reported in malignant melanomas. In the present study, D816H and K558E mutations of the KIT gene were revealed in the melanoma arising from a mature cystic teratoma, but not in a mature cystic teratoma. No mutations of the BRAF and NRAS genes were found in the melanoma. These results indicate that LOH of the PTEN region is one of the molecular alterations of an ovarian mature cystic teratoma and a KIT mutation is an additional promotional event associated with the oncogenesis of a melanoma arising from an ovarian mature cystic teratoma.

  6. 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.

  7. “Looks Can Be Deceiving”: Adrenal Teratoma Causing Diagnostic Difficulty

    PubMed Central

    Nadeem, Mehwash; Ather, Muhammad Hammad; Sulaiman, M. Nasir

    2015-01-01

    Teratomas are unusual tumours that derived from totipotent cells with their origin from more than one or usually all three germ cells. Here authors are presenting a case of primary retroperitoneal tumour that is a rare clinical entity. A 19-year-old male presented with right lumbar pain and was found to have complex cyst with large calcification in right adrenal gland on imaging. Intraoperatively, he was found to have a solid mass with areas of soft consistency, which was excised en bloc. On gross examination, the cyst contained pieces of bone, few teeth, and hairs entangled in mucinous material. On histological evaluation, it was confirmed to be mature teratoma arising from the right adrenal gland. He made uneventful recovery and was kept well on annual follow-up. PMID:26788398

  8. [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.

  9. [Surgery of retroperitoneal pheochromocytoma].

    PubMed

    Duff, C; van Segesser, L; Schmid, E R; Ziegler, W; Turina, M

    1989-06-01

    Retroperitoneal pheochromocytomas are very uncommon tumors. During the last 10 years only 3 cases have been operated on in our clinic. Two of them were primary retroperitoneal paragangliomas. These two patients are now well and without recurrence 1 and 10 years respectively after operation. The third patient had retroperitoneal metastases of an adrenal pheochromocytoma. Now, after 1 year he has disseminated metastases without response to therapy with (131I)metaiodobenzylguanidine (MIBG). The surgical procedure includes often extended resection and replacement of retroperitoneal blood vessels and requires therefore an experienced surgical team and optimal anesthetic management (Swan-Ganz-katheter). In two cases the aorta was resected and replaced with a Dacron-graft. Reimplantation of both renal arteries into the graft was necessary in one case. Further interventions were: nephrectomy (2x), resection and ligation of the inferior vena cava (1x), resection and replacement of the left renal vein (1x).

  10. Pediatric Gastric Teratoma

    PubMed Central

    Valenzuela-Ramos, Marco Cesar; Mendizábal-Méndez, Ana Luisa; Ríos-Contreras, Carlos Alberto; Rodríguez-Montes, Claudia Esther

    2010-01-01

    Neoplasms from germ cell origin are a heterogeneous group of tumors rarely seen in the pediatric population, teratoma is the most frequent among them. They can occur in either gonadal or extragonadal locations. Extragonadal teratoma arising from abdominal viscera is very unusual. There are less than a hundred reported cases of gastric teratoma in the worldwide literature. Since the occurrence of this pathology in the pediatric age group is quite rare, we describe a case of a teratoma located in the lesser curvature of the stomach in an infant with an emphasis in radiologic-pathologic correlation. PMID:22470691

  11. Postchemotherapy Retroperitoneal Lymph Node Dissection in Patients With Nonseminomatous Testicular Cancer: A Single Center Experiences

    PubMed Central

    Nowroozi, Mohamadreza; Ayati, Mohsen; Arbab, Amir; Jamshidian, Hassan; Ghorbani, Hamidreza; Niroomand, Hassan; Taheri Mahmoodi, Mohsen; Amini, Erfan; Salehi, Sohrab; Hakima, Hamid; Fazeli, Farid; Haghdani, Saeid; Ghadian, Alireza

    2015-01-01

    Background: Testicular cancer accounts for about 1 - 1.5% of all malignancies in men. Radical orchiectomy is curative in 75% of patients with stage I disease, but advance stage with retroperitoneal lymph node involvement needs chemotherapy. All patients who have residual masses ≥ 1 cm after chemotherapy should undergo postchemotherapy retroperitoneal lymph node dissection (PC-RPLND). Objectives: Treatment of advanced nonseminomatous testicular cancer is usually a combination of chemotherapy and surgery. We described our experience about postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) in our center. Patients and Methods: In a retrospective cross-sectional study between 2006 and 2011, patients with a history of postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) in Imam Khomeini hospital were evaluated. All patients had normal postchemotherapy serum tumor markers and primary nonseminomatous cancer. We reviewed retrospectively clinical, pathological, and surgical parameters associated with PC-RPLND in our center. Results: Twenty-one patients underwent bilateral PC-RPLND. Mean age was 26.3 years (ranged 16 - 47). Mean size of retroperitoneal mass after chemotherapy was 7.6 cm. Mean operative time was 198 minutes (120 - 246 minutes). Mean follow-up time was 38.6 months. Pathologic review showed presence of fibrosis/necrosis, viable germ cell tumor and teratoma in 8 (38.1%), 10 (47.6%) and 3 (14.28%) patients, respectively. One patient in postoperative period of surgery and three patients in two first years after surgery were expired. Of 17 alive patients, only two (11.8%) had not retrograde ejaculation. Conclusions: PC-RPLND is one the major operations in the field of urology, which is associated with significant adjunctive surgeries. In appropriate cases, PC-RPLND was associated with good cancer specific survival in tertiary oncology center. PMID:26539420

  12. [Retroperitoneal fibrosis].

    PubMed

    Babski, Paweł; Wojtuń, Stanisław; Gil, Jerzy

    2007-05-01

    Retroperitoneal fibrosis is a rare clinical entity characterised by the presence of patologic collagen tissue in a retroperitoneal space. The fibrous mass covers abdominal organs causing their disfunctions. RPF was described at the begining of XX century but its etiology is not clear yet. Usually it causes an ureter obstuction and hydronephrosis, that is why most commonly is diagnosed by urologists and nephrologists. However, retroperitoneal fibrosis can be multifacial disease. In some patients localisation of fibrosis is atypical and manifestationns can be varied. Gastrological symptoms like jaundice, bowel obstuction, ascites can occure. Besides, some early signs of RPF are nonspecific and can imitate alarming symptoms of neoplasma, e.g.: weight loss, anemia, malaise, anorexia, fever. This force us to initiate gastrological investigation. The awareness of this disease is important. The early diagnosis and treatment improves prognosis and alows to avoid heavy complications. In typical cases radiology is often enough for diagnosis. However, histological examination is needed in many cases, especialy when patological mass is located atypical. A treatment is made up of farmacology and surgery. The first one is based on steroids, immunossuppressant and tamoxifen. Surgery is needed to eliminate organs obstruction.

  13. Detection of vital germ cell tumor cells in short-term cell cultures of primary tumors and of retroperitoneal metastasis--clinical implications.

    PubMed

    Otto, T; Virchow, S; Fuhrmann, C; Steinberg, F; Streffer, C; Goepel, M; Rübben, H

    1997-01-01

    By establishing short-term cell cultures derived from retroperitoneal metastasis after neoadjuvant chemotherapy, our aim was to improve the diagnosis and prognosis in patients with advanced testicular germ cell tumors. The histological evaluation of surgically removed metastatic tissue by retroperitoneal lymphadenectomy (RLA) is extremely complicated after previous chemotherapy, but knowledge of persistence of vital tumor cells in residual lesions is of great prognostic value and therapeutic consequence in patients with testicular germ cell tumors. We therefore investigated whether vital tumor tissue could be detected in short-term cell cultures derived from such metastatic lesions by measuring the concentration of the tumor markers beta human chorionic gonadotropin (beta HCG) and alpha-1 fetoprotein (AFP) in cell culture supernatants. We initially demonstrated the specificity of the determination in cell cultures of human transitional-cell carcinoma cell lines, human foreskin fibroblasts and normal testicular tissue. In a group of 20 patients with untreated primary testicular germ cell tumors, detection of beta HCG and AFP was increased about threefold in cell culture supernatants in comparison to the serum concentration. Finally, we prepared primary cell cultures from surgically removed retroperitoneal metastasis of 12 patients with testicular germ cell tumors after chemotherapy. The serum concentrations of beta HCG and AFP of all patients were at normal values when RLA was performed. However, pathologically increased concentrations of beta HCG (3/3) and AFP (2/3) in cell culture supernatants were found in 3 of 12 cell cultures. Interestingly, these three patients with a pathological increase in beta HCG and AFP as determined in the supernatant of the short-term cell cultures had tumor progression within a mean follow-up of 3 +/- 1 months (P < 0.01), whereas 9 of 12 patients who had no pathological increase in beta HCG and AFP as determined in the supernatant of

  14. Genetics Home Reference: retroperitoneal fibrosis

    MedlinePlus

    ... 1 link) Retroperitoneal fibrosis Educational Resources (3 links) Disease InfoSearch: Retroperitoneal fibrosis MalaCards: retroperitoneal fibrosis Orphanet: IgG4-related retroperitoneal fibrosis Patient Support and Advocacy Resources (2 ...

  15. Congenital Orbital Teratoma

    PubMed Central

    Pellerano, Fernando; Guillermo, Elvis; Garrido, Gloreley; Berges, Pedro

    2017-01-01

    We report a case of congenital orbital teratoma. A 3-day-old male, born at 39 weeks’ gestation without relevant prenatal history, presented with a large vascularized proptotic mass distorting the left midface. Laboratory studies showed elevated serum alpha-fetoprotein (12,910 ng/ml). Computed tomography showed a multiloculated heterogeneous lesion composed of hypodense and hyperdense calcified areas encompassing the whole orbital cavity with expansion of the bony walls, as well as forward displacement and compression of the eyeball without extension to surrounding structures. Clinical, imaging and laboratory features were consistent with congenital orbital teratoma. Due to pronounced proptosis with exposure keratopathy and corneal perforation, no motility of the globe and no vision in the affected eye in a resource-limited setting, the patient underwent orbital exenteration. Histopathological examination confirmed the diagnosis of mature cystic teratoma. We describe the clinical course, radiographic and histopathological findings of this rare orbital tumor. PMID:28275597

  16. Congenital Orbital Teratoma.

    PubMed

    Pellerano, Fernando; Guillermo, Elvis; Garrido, Gloreley; Berges, Pedro

    2017-01-01

    We report a case of congenital orbital teratoma. A 3-day-old male, born at 39 weeks' gestation without relevant prenatal history, presented with a large vascularized proptotic mass distorting the left midface. Laboratory studies showed elevated serum alpha-fetoprotein (12,910 ng/ml). Computed tomography showed a multiloculated heterogeneous lesion composed of hypodense and hyperdense calcified areas encompassing the whole orbital cavity with expansion of the bony walls, as well as forward displacement and compression of the eyeball without extension to surrounding structures. Clinical, imaging and laboratory features were consistent with congenital orbital teratoma. Due to pronounced proptosis with exposure keratopathy and corneal perforation, no motility of the globe and no vision in the affected eye in a resource-limited setting, the patient underwent orbital exenteration. Histopathological examination confirmed the diagnosis of mature cystic teratoma. We describe the clinical course, radiographic and histopathological findings of this rare orbital tumor.

  17. Mature Teratoma at Left Lumbar Region in an Infant: A Case Report

    PubMed Central

    2016-01-01

    Common location of teratomas in children are sacrococcygeal, gonadal, mediastinal and retroperitoneal, but teratomas may also occur at very unusual locations. A six-month-old boy presented with a large swelling at his left lumbar region. He presented with gradually increasing lump at his back, involving more on left lumbar region and midline since birth. Clinical examination revealed a solitary, non-tender, soft to firm, irregular mass, occupying more on his left lumbar and midline region and also crossing the midline. Radiological investigations revealed a well defined mass of 9.6 x 7.5cm, with bony elements and spina bifida at L1 and L2 levels. His Computed Tomography (CT) scan finding was consistent with mature teratoma. Complete surgical excision of the tumour was done without any difficulties. Histology of the excised tumour was conclusive of mature teratoma. His post-operative period was excellent, but he lost to follow-up after discharge. PMID:28208935

  18. Rare Skin Adnexal and Melanocytic Tumors Arising in Ovarian Mature Cystic Teratomas: A Report of 3 Cases and Review of the Literature.

    PubMed

    Moulla, Alexandra A; Magdy, Nesreen; Francis, Nicholas; Taube, Janis; Ronnett, Brigitte M; El-Bahrawy, Mona

    2016-09-01

    Mature teratoma of the ovary is the most common primary ovarian tumor accounting for 15% (10%-20%) of all ovarian neoplasms. Skin and skin adnexal structures are the most common elements identified in mature teratomas. Benign and malignant skin tumors can arise in ovarian teratomas, the most common being epithelial tumors. Melanocytic and adnexal tumors developing in a teratoma are rare and can be easily overlooked. We report 3 cases and review melanocytic and skin adnexal tumors encountered in ovarian teratomas.

  19. Giant mature teratoma in the mediastinum presenting with rapid growth

    PubMed Central

    Fujita, Kohei; Hayashi, Kazuki; Motoishi, Makoto; Sawai, Satoru; Terashima, Tsuyoshi; Mio, Tadashi

    2016-01-01

    Teratomas are primary germ-cell tumours in the mediastinum. Although they are generally slow-growing and asymptomatic, rapid growth causing life-threatening complications can occur. Sebaceous secretion, insulin production, chorionic gonadotropin secretion and pancreatic enzyme secretion are the presumptive causes of tumour progression. Only few cases of rapidly growing teratomas have been reported previously. Here, we present a case of a giant mature teratoma in the mediastinum that presented with rapid growth and compare the characteristics of this case with those of previous cases. PMID:28031857

  20. Extragonadal omental teratoma: a case report.

    PubMed

    Hegde, Prashanth

    2014-02-01

    Mature cystic teratomas or dermoid cysts are among the most common ovarian tumors; however, teratomas of extragonadal origin are extremely rare. The most common extragonadal site of these teratomas is the omentum. It is generally accepted that teratomas arise from germ cells that originate in the mature gonads. Of the three proposed causes of omental teratoma, auto-amputation and subsequent re-implantation of gonadal teratoma is the most likely preceding event. A review of the published reports reveals that only 31 cases of teratoma of the greater omentum have been published to date and three cases reported wherein omental teratoma and dermoid of the ovary were coexisting. We report a rare case of an omental teratoma in a 26-year-old woman who underwent ovarian cystectomy for dermoid cyst. This is the fourth case of an omental mature teratoma with coexisting ovarian dermoid cyst.

  1. Sacrococcygeal teratoma: case report.

    PubMed

    Molina Vital, Ricardo; de Santiago Valenzuela, José Martín; de Lira Barraza, Roberto Carlos

    2015-05-12

    We present a male newborn child with a sacrococcygeal mass who was sent to clinic 46 of the Mexican Social Security Institute located in Gomez Palacio, Durango, Mexico for pediatric/neonatal surgical resolution. The mass was detected on gestation week 24 in the sacrococcygeal area and was initially interpreted as a myelomeningocele. On gestation week 32, the mass had grown, so the diagnosis of cystic hygroma was posed. The child was born at 38 weeks of gestational age with a large tumor in the sacrococcygeal area. Images were obtained, and tumor resection was performed without complications. Pathologic examination confirmed the diagnosis of sacrococcygeal teratoma. The postoperative course was uneventful and there were no further complications.

  2. An Aggressive Retroperitoneal Fibromatosis

    PubMed Central

    Campara, Zoran; Spasic, Aleksandar; Aleksic, Predrag; Milev, Bosko

    2016-01-01

    Introduction: Aggressive fibromatosis (AF) is a heterogeneous group of mesenchymal tumors that have locally infiltrative growth and a tendency to relapse. The clinical picture is often conditioned by the obstruction of the ureter or small intestine. Diagnosis is based on clinical, radiological and histological parameters. A case report: We report a case of male patient, aged 35 years, with the retroperitoneal fibromatosis. He reported to the physician because of frequent urination with the feeling of pressure and pain. Computed tomography revealed the tumor mass on the front wall of the bladder with diameter of 70mm with signs of infiltration of the musculature of the anterior abdominal wall. Endoscopic transurethral biopsy showed proliferative lesion binders by type of fibromatosis. The tumor was surgically removed in a classical way. The patient feels well and has no recurrence thirty-six months after the operative procedure. Conclusion: The complete tumor resection is the therapeutic choice for the primary tumor as well as for a relapse. PMID:27147794

  3. [Retroperitoneal tumors: a case of liposarcoma].

    PubMed

    Cassinelli, G; Arena, E; Bronzino, P; Cuneo, A E; Partipilo, F; Rusca, I; Rassu, P C; Casaccia, M

    2002-01-01

    The Authors report a case of retroperitoneal sarcoma, in the histological subtype "liposarcoma", occasionally diagnosed in a 57 year old woman, who presented aspecific symptoms, characterized by dry cough, vomiting and chest pain. Chest TC showed a neoplasm of the retroperitoneum, close to liver, right colon, duodenum and right kidney. The arteriography of the right renal artery showed a neovascolarization inside the mass. A surgical resection was performed. The histological examination confirmed the diagnosis of liposarcoma. Liposarcomas account for 30% of the retroperitoneal soft tissue sarcomas; symptoms and diagnosis are late and the surgical resection of primary neoplasm, metastases and local recurrences representing the only therapeutic choice.

  4. Giant retroperitoneal sarcomas.

    PubMed

    Ianoşi, G; Neagoe, Daniela; Buteică, Elena; Ianoşi, Simona; Drighiciu, Corina; Stănoiu, B; Burada, F; Mercuţ, D

    2007-01-01

    Retroperitoneal sarcomas are rare malignant tumors, which are developing from mesenchymal stem cells residing in muscle, fat, and connective tissues. Underlying the rarity of this kind of tumors in general population, the aim of this paper is to present three cases of retroperitoneal sarcomas operated in Surgical Department of Military Hospital of Craiova (a retroperitoneal liposarcoma, a dedifferentiated liposarcoma and a malignant fibrous histiocytoma). From clinical point of view, we note the poverty of symptoms and non-specificity of these and a great tolerability of retroperitoneal space that offers the possibility for a great development of the tumor. CT-scan and MRI are the best investigations for diagnosis but surgical exploration is the best way for a good evaluation of these tumors. From histological point of view, we try to present new features about these kinds of tumors in order to classify them. Results of surgery correlated with complementary therapies were good without per-operative mortality or postoperative morbidity but we noted a recidive of one tumor (with different histological pattern) 21 months after the surgical intervention. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

  5. [Retroperitoneal germ cell tumor].

    PubMed

    Borrell Palanca, A; García Garzón, J; Villamón Fort, R; Domenech Pérez, C; Martínez Lorente, A; Gunthner, S; García Sisamón, F

    1999-03-01

    We report a case of retroperitoneal extragonadal germ-cell tumor in an 17 years old patient who presented with aedema and pain in left inferior extremity asociated with hemopthysis caused by pulmonar metastasis, who was treated with chemotherapy and resection of residual mass and pulmonary nodes. Dyagnosis was stableshed by fine neadle aspiration biopsy of the wass. We comment on the difficult of stableshing differential dyagnosis between retroperitoneal extragonadal germ-cell tumor and metastasis of a testicular tumor. Dyagnosis is stableshed by the finding of a histologically malignant germ-cell tumor with normal testis. We considered physical examination and ecographyc exploration enough for a correct dyagnosis.

  6. Fetal intracranial teratoma. A review.

    PubMed

    Isaacs, Hart

    2014-01-01

    A literature and institutional review of fetal intracranial teratomas yielded 90 tumors. The mean age at ultrasound diagnosis was 32 weeks, ranging from 21 to 41 weeks. Males and females were equally affected. The average, maximum tumor size was 10 cm, varying between 3.5 and 23 cm. Forty-two percent of patients died within the first week of life. Death rate was exceptionally high before 30 weeks gestation where almost half the affected fetuses expired. The overall survival rate for 90 fetuses with intracranial teratoma was only 7.8%.

  7. Growing teratoma syndrome: first case report in a 4-year-old girl.

    PubMed

    Daher, Paul; Riachy, Edward; Khoury, Antoine; Raffoul, Lara; Ghorra, Claude; Rehayem, Caline

    2015-02-01

    Growing teratoma syndrome (GTS) consists of germ cell tumors that grow following chemotherapy despite complete eradication of the malignant cells. They can metastasize to any site, particularly the retroperitoneum, mediastinum and cervical region. It typically affects young adults and adolescents. Here we describe the youngest case reported in a 4-year-old girl with an ovarian mixed germ cell tumor who underwent an oophorectomy. Her tumor markers normalized by the end of her chemotherapeutic treatment; however, she developed a retroperitoneal mass that was subsequently resected. Histopathology revealed a mature teratoma, consisting of a GTS. We stress the need for early recognition and treatment of GTS to avoid the subsequent morbidity and mortality associated with it. Although GTS has an excellent prognosis when completely resected, it is essential that the patient be regularly followed-up with serum tumor markers and imaging.

  8. Congenital Orbital Teratoma with Unilateral Proptosis.

    PubMed

    Gulzar, Rubina; Shahid, Ruqaiya; Mirza, Talat

    2017-03-01

    Teratoma is a rare tumor, containing structures originating from all three germinal layers. The most frequent location of teratoma is the gonads. Orbital teratoma is extremely rare. In 1969, Jensen reported that only 40 cases of orbital teratoma existed in the world. We describe a rare case of orbital teratoma in a 15-day girl who presented with marked proptosis of the left eye. The eyeball was embedded within the mass that could be seen all around the globe. Orbitectomy was performed with the impression of retinoblastoma. Histopathological examination was reported as benign mature cystic teratoma; no immature component was identified. The case is being reported because of its rarity and also to highlight the use of modern neuroimaging techniques in making an accurate preoperative diagnosis, which helps in better operative management of these patients.

  9. Sacrococcygeal Teratoma in Nigerian Children

    PubMed Central

    Williams, A. Olufemi; Lagundoye, S. B.; Bankole, M. A.

    1970-01-01

    Two-thirds of all teratomas seen in 31 Nigerian children at Ibadan were situated in the sacrococcygeal region. About one-quarter of all the sacrococcygeal teratomas were malignant as shown on radiological and pathological examinations. The malignant tumours had papillary carcinomatous pattern, probably derived from the choroid plexus. The clinicopathological features of one patient with malignant change is described to illustrate the biological behaviour of this tumour. Malignancy in all the sacrococcygeal tumours appears to be associated with growth of the tumour into the pelvic cavity and metastasis initially to inguinal lymph nodes and later to distant sites. Inferior venacavogram can be of great value in defining the extent of the intrapelvic mass and the displacement of the genito-urinary organs, particularly when surgery is contemplated. ImagesFig. PMID:5440177

  10. [Diagnostic-therapeutic approach for retroperitoneal tumors].

    PubMed

    Cariati, A

    1993-12-01

    After a careful review of the Literature, diagnostic and therapeutic strategies for Primary Retroperitoneal Tumours (PRT) are reported. The Author analyzes the experience of the Institute of Clinica Chirurgica "R" (Chief: Prof. E. Tosatti) as well as that of Anatomia Chirurgica (Chief: Prof. E. Cariati),--University of Genoa--in the management of PRT, stressing the importance of preoperative staging for a correct surgical approach.

  11. More Cases of Benign Testicular Teratomas are Detected in Adults than in Children. A Clinicopathological Study of 543 Testicular Germ Cell Tumor Cases.

    PubMed

    David, Semjén; András, Farkas; Endre, Kalman; Balint, Kaszas; Árpad, Kovács; Csaba, Pusztai; Karoly, Szuhai; Tamás, Tornóczky

    2016-10-28

    Benign testicular teratomas are always thought to be pediatric neoplasms and previously all the teratoid tumors in the adult testis regarded as malignant. Recently, three publications reported benign testicular teratomas in adulthood and the latest WHO classification refers them as "prepubertal type of teratomas" which rarely appear in adulthood. These neoplasms behave benign and seemingly analogous independently whether they appear in pre- or postpubertal patients. The aim of our study was to investigate the frequency of benign testicular teratomas both in children and adults. 593 cases of testicular neoplasms were found in a period of 17 years ranging from 1998 to 2014 in the archive of our department (Department of Pathology, Medical Center, Pécs University). 543 cases diagnosed as germ cell tumor which have all been further evaluated in conjunction with the clinical data available. Of all germ cell tumor cases 14 (2.5 %) were pure teratomas. Ten out of 14 were the WHO-defined "conventional" teratoma, 4 of the 14 were the "benign or the so called prepubertal type" from which three occurred in adult patients. Only one of the 14 occurred in childhood, indicating that benign prepubertal type teratomas -which are regarded generally as childhood tumors- are more frequently detected in adults than in children. Benign adult testicular teratomas comprised 21 % of all pure teratoma cases in our series. Practicioners in the field have to be aware of its existence also in adulthood to avoid overtreatment and not to expose their patients to unnecessary chemotherapy, retroperitoneal lymphadenectomy (RLA) and the potential complications of these interventions.

  12. Non-pancreatic retroperitoneal mucinous neoplasms and a discussion of the differential diagnosis

    PubMed Central

    Cobb, Camilla; Raza, Anwar Sultana

    2016-01-01

    Retroperitoneal mucinous neoplasms are common and have a wide range of differential diagnoses to consider. It is of utmost importance to maintain a multidisciplinary approach when evaluating these lesions. Clinical history, surgical impression and radiographic studies should be obtained and used in conjunction with morphology and immunohistochemistry to help guide the pathologist to the correct diagnosis. We recently encountered a case of a 51-year-old man who presented with an 11.5 cm left-sided retroperitoneal mucinous neoplasm. This lesion was initially diagnosed as a low-grade mucinous carcinoma of probable pancreatic origin at an outside facility, and he was subsequently treated with chemotherapy. One year later he presented to our institution and underwent retroperitoneal en bloc resection of the 7-cm residual multiloculated mucinous neoplasm. When discussed at tumor board additional medical history was obtained, and review of the patient’s chart revealed a remote history of left orchiectomy for a mixed malignant germ cell tumor and metastatic embryonal carcinoma in 2 of 34 retroperitoneal/para-aortic lymph nodes. With no clinical evidence of tumor in the pancreas or extension from it, the predominately para-aortic location of the tumor favors a mucinous carcinoma arising from a rest of mature metastatic/proliferating teratoma that persisted after chemotherapy. This case illustrates the importance of a thorough history, however remote, and correlation with imaging in the development of differential diagnoses, as well as, the need to consider non-pancreaticobiliary sources of retroperitoneal mucinous tumors. PMID:27034817

  13. Relationship between primary and metastatic testicular germ cell tumors: a clinicopathologic analysis of 100 cases.

    PubMed

    Tarrant, William P; Czerniak, Bogdan A; Guo, Charles C

    2013-10-01

    Testicular germ cell tumors (GCTs) commonly metastasize to the retroperitoneal lymph nodes (RPLNs). We evaluated 100 cases of RPLN dissection specimens with viable GCTs after chemotherapy and compared them with their corresponding orchiectomy specimens. The mean age of patients was 28 years (range, 15-58 years). The testicular tumors consisted of mixed GCT (n = 72), teratoma (n = 18), seminoma (n = 4), embryonal carcinoma (n = 3), yolk sac tumor (n = 1), and no viable tumor (n = 2). Somatic malignant components were found in 5 cases. The metastatic tumors in the RPLNs consisted of only teratoma (n = 77) and non-teratomatous GCT (n = 23). Twenty-one patients had only teratoma in the RPLNs but not in the testis, and 10 patients had metastatic non-teratomatous GCT components that were not observed in the testis. Six patients had somatic malignant components in the RPLNs, but only one of them had such a component in the testis. Overall, 13 patients died of disease in a mean of 42 months, and the patients with only teratoma in the RPLNs had a lower mortality rate (9%) than those with non-teratomatous components (26%) (P = .044). One patient with somatic components in the primary GCT and 3 patients with somatic components in the metastases died of disease. Our study demonstrates that there is frequent discordance of histologic composition between primary and metastatic testicular GCTs. Teratoma is the most common component in treated GCTs and is usually associated with a more favorable clinical outcome than non-teratomatous GCTs. The presence of somatic components in the RPLNs metastasis indicates a poor prognosis.

  14. Robot - assisted laparoscopic retroperitoneal lymph node dissection in testicular tumor

    PubMed Central

    Torricelli, Fabio C. M.; Jardim, Denis; Guglielmetti, Giuliano B.; Patel, Vipul; Coelho, Rafael F.

    2017-01-01

    ABSTRACT Introduction and objective Retroperitoneal lymph node dissection (RPLND) is indicated for patients with non-seminomatous germ cell tumor (NSGCT) with residual disease after chemotherapy. Although the gold standard approach is still the open surgery, few cases of robot-assisted laparoscopic RPLND have been described. Herein, we aim to present the surgical technique for robot-assisted laparoscopic RPLND. Patient and method A 30 year-old asymptomatic man presented with left testicular swelling for 2 months. Physical examination revealed an enlarged and hard left testis. Alpha-fetoprotein (>1000ng/mL) and beta-HCG (>24.000U/L) were increased. Beta-HCG increased to >112.000U/L in less than one month. The patient underwent a left orchiectomy. Pathological examination showed a mixed NSGCT (50% embryonal carcinoma; 30% teratoma; 10% yolk sac; 10% choriocarcinoma). Computed tomography scan revealed a large tumor mass close to the left renal hilum (10x4x4cm) and others enlarged paracaval and paraortic lymph nodes (T2N3M1S3-stage III). Patient was submitted to 4 cycles of BEP with satisfactory response. Residual mass was suggestive of teratoma. Based on these findings, he was submitted to a robot-assisted RPLND. Results RPLND was uneventfully performed. Operative time was 3.5 hours. Blood loss was minimal, and there were no intra- or postoperative complications. The patient was discharged from hospital in the 1st postoperative day. Pathological examination showed a pure teratoma. After 6 months of follow-up, patient is asymptomatic with an alpha-fetoprotein of 2.9ng/mL and an undetectable beta-HCG. Conclusion Robot-assisted laparoscopic RPLND is a feasible procedure with acceptable morbidity even for post chemotherapy patients when performed by an experienced surgeon.

  15. [Radiotherapy for retroperitoneal sarcomas].

    PubMed

    Sargos, P; Stoeckle, E; Henriques de Figueiredo, B; Antoine, M; Delannes, M; Mervoyer, A; Kantor, G

    2016-10-01

    The management of retroperitoneal sarcoma can be very challenging, and the quality of initial treatment strategy appears to be a crucial prognostic factor. En bloc surgery is currently the standard of care for these rare tumours and perioperative treatments such as chemotherapy or radiotherapy have not been validated yet. However, local-regional relapse constitutes the most common disease course. While adjuvant radiotherapy is less and less common due to gastrointestinal toxicities, preoperative radiation therapy offers numerous advantages and is being evaluated as part of a national multicentre phase II study (TOMOREP trial) and is the subject of a European randomized phase III study (STRASS trial). The objective of this article is to present data on preoperative irradiation in terms of dose, volumes and optimal radiotherapy techniques for the treatment of this rare disease.

  16. Percutaneous Radiofrequency Ablation for Treatment of Recurrent Retroperitoneal Liposarcoma

    SciTech Connect

    Keil, Sebastian Bruners, Philipp; Brehmer, Bernhard; Mahnken, Andreas Horst

    2008-07-15

    Percutaneous CT-guided radiofrequency ablation (RFA) is becoming more and more established in the treatment of various neoplasms, including retroperitoneal tumors of the kidneys and the adrenal glands. We report the case of RFA in a patient suffering from the third relapse of a retroperitoneal liposarcoma in the left psoas muscle. After repeated surgical resection and supportive radiation therapy of a primary retroperitoneal liposarcoma and two surgically treated recurrences, including replacement of the ureter by a fraction of the ileum, there was no option for further surgery. Thus, we considered RFA as the most suitable treatment option. Monopolar RFA was performed in a single session with a 2-cm umbrella-shaped LeVeen probe. During a 27-month follow-up period the patient remained free of tumor.

  17. Effective treatment for malignant mediastinal teratoma.

    PubMed Central

    Parker, D; Holford, C P; Begent, R H; Newlands, E S; Rustin, G J; Makey, A R; Bagshawe, K D

    1983-01-01

    Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series. Images PMID:6198739

  18. Angiography of nonneoplastic retroperitoneal masses

    SciTech Connect

    Lois, J.F.; Levin, D.C.; Hooshmand, I.

    1982-01-01

    Although noninvasive imaging modalities can be used to initially detect retroperitoneal masses, angiography is still desirable for several reasons in those cases in which surgery is contemplated. The latter can forewarn the surgeon as to possible hemorrhagic complications in highly vascular lesions. In some cases it can predict the malignant potential of the lesion. Finally, since these masses may derive blood supply from multiple sources, a vascular ''road map'' is provided to the surgeon. The angiographic findings of malignant retroperitoneal tumors are well known, but very little has been published dealing with benign nonneoplastic retroperitoneal masses. We have performed angiography in 11 surgically proven nonneoplastic retroperitoneal masses (9 inflammatory lesions, 2 hematomas). Major arterial or renal displacement occurred in 10. The 2 hematomas and 3 inflammatory lesions were totally avascular; 6 of the 9 inflammatory lesions revealed fine neovascularity and 5 of these 6 had an associated capillary blush. Sources of vascular supply included the lumbar, renal capsular, superior mesenteric, and gastroduodenal arteries. Although angiography is helpful in preoperative evaluation of retroperitoneal masses for the aforementioned reasons, it may be difficult or impossible to differentiate benign from malignant lesions based on the angiographic findings alone.

  19. Retroperitoneal liposarcoma: a comprehensive review.

    PubMed

    Vijay, Adarsh; Ram, Lakshmi

    2015-04-01

    Retroperitoneal liposarcomas are rare mesenchymal tumors of the retroperitoneum that typically present with advanced disease and often carry a poor prognosis. Because of their rarity and anatomic location, these malignant tumors can cause a diagnostic dilemma and present several therapeutic challenges. They are usually associated with a high rate of recurrence despite grossly complete resection, thus requiring long-term and often indefinite follow-up. Relevant data on this topic was procured and synthesized with the aid of a comprehensive Medline search in addition to oncologic, pathologic, urologic, radiologic, and surgical literature review on retroperitoneal sarcomas. This article provides an in-depth review into the natural history, pathology, clinical manifestations, and prognostic features of retroperitoneal liposarcomas. It also discusses the reliability of diagnostic procedures and novel curative approaches that are currently being evaluated for the disease.

  20. Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas

    SciTech Connect

    Serizawa, Itsuko; Kagei, Kenji; Kamada, Tadashi; Imai, Reiko; Sugahara, Shinji; Okada, Tohru; Tsuji, Hiroshi; Ito, Hisao; Tsujii, Hirohiko

    2009-11-15

    Purpose: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control. Methods and Materials: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT. Age ranged from 16 to 77 years (median, 48.6 years). Of the patients, 16 had primary disease and 8 recurrent disease. Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients. The histologic grades were as follows: Grade 3 in 15, Grade 2-3 in 2, Grade 2 in 3, and unknown in 4. Clinical target volumes ranged between 57 cm{sup 3} and 1,194 cm{sup 3} (median 525 cm{sup 3}). The delivered carbon ion dose ranged from 52.8 to 73.6 GyE in 16 fixed fractions over 4 weeks. Results: The median follow-up was 36 months (range, 6-143 months). The overall survival rates at 2 and 5 years were 75% and 50%, respectively. The local control rates at 2 and 5 years were 77% and 69%. No complications of the gastrointestinal tract were encountered. No other toxicity greater than Grade 2 was observed. Conclusions: Use of CIRT is suggested to be effective and safe for retroperitoneal sarcomas. The results obtained with CIRT were a good overall survival rate and local control, notwithstanding the fact that most patients were not eligible for surgical resection and had high-grade sarcomas.

  1. TRALI Syndrome Complicated by Retroperitoneal Bleeding.

    PubMed

    Singh, Vijay A; Zeltsman, David

    2011-09-01

    Transfusion-related acute lung injury (TRALI) is an underdiagnosed and underreported syndrome which by itself is the third leading cause of transfusion-related mortality. The incidence of TRALI is reported to be 1 in 2000 to 5000 transfusions. When combined with uncontrollable bleeding, survival is unachievable. We report the case of a 25-year-old man, who underwent open heart surgery as an infant to correct his congenital heart disease in association with right pulmonary artery atresia. He presented with hemoptysis secondary to aspergilloma and required a pneumonectomy of the nonfunctional right lung. During pneumolysis, significant bleeding occurred from the superior vena cava. The patient required a blood transfusion and was placed on cardiopulmonary bypass to control the bleeding. Simultaneous occurrence of severe pulmonary edema and retroperitoneal bleeding were noted. Approximately 8 L of frothy edema fluid were drained from the only functional left lung starting ~15 minutes after the transfusion and lasting for several hours until the end of the case. It most likely represented TRALI syndrome. Increasing abdominal girth and poor volume return to the pump were consistent with and pathognomonic for retroperitoneal bleeding. Though primary surgical bleeding in the chest was controlled successfully and a pneumonectomy performed without further difficulty, we were unable to separate the patient from cardiopulmonary bypass due to the inability to oxygenate. As a result, we could not reverse the anti-coagulation which potentially exacerbated the retroperitoneal bleeding. After multiple unsuccessful attempts the patient succumbed. This ill-fated case demonstrates the quandary of obtaining vascular access for emergency cardiopulmonary bypass while in the right thoracotomy position. It may be beneficial to have both the femoral artery and vein cannulated before positioning a patient in a lateral decubitus position. In addition, early direct access to the right atrium

  2. Cleft palate caused by congenital teratoma.

    PubMed

    Veyssière, Alexis; Streit, Libor; Traoré, Hamady; Bénateau, Hervé

    2017-02-01

    A cleft palate results from incomplete fusion of the lateral palatine processes, the median nasal septum and the median palatine process. This case report describes a rare case of congenital teratoma originating from the nasal septum that may have interfered with the fusion of the palatal shelves during embryonic development, resulting in a cleft palate. An infant girl was born at 40 weeks of gestation weighing 3020 g with a complete cleft palate associated with a large central nasopharyngeal tumour. Computed tomography (CT) of the head showed a well defined mass of mixed density. The tumour was attached to the nasal septum in direct contact with the cleft palate. A biopsy confirmed the teratoma. Tumour resection was performed at 5 months, soft palate reconstruction at 7 months and hard palate closure at 14 months. There was no sign of local recurrence 1 year later. Most teratomas are benign and the prognosis is usually good. However, recurrence is not rare if germ cell carcinomatous foci are present within the teratoma. For these reasons, we advocate the use of a two-stage procedure in which closure of the cleft palate is postponed until histological examination confirms complete excision of the teratoma.

  3. Combined mediastinal and retroperitoneal fibrosis

    PubMed Central

    Salmon, H. W.

    1968-01-01

    A case of combined idiopathic mediastinal fibrosis and retroperitoneal fibrosis is described. It is possibly the twelfth case to be reported during life. A review of the literature reveals the `ubiquity' of localized collagenosis and the trend of opinion as regards aetiology and treatment. Images PMID:5654073

  4. Retroperitoneal Fasciitis: Spectrum of CT Findings in the Abdomen and Pelvis.

    PubMed

    Chingkoe, Christina M; Jahed, Ali; Loreto, Michael P; Sarrazin, Josée; McGregor, Caitlin T; Blaichman, Jason I; Glanc, Phyllis

    2015-01-01

    Retroperitoneal fasciitis is a rare but potentially lethal complication of infection. Early diagnosis is crucial and is usually made when there is a high degree of clinical suspicion combined with characteristic imaging findings leading to early surgical intervention. Computed tomography (CT) can play a central role in demonstrating early findings, assessing the extent of disease to help determine the best surgical approach, identifying the primary source of infection, and evaluating the treatment response. The possible presence of retroperitoneal fasciitis should be considered in patients presenting with symptoms of sepsis, including pain that is disproportionate with the clinical abnormality. When retroperitoneal fasciitis is suspected, emergency CT can facilitate early diagnosis and evaluation of the extent of disease. Common findings at CT include fascial thickening and enhancement, muscular edema, fat stranding, fluid collections, and abscess formation. Gas tracking along fascial planes in the retroperitoneum is the hallmark of retroperitoneal fasciitis but is not seen in all cases. Another important clue to the diagnosis is asymmetric involvement of the retroperitoneal fascial planes and deep tissues. Fasciitis in the retroperitoneum may originate from infected retroperitoneal organs or from infection that spreads along indirect and/or direct pathways from a primary source elsewhere in the body. Findings of indirect tracking and transgression of fascial planes may indicate more severe infection associated with the necrotizing form of retroperitoneal fasciitis. Despite aggressive antibiotic treatment, early and repeated surgical débridement may be required to remove nonviable tissue in patients with the necrotizing form of retroperitoneal fasciitis. Awareness of the anatomy of the retroperitoneum, potential routes of spread of infection, and the spectrum of CT findings in retroperitoneal fasciitis is needed to achieve prompt diagnosis and guide treatment.

  5. [Autoimmune hemolytic anemia associated with mesenteric teratoma].

    PubMed

    Ntanishyan, K I; Sabirov, K R; Shcherbakova, O V; Vybornykh, D E; Shupletsova, I A; Tsvetaeva, N V

    2017-01-01

    The paper describes a case of autoimmune hemolytic anemia (AIHA) in a 27-year-old woman whose examination revealed mesenteric teratoma. AIHA was characterized by a hypertensive crisis and a temporary response to corticosteroid therapy that was complicated by the development of somatogenic psychosis and discontinued. A relapse of hemolysis developed 6 months later. The patient underwent laparoscopic splenectomy and removal of mesenteric root teratoma. Immediately after surgery, a hematological response was obtained as relief of hemolysis and restoration of a normal hemoglobin level. There is a sustained remission of AIHA for the next 16 months.

  6. Mature Cardiac Teratoma in an Adult

    PubMed Central

    Cohen, Ronny A; Loarte, Pablo; Navarro, Victor; Mirrer, Brooks

    2012-01-01

    The incidental diagnosis in adult age is very unusual and the presence of clinical symptoms is related to its location, which is most commonly intrapericardial. The presence of intramyocardial teratoma lesions is even rarer and has been reported in few publications. The recommendations for the diagnosis and management of a cardiac teratoma depends upon the imaging studies and the pathological report after surgical excision. The prognosis of surgically treated patient is very good and a complete surgical excision is preferred in order to avoid complications.

  7. Huge Gastric Teratoma in an 8-Year Old Boy

    PubMed Central

    Ratan, Simmi K; Man, Parveen k

    2016-01-01

    Gastric teratoma is very rare tumor and usually presents in early infancy. An 8-year-old boy presented with a huge mass in abdomen extending from epigastrium to the pelvis. Ultrasound and CT scan of abdomen revealed a huge mass with solid and cystic components and internal calcifications. The preoperative diagnosis was a teratoma but not specifically gastric one. At operation, it was found to be gastric teratoma. The mass was excised completely with part of the stomach wall. The histopathology confirmed it to be mature gastric teratoma. The rarity of the teratoma with delayed presentation prompted us to report the case. PMID:27900279

  8. A Rare Nasopharyngeal Teratoma Arising From the Vomer.

    PubMed

    Pang, Xiaoxiao; Kwon, Hyuk-Jae; Shi, Bing; Li, Chenghao

    2016-03-01

    Teratomas are rare germ cell neoplasms derived from the 3 germinal layers (ectoderm, mesoderm, and endoderm). Nasopharyngeal teratoma is a very rare teratoma arising anywhere from the oronasal cavity, regarded as an expanding, avity filling lesion, with a high mortality rate because of severe airway obstruction, especially in the neonatal period and make up only 2% of all teratomas. The authors present a case of an infant girl with a single, finger-like, hairy teratoma arising from the vomer and protruding from the mouth with bilateral complete cleft palate, cleft lip, and cleft alveolus. Complete intraoral resection of the teratoma and cleft lip repair was conducted simultaneously. Reconstruction of the cleft palate was performed at a later stage. Recurrence occurred 9 months after surgery and extended complete surgical excision was performed after recurrence, with no recurrence observed again to date. Histopathologic examination confirmed the diagnosis of congenital mature teratoma.

  9. Ovarian teratoma and endometritis in a mare

    PubMed Central

    2005-01-01

    Abstract An 8-year-old Arabian mare was admitted for a large ovarian anovulatory follicle. A clinical diagnosis of ovarian tumor and endometritis was established. Histological examinations revealed an ovarian teratoma and a grade II endometritis. Three months after unilateral ovariectomy, the mare was confirmed pregnant and eventually gave birth uneventfully. PMID:16363331

  10. Retroperitoneal Angiomyolipoma: A Case Report and Review of the Literature

    PubMed Central

    King, Jonathan; McClintock, Scott

    2013-01-01

    Retroperitoneal angiomyolipoma is a rare tumour that is difficult to diagnose preoperatively. We present a case of retroperitoneal angiomyolipoma that highlights its diagnostic dilemma. We also performed a literature review and present a review of retroperitoneal angiomyolipoma. PMID:24303222

  11. Retroperitoneal hematoma following trauma: its clinical importance.

    PubMed

    Grieco, J G; Perry, J F

    1980-09-01

    Records of 100 consecutive patients treated in 1973 through 1977 with post-traumatic retroperitoneal hematomas (RH) were studied. Eighty RH followed blunt injury and 20 were due to penetrating trauma. Overall mortality was 26%. The worst prognosis was associated with RH from automobile accidents and pedestrian injuries. Pelvic RH were almost uniformly associated with pelvic fracture and were the primary cause of 39% of deaths. Blunt perinephric RH required renal exploration in 47% of patients. Blunt RH in other locations were associated with major visceral or vascular injury in half the patients and were the cause of death in five. Sixty-five per cent of RH due to penetrating trauma had visceral or vascular injury requiring operative correction. Contained rupture of descending choracic aorta presented as retrogastric RH in two patients. RH from penetrating trauma should be explored routinely, since 65% are associated with visceral or vascular injury.

  12. [Retroperitoneal hematoma in pelvic fractures].

    PubMed

    Purghel, F; Jemna, C; Ciuvică, R

    2011-01-01

    Retroperitoneal trauma implies a wide variety of organs in multiple systems (digestive, urinary, circulatory, musculoskeletal); although their common result is the retroperitoneal hematoma, their management is completely different, an intervention indicated for a particular lesion being able to completely decompensate other lesions in case of insufficient diagnostic. The present material highlights the recent diagnostic and therapeutic particularities in retroperitoneal hematoma from pelvic fractures. We noted a recent trend in diminishing the role of the fracture pattern on standard pelvis X-ray in assessing the risk of hemodinamic instability, new markers being indicated as more predictive. CT scan with contrast substance, when applies, remains the gold standard in identifying the source of the vascular bleeding and in guiding the subsequent therapeutic maneuvers. The angiographic embolisation in arterial lesions remains the main therapeutic procedure in hemodinamical unstable patients, with the possibility of repeating it when needed; the C-clamp external fixator application is associated. The pre-peritoneal packing constantly gains support as an emergency hemostasis maneuver. The treatment should be adapted in each case, the hemodinamic instability being the trigger in initiation and repetition of the emergency therapeutic interventions mentioned above.

  13. Nephroblastoma Arising from Primary Testicular Germ Cell Tumor: A Case Report and Literature Review

    PubMed Central

    Alatassi, Houda; O'Bryan, Brittany E.; Messer, Jamie C.

    2016-01-01

    Adult extrarenal nephroblastoma is a very rare tumor. Nephroblastoma arising from primary testicular germ cell tumor is exceedingly rare. To our knowledge, only three cases have been reported in the English literature. We report a case of a 19-year-old man who presented with a large right testicle. Image studies showed a large retroperitoneal mass along with liver and lung metastases. Orchiectomy demonstrated a mixed germ cell tumor composed of yolk sac tumor, embryonal carcinoma, and mature and immature teratoma with a significant portion of nephroblastoma. The patient received chemotherapy and no recurrence was noted during six months of followup. WT-1 expression was also studied due to the lack of consistency of its expression in testicular nephroblastoma in the literature. We also present a discussion and review of the literature due to its rarity, which indicate an adverse prognosis for patients with nephroblastoma components receiving standard chemotherapeutical regimes for testicular germ cell tumors. PMID:27957372

  14. Symptoms at diagnosis as independent prognostic factors in retroperitoneal liposarcoma.

    PubMed

    Taguchi, Satoru; Kume, Haruki; Fukuhara, Hiroshi; Morikawa, Teppei; Kakutani, Shigenori; Takeshima, Yuta; Miyazaki, Hideyo; Suzuki, Motofumi; Fujimura, Tetsuya; Nakagawa, Tohru; Ishikawa, Akira; Igawa, Yasuhiko; Homma, Yukio

    2016-02-01

    The prognostic factors of retroperitoneal liposarcoma have yet to be clearly determined due to its rarity, whereas the prognostic value of symptoms at diagnosis has never been evaluated to date. In this context, we reviewed 24 consecutive patients with primary retroperitoneal liposarcoma who underwent surgical resection with curative intent at our institution. The Kaplan-Meier analysis and the log-rank test were used to estimate progression-free survival (PFS; primary endpoint) and sarcoma-specific survival (SSS; secondary endpoint). The effect of various clinicopathological factors, including symptoms at diagnosis, on these two endpoints was assessed with a Cox proportional hazards model. During the study period, 11 patients (45.8%) developed recurrence after the initial surgery and 8 (33.3%) succumbed to retroperitoneal liposarcoma, with a median follow-up of 64 months. A total of 16 patients (66.7%) had symptoms at diagnosis, while the remaining 8 (33.3%) were diagnosed incidentally. The symptoms were palpability of the tumor (n=8); abdominal pain/fullness (n=3); flank pain/fullness (n=2); lower extremity pain (n=1); testicular pain due to varicocele (n=1); and discomfort on urination (n=1). Patients with symptoms at diagnosis were significantly more likely to develop recurrence (log-rank test, P=0.0196) and were also more likely to succumb to sarcoma (P=0.0778) compared with asymptomatic patients. On the multivariate analysis, symptoms at diagnosis and dedifferentiated components were independent predictors of poor PFS, while positive surgical margins were predictors of poor SSS. Given that symptoms at diagnosis are easily accessible for physicians, they may prove to be useful additional prognostic factors for primary retroperitoneal liposarcoma.

  15. Exit: a salvage procedure for intraoral teratoma.

    PubMed

    Masaany, B M; Ida Sadja'ah, S; Norleza, A N; Norzi, B G; Siti Sabzah, M H

    2012-10-01

    Teratomas arising from the palate or pharynx may cause immediate life-threatening airway obstruction to the newborn. Early diagnosis via antenatal ultrasound enables the treating team to perform an ex utero intrapartum treatment (EXIT) procedure to secure an airway and perform subsequent tumour resection. We present the first EXIT procedure done in Malaysia. A 34 year old , gravida 4, para 3, her unborn child diagnosed at 24 weeks of gestation to have a large oropharyngeal mass. Upper airway obstruction was anticipated. Orchestration of multiple specialities was executed to properly plan and perform the EXIT procedure. The fetus was delivered at 33 weeks of gestation and managed to be intubated. The extraoral portion of the multilobular mass originating from the palate was resected. Complete resection of the intraoral teratoma was successfully done at day 22 of life.

  16. Fetal Cyst Reveling Retroperitoneal Enteric Duplication

    PubMed Central

    Ayadi, Imene Dahmane; Bezzine, Ahlem; Hamida, Emira Ben; Marrakchi, Zahra

    2017-01-01

    Retroperitoneum is a very uncommon site of enteric duplication (ED). We report a new case of retroperitoneal ED cyst suspected in utero. Prenatal ultrasound showed an abdominal cystic mass. Noncommunicating retroperitoneal ED cyst measuring 70 mm × 30 mm was resected. Histopathologic examination confirmed the diagnosis. PMID:28082784

  17. Neonatal Sacrococcygeal Neuroblastoma Mimicking a Teratoma

    PubMed Central

    Lugo-Vicente, Humberto; Correa-Rivas, María; Bouet, Kary; Reyes Bou, Zayhara; Suleiman, Mohammed

    2017-01-01

    We reported the first case of a congenital intrapelvic presacral neuroblastoma in Puerto Rico managed in the early neonatal period. The preoperative diagnosis was a sacrococcygeal teratoma Altman stage IV classification. This case confirms the importance of a comprehensive physical examination and observation of low-risk newborn infants with a history of adequate prenatal care and an unremarkable fetal ultrasonogram during pregnancy. PMID:28116200

  18. A Review of the Literature on Extrarenal Retroperitoneal Angiomyolipoma

    PubMed Central

    Venyo, Anthony Kodzo-Grey

    2016-01-01

    Background. Extrarenal retroperitoneal angiomyolipomas are rare. Aim. To review the literature. Results. Angiomyolipomas, previously classified as hamartomas, are now classified as benign tumours. Thirty cases of primary retroperitoneal angiomyolipomas have been reported. Diagnosis of the disease upon is based radiological and pathological findings of triphasic features of (a) fat and (b) blood vessels and myoid tissue. Immunohistochemistry tends to be positive for HMB45, MART1, HHF35, calponin, NKI-C3, and CD117. The lesion is common in women. Treatment options have included the following: (a) radical surgical excision of the lesion with renal sparing surgery or radical nephrectomy in cases where malignant tumours could not be excluded and (b) selective embolization of the lesion alone or prior to surgical excision. One case of retroperitoneal angiomyolipoma was reported in a patient 15 years after undergoing radical nephrectomy for angiomyolipoma of kidney and two cases of distant metastases of angiomyolipoma have been reported following radical resection of the tumour. Conclusions. With the report of two cases of metastases ensuing surgical resection of the primary lesions there is need for academic pathologists to debate and review angiomyolipomas to decide whether to reclassify angiomyolipomas as slow-growing malignant tumours or whether the reported cases of metastases were de novo tumours or metastatic lesions. PMID:26989509

  19. Retroperitoneal fibrosis associated with Riedel's struma

    PubMed Central

    Rao, Chandar R.; Ferguson, George C.; Kyle, Victor N.

    1973-01-01

    A case of retroperitoneal fibrosis with bilateral ureteral obstruction in association with Riedel's struma of the thyroid is reported. There has been a definite increase in incidence of retroperitoneal fibrosis, but with prompt recognition and adequate treatment the mortality rate has been decreased from the original 14%. The association of Riedel's struma with retroperitoneal fibrosis has been noted in the past and its association with sclerosing cholangitis has also been mentioned. It is not known whether Riedel's thyroiditis originates in the thyroid gland and spreads or whether true thyroiditis is part of a generalized process. The temporal relationship of thyroiditis and retroperitoneal fibrosis suggests an extension of fibrosis from the thyroid, but one cannot be sure which condition occurred first. Whatever the cause, the treatment remains the same as for retroperitoneal fibrosis from other causes. ImagesFIG. 1FIG. 2 PMID:4699273

  20. Large cardiac fibroma and teratoma in children- case reports.

    PubMed

    Jha, Neerod Kumar; Kiraly, Laszlo; Tamas, Csaba; Talo, Haitham; Khan, Mohammad Daud; El Badaoui, Hazem; Jain, Anurag; Hammad, Azzam

    2015-03-22

    Primary cardiac tumours in paediatric population are an unusual occurrence. Although, majority of such tumours are benign (90%), the frequency and type of cardiac tumours in this age group is different from the adult population. There are several consecutive series published in the last decade on cardiac neoplasms. Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms. We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision. A possible role of cardiac remodeling in myocardial tissue healing after extensive tissue resection in such patients is hypothesised through available experimental or limited clinical information.

  1. Concurrent split cord malformation and teratoma: dysembryology, presentation, and treatment.

    PubMed

    Babu, Ranjith; Reynolds, Renee; Moreno, Jessica R; Cummings, Thomas J; Bagley, Carlos A

    2014-02-01

    Split cord malformation (SCM) is a rare form of spinal dysraphism in which the spinal cord is divided in the sagittal plane, forming a double neural tube. In addition to being associated with a variety of malformations, SCM may occur with spinal cord tumors, with only exceptional cases involving teratomas. As only eight patients with a teratoma associated with SCM have been reported, their presentation characteristics and treatment are currently unclear. We review the literature of all patients with SCM with concurrent spinal teratoma, discuss the potential dysembryology, and report the first case of SCM with concurrent spinal teratoma in an elderly patient. The mean age of those with concurrent SCM and teratomas was 39.4 years, with 55.6% occurring in females. The lumbar spine was the most frequent location for teratomas (66.7%), with the Type II malformation more commonly occurring with these tumors (75%). The duration of symptoms varied widely, ranging from 1 month to 5 years, with the average duration being nearly 2 years. Back pain (87.5%) and lower extremity weakness (75%) were the most common presenting symptoms. As SCM may be associated with progressive neurological deterioration and teratomas can contain immature or malignant components, surgery should be attempted with the goal of gross total resection. Nonetheless, in patients with a concurrent tumor and spinal dysraphism, spinal teratomas should be considered in the differential diagnosis. Gross total resection of these lesions may be safely achieved even in the presence of SCM using intraoperative electrophysiologic monitoring.

  2. [Giant retroperitoneal liposarcoma--case report].

    PubMed

    Bánky, Balázs; Bányász, Zsolt; Mayer, Arpád; Almási, Kálmán; Szucs, Iván

    2005-06-01

    Retroperitoneal liposarcomas are the most frequent soft tissue sarcomas and the second most frequent retroperitoneal tumours. They represent less then 0.1% of all human malignancies. Hereby we describe the treatment of our patient where of a 15-kg giant retroperitoneal liposarcoma was successfully removed and a local recurrence two years later was operated on as well. This was the 3rd largest retroperitoneal malignant tumor that was successfully removed according to the available literature of the last five decades. These typically symptom-free tumors usually grow extreme size before diagnosed. The "gold-standard" of treatment remains surgical total excision, but the high local recurrence rate (50-60%) hopefully can be reduced by adjuvant radio- and chemotherapy. Multidisciplinary treatment and long-time follow-up can provide as high as 40% 5-year survival rate. We summarize the recent clinical, diagnostic and therapeutic methods of this rare condition.

  3. Antiplatelet Therapy and Spontaneous Retroperitoneal Hematoma: A Case Report and Literature Review

    PubMed Central

    Ibrahim, Walid; Mohamed, Abdelaziz; Sheikh, Muhammed; Shokr, Mohamed; Hassan, Abubaker; Weinberger, Jarrett; Afonso, Luis C.

    2017-01-01

    Patient: Male, 66 Final Diagnosis: Spontaneous retroperitoneal hematoma seconday dual antiplatelet therapy Symptoms: Anemia • knee joint pain Medication: — Clinical Procedure: None Specialty: Cardiology Objective: Rare disease Background: Dual antiplatelet therapy has proven efficacy in primary and secondary prevention of coronary artery disease with a relatively good safety profile. Review of the literature revealed 8 cases of spontaneous retroperitoneal hematoma secondary to antiplatelet treatment. Case Report: We report the case of a 66-year-old male with a flare of acute gout secondary to uncontrolled chronic myeloid leukemia. The patient was started on dual antiplatelet treatment following a drug-eluted stent placement for symptomatic coronary artery disease. He suffered from an unexplained acute drop of five grams of hemoglobin from 10.4 to 5.8 g/dL and symptomatic anemia. The initial labs excluded occult GI bleeding, hemolysis, and bone marrow suppression. However, an abdominal CT scan showed an approximately 7.2×4.7×6.7 cm spontaneous retroperitoneal hematoma involving the left iliacus muscle. The patient was successfully treated conservatively by discontinuing antiplatelet therapy and supportive measures. Conclusions: A spontaneous retroperitoneal hematoma often presents without localizing signs and symptoms and therefore should be considered in any case of unexplained blood loss in patients on antiplatelet therapy. CT without contrast is the modality of choice to diagnose retroperitoneal hematoma. PMID:28119516

  4. Gallium-67 imaging and computed tomography in early retroperitoneal fibrosis

    SciTech Connect

    Leibowich, S.; Tumeh, S.S.

    1988-11-01

    Retroperitoneal fibrosis is characterized by symptoms associated with gradual compression of retroperitoneal structures. This case report demonstrates the use of Ga-67 imaging and CT in the early diagnostic work-up of this disorder.

  5. Sacrococcygeal teratoma: prenatal diagnosis and management.

    PubMed

    Gross, S J; Benzie, R J; Sermer, M; Skidmore, M B; Wilson, S R

    1987-02-01

    Although sacrococcygeal teratoma is a rare and potentially malignant tumor, 10 cases were documented during a 5-year period at the University of Toronto Perinatal Complex. Diagnosis was made in the six cases in which prenatal ultrasound examination was performed. One patient with twins elected to terminate the pregnancy at 19 weeks. In three of the cases diagnosed prenatally, serial ultrasound was performed. There was a 75% cesarean section rate. In all cases diagnosed prenatally, the large tumor size affected the mode of delivery. In the four cases without prenatal diagnosis, two infants were delivered vaginally, and two were delivered abdominally for obstetric reasons. There was one case of neonatal morbidity where tumor vascularity and rupture resulted in hypovolemic shock. All tumors were resected and found to be benign. A plan of management is recommended and, with appropriate obstetric and pediatric care, a good outcome can be anticipated in most cases.

  6. Retroperitoneal hematoma following radical orchiectomy: Two cases

    PubMed Central

    Glicksman, Rachel; Hamilton, Robert J.; Chung, Peter

    2017-01-01

    Treatment of testicular cancer is dependent on the stage of disease at presentation. Stage 1 testicular cancer is treated with radical orchiectomy, followed by active surveillance, radiotherapy, or chemotherapy. Occasionally, unusual and unexpected postoperative changes can be seen on computed tomography (CT), and may raise concern for metastatic disease. Here, we present two cases of testicular cancer patients who developed retroperitoneal hematomas post-radical orchiectomy, one as a classical clinical presentation, and the other as an atypical radiological entity only. The first is a case of a 38-year-old male with a non-seminoma testicular cancer, who developed severe flank pain, hemodynamic instability, and progressive anemia from a retroperitoneal hematoma in the immediate (<24 hours) postoperative period, requiring urgent surgical evacuation. The second is a case of a 33-year-old male with a testicular seminoma who had a large, suspicious retroperitoneal mass on a staging CT scan concerning for metastatic disease, which was later diagnosed as a retroperitoneal hematoma. These cases reveal the clinical variability with which a retroperitoneal hematoma post-radical orchiectomy may present. In addition, the second case demonstrates the importance of recognizing radiological postoperative changes and ensuring that these findings are not mistaken for and treated as metastatic disease. PMID:28163811

  7. [Retroperitoneal perforations of the colon. Apropos of 2 cases].

    PubMed

    Jurczak, F; Likholatnikov, D; Courant, O; Hamy, A; Visset, J; Paineau, J

    1994-01-01

    The retroperitoneal perforation of the colon is rare and our observations illustrate its two modes of revelation: a retroperitoneal suppuration; it must be traited quickly in order to decrease the mortality. Note that the abscess of the thigh is exceptional. Retroperitoneal perforations during colonoscopy whose treatment (initially medical) become surgical if there is no clinical improvement.

  8. Mature cystic teratomas arise from meiotic oocytes, but not from pre-meiotic oogonia.

    PubMed

    Kaku, Hiroshi; Usui, Hirokazu; Qu, Jia; Shozu, Makio

    2016-04-01

    Mature cystic teratomas (MCTs) in the ovaries have been thought to originate from germ cells from all developmental stages, i.e., from pre-meiotic oogonia through meiotic oocytes to mature post-meiotic ova. This view was based on research on MCTs by classical methods, including those involving centromeric heteromorphisms in karyotypes, enzyme polymorphisms, and DNA polymorphisms. However, insufficient genomic information was obtained in those studies. The current study aimed to confirm the cytogenetic origin of ovarian MCTs by using short tandem repeat (STR) polymorphism analysis to obtain sufficient genomic information, especially in connection with centromeric loci. Tissue samples of MCTs (57 ovaries from 51 patients, 91 MCTs, 156 specimens in total) obtained from cystectomies or oophorectomies were used. We categorized the specimens into two groups: i) solid components of MCTs and ii) cyst walls. The numbers of solid components of MCTs from pre-meiotic oogonia, primary oocytes, secondary oocytes, and ova were 0, 33, 16, and 15, respectively. There were no pre-meiotic oogonia in this series of solid-component specimens. We propose a hypothesis for the tumorigenesis of ovarian MCTs: the precursors of ovarian MCTs are not functional oocytes or ova, but are primary oocytes that have escaped from meiotic arrest. This hypothesis could satisfactorily explain the lack of pre-meiotic teratomas observed in this study and the nearly equal distribution of teratomas originating from primary oocytes, secondary oocytes, and ova in previous studies. Furthermore, this hypothesis could provide a starting point for determining the mechanism underlying tumorigenesis of ovarian MCTs.

  9. Laparoscopic resection of retroperitoneal benign neurilemmoma

    PubMed Central

    Park, Joon Seong; Kang, Chang Moo; Yoon, Dong Sup; Lee, Woo Jung

    2017-01-01

    Purpose The aim of this study was to verify that laparoscopic resection for treating retroperitoneal benign neurilemmoma (NL) is expected to be favorable for complete resection of tumor with technical feasibility and safety. Methods We retrospectively analyzed 47 operations for retroperitoneal neurogenic tumor at Yonsei University College of Medicine, Severance Hospital and Gangnam Severance Hospital between January 2005 and September 2015. After excluding 21 patients, the remaining 26 were divided into 2 groups: those who underwent open surgery (OS) and those who underwent laparoscopic surgery (LS). We compared clinicopathological features between the 2 groups. Results There was no significant difference in operation time, estimated blood loss, transfusion, complication, recurrence, or follow-up period between 2 groups. Postoperative hospital stay was significantly shorter in the LS group versus the OS group (OS vs. LS, 7.00 ± 3.43 days vs. 4.50 ± 2.16 days; P = 0.031). Conclusion We suggest that laparoscopic resection of retroperitoneal benign NL is feasible and safe by obtaining complete resection of the tumor. LS for treating retroperitoneal benign NL could be useful with appropriate laparoscopic technique and proper patient selection. PMID:28289669

  10. Spontaneous Retroperitoneal Hemorrhage from Adrenal Artery Aneurysm

    SciTech Connect

    Gonzalez Valverde, F.M. Balsalobre, M.; Torregrosa, N.; Molto, M.; Gomez Ramos, M.J.; Vazquez Rojas, J.L.

    2007-04-15

    Spontaneous adrenal hemorrhage is a very rare but serious disorder of the adrenal gland that can require emergent treatment. We report on a 42-year-old man who underwent selective angiography for diagnosis and treatment of retroperitoneal hemorrhage from small adrenal artery aneurysm. This case gives further details about the value of transluminal artery embolization in the management of visceral aneurysm rupture.

  11. Ovarian Mature and Immature Teratomas in Monozygotic Twins: A Case Report of Simultaneous Presentation

    PubMed Central

    Kawai, Naoki; Takeshita, Toshiyuki

    2017-01-01

    Mature cystic teratoma is one of the most common kinds of ovarian tumor, and immature teratoma is a rare tumor, representing less than 1% of all ovarian teratomas. Although there are some reports about familial occurrences of ovarian tumors, literature concerning the clinical cases of monozygotic twins is rare. We report the 5-year clinical courses of a 12-year-old Japanese girl with a recurrent bilateral ovarian mature teratoma and her monozygotic twin with a unilateral ovarian mature teratoma and contralateral ovarian immature teratoma. This is the first clinical report on mature and immature teratomas of the ovary in monozygotic twins. Our cases support future clarification of the molecular mechanism and pathogenesis of teratoma. PMID:28293443

  12. 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.

  13. Sebaceous carcinoma arising in mature cystic teratoma of ovary.

    PubMed

    An, Hyo Jeong; Jung, Yong Han; Yoon, Hye Kyoung; Jung, Soo Jin

    2013-08-01

    Roughly 1% of mature cystic teratomas undergo malignant transformation. In particular, cutaneous-type adnexal neoplasms may occur in mature cystic teratomas. Sebaceous carcinomas, which arise from mature cystic teratomas, have rarely been observed, with only seven cases previously reported. Here, we present a case of a 69-year-old female who had pelvic pain for two weeks and who subsequently underwent bilateral salpingo-oophorectomy and hysterectomy. Her left ovary showed a unilocular cyst, measuring 22.0 cm in diameter, filled with sebaceous material and a few hairs. A luminally-protruding solid mass measuring 4.0 cm in diameter was also noted. Microscopic findings revealed lobular or diffusely arranged basophilic, atypical sebaceous cells connected to a typical mature cystic teratoma. Tumor cells demonstrated positive immunoreactivity for high molecular weight cytokeratin, cytokeratin 7, cytokeratin 19, epithelial membrane antigen, and carcinoembryonic antigen. Here, we present a case of sebaceous carcinoma arising from a mature cystic teratoma along with a review of previously published reports.

  14. Congenital benign teratoma of the tongue with bifid tip, ankyloglossia and polydactyly: report of a case.

    PubMed

    Andrade, Neelam N; Raikwar, Kanchan

    2010-09-01

    Teratomas of the tongue are rare, and often accompany other anomalies within the head and neck. We describe a combination of anomalies in a 6-week-old infant with teratoma and bifid tip of the tongue, severe tongue tie, and polydactyly. The teratoma was excised and the tongue tie released with no complications.

  15. Port-site metastasis as a primary complication following retroperitoneal laparoscopic radical resection of renal pelvis carcinoma or nephron-sparing surgery: A report of three cases and review of the literature

    PubMed Central

    WANG, NING; WANG, KAI; ZHONG, DACHUAN; LIU, XIA; SUN, JI; LIN, LIANXIANG; GE, LINNA; YANG, BO

    2016-01-01

    The present study reports the clinical data of two patients with renal pelvis carcinoma and one patient with renal carcinoma who developed port-site metastasis following retroperitoneal laparoscopic surgery. The current study aimed to identify the cause and prognosis of the occurrence of port-site metastasis subsequent to laparoscopic radical resection of renal pelvis carcinoma and nephron-sparing surgery. Post-operative pathology confirmed the presence of high-grade urothelial cell carcinoma in two patients and Fuhrman grade 3 renal clear cell carcinoma in one patient. Port-site metastasis was initially detected 1–7 months post-surgery. The two patients with renal pelvis carcinoma succumbed to the disease 2 and 4 months following the identification of the port-site metastasis, respectively, whereas the patient with renal carcinoma survived with no disease progression during the targeted therapy period. The occurrence of port-site metastasis may be attributed to systemic and local factors. Measures to reduce the development of this complication include strict compliance with the operating guidelines for tumor surgery, avoidance of air leakage at the port-site, complete removal of the specimen with an impermeable bag, irrigation of the laparoscopic instruments and incisional wound with povidone-iodine when necessary, and enhancement of the body's immunity. Close post-operative follow-up observation for signs of recurrence or metastasis is essential, and systemic chemotherapy may be required in patients with high-grade renal pelvis carcinoma and renal carcinoma in order to prolong life expectancy. PMID:27313720

  16. Monophasic teratoma of the ovarian remnant in a bitch.

    PubMed

    Rota, A; Tursi, M; Zabarino, S; Appino, S

    2013-04-01

    An exploratory laparotomy on a mixed-breed bitch of an estimated age of 5 years revealed that she had undergone ovariectomy in the past, but a cystic structure was present in the area of the right ovary and a whitish mass, approximately 3 cm in diameter, in the area of the left ovary. These structures were removed together with an apparently normal uterus. Histological examination of the cyst showed a thin layer of connective tissue, while the left ovarian mass revealed ovarian tissue and highly differentiated nervous tissue, confirmed through immunohistochemistry. A presumptive diagnosis of mature ovarian teratoma was made. Although teratomas generally contain recognizable elements from more than one of the three germ cell layers, they can also be monophasic, when there is only one germ layer component. Ovarian teratomas are rare in the dog and never before have been reported in an ovarian fragment.

  17. Dumb-bell-type teratoma in the lumbar spine.

    PubMed

    Okuyama, K; Abe, E; Hoshi, N; Misawa, A; Tamura, Y; Chiba, M

    2000-02-01

    We report a case of a lumbar teratoma in a 50-year-old woman. The teratoma showed a dumb-bell-type expansion at the level of the left L3/4 foramen with massive erosion of the L3 vertebral body. MRI revealed inhomogeneous signal changes in the tumor, which were histologically compatible with a mixture of bone, muscle, fat, and cyst containing sebaceous material. Complete resection of the tumor and spinal arthrodesis with pedicle screw fixation was necessary to obtain stability of the affected spinal segment.

  18. Retroperitoneal abscess: an extra-abdominal manifestation

    PubMed Central

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-01

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur revealed widespread gas between muscular planes. A retroperitoneal abscess involving the left renal fossa, psoas, iliacus and upper thigh muscles was revealed on an urgent CT scan. The patient was transferred to intensive care unit (ICU) and underwent an emergency drainage. Despite ICU the patient died 2 days after admission. PMID:25576509

  19. Retroperitoneal abscess: an extra-abdominal manifestation.

    PubMed

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-09

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur revealed widespread gas between muscular planes. A retroperitoneal abscess involving the left renal fossa, psoas, iliacus and upper thigh muscles was revealed on an urgent CT scan. The patient was transferred to intensive care unit (ICU) and underwent an emergency drainage. Despite ICU the patient died 2 days after admission.

  20. Retroperitoneal fibromatosis presenting as a presacral mass

    PubMed Central

    Choi, Seok Jin; Jeon, Ung Bae; Choo, Kie Seok

    2014-01-01

    Aggressive fibromatosis arising from the retroperitoneum is extremely rare. It may occur in association with previous trauma, abdominal surgery, drugs, Gardner’s syndrome, or familial adenomatous polyposis. We report a case of retroperitoneal fibromatosis presenting as a presacral mass with an infiltrating nature, relatively intense enhancement on enhanced computed tomography scanning, and low-signal intensity on T2-weighted magnetic resonance imaging in a patient with no significant medical or surgical history. PMID:24778806

  1. Haemoperitoneurn Secondary to Rupture of Retroperitoneal Variceal

    PubMed Central

    Molina-Perez, M.; Rodriguez-Moreno, F.; Perez-Palma, J.; Santolaria-Fernandez, F.; Essardas-Daryanani, H.; Martinez-Riera, A.

    1997-01-01

    A 45-year-old alcoholic male patient presented with hypovolemic shock and intense anemia (Hemoglobin 04.7 g/dl), and was operated on. A bleeding retroperitoneal varix located near the right colon was responsible for the clinical picture and was sutured. After operation the patient developed haemodynamic instability and pneumonia a situation which was reverted with intensive medical therapy. The patient is now doing well. PMID:9298389

  2. Hemorrhagic, calcified, and ossified benign retroperitoneal schwannoma

    PubMed Central

    Xu, Shao-Yan; Sun, Ke; Xie, Hai-Yang; Zhou, Lin; Zheng, Shu-Sen; Wang, Wei-Lin

    2016-01-01

    Abstract Background: Schwannomas are mesenchymal tumors arising from the neural sheaths of peripheral nerves. They can almost develop in any part of the body, while head, neck and extremities are the most common sites. Occurrence in the retroperitoneum is rare. Schwannomas can show secondary degenerative changes including cyst formation, hyalinization, hemorrhage, and calcification, whereas the ossified retroperitoneal schwannoma was only reported in a malignant one. Case summary: We first present a benign ossified retroperitoneal schwannoma in a 61-year-old female. The mass was found by a routine health examination. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well-defined mass in the area among duodenum, right liver, and kidney. Definitive preoperative diagnosis of the mass was difficult. By laparotomy, the mass was found in the retroperitoneum. We completely removed the tumor and gross specimen showed a mass with a capsule and 6 × 6 × 4.8 cm in size. Microscopic examination showed the tumor is composed of spindle-shaped cells with degenerative changes of hemorrhage, calcification, and ossification. Immunohistochemically, S-100 protein was strongly positive. Finally, the mass was diagnosed as a hemorrhagic, calcified, and ossified benign schwannoma in the retroperitoneum. The patient was followed up for a period of 21 months, during which she was well with no evidence of recurrence. Conclusion: We report the first case of a benign retroperitoneal schwannoma with secondary degenerative changes including hemorrhage, calcification, and ossification. Precise preoperative diagnosis of the tumor is challenging even with multiple preoperative imaging modalities. After complete resection, patients with benign retroperitoneal schwanommas generally have good prognosis. PMID:27472709

  3. Spontaneous retroperitoneal hemorrhage caused by segmental arterial mediolysis.

    PubMed

    Phillips, Courtney K; Lepor, Herbert

    2006-01-01

    Spontaneous retroperitoneal hemorrhage is a rare clinical entity; signs and symptoms include pain, hematuria, and shock. Spontaneous retroperitoneal hemorrhage can be caused by tumors, such as renal cell carcinoma and angiomyolipoma; polyarteritis nodosa; and nephritis. The least common cause is segmental arterial mediolysis. Although computed tomography is used for the diagnosis of spontaneous retroperitoneal hemorrhage, it can miss segmental arterial mediolysis as the cause of the hemorrhage. The diagnosis of segmental arterial mediolysis as a cause of spontaneous retroperitoneal hemorrhage requires angiography, with pathologic confirmation for a definitive diagnosis.

  4. Spontaneous Retroperitoneal Hemorrhage Caused by Segmental Arterial Mediolysis

    PubMed Central

    Phillips, Courtney K; Lepor, Herbert

    2006-01-01

    Spontaneous retroperitoneal hemorrhage is a rare clinical entity; signs and symptoms include pain, hematuria, and shock. Spontaneous retroperitoneal hemorrhage can be caused by tumors, such as renal cell carcinoma and angiomyolipoma; polyarteritis nodosa; and nephritis. The least common cause is segmental arterial mediolysis. Although computed tomography is used for the diagnosis of spontaneous retroperitoneal hemorrhage, it can miss segmental arterial mediolysis as the cause of the hemorrhage. The diagnosis of segmental arterial mediolysis as a cause of spontaneous retroperitoneal hemorrhage requires angiography, with pathologic confirmation for a definitive diagnosis. PMID:16985559

  5. [Retroperitoneal dedifferentiated liposarcoma extending into the iliocostal muscle and the quadratus lumborum muscle accompanied with bone formation: case report].

    PubMed

    Matsuura, H; Sakurai, M; Arima, K

    2001-12-01

    A 72-year-old man with back pain on the left side was admitted. Imaging analysis revealed a retroperitoneal mass and a mass in the left iliocostal muscle and the left quadratus lumborum muscle. The two masses could not be resected en bloc, and were resected separately. The clinicopathological findings of these tumors revealed dedifferentiated liposarcoma. The primary dedifferentiated liposarcoma appeared to have originated from the retroperitoneal space extending into the iliocostal muscle and the quadratus lumborum muscle. Then the mass was thought to have formed accompanied with osteogenesis.

  6. Characterization of human ovarian teratoma hair by using AFM, FT-IR, and Raman spectroscopy.

    PubMed

    Kim, Kyung Sook; Lee, Jinwoo; Jung, Min-Hyung; Choi, Young Joon; Park, Hun-Kuk

    2011-12-01

    The structural, physical, and chemical properties of hair taken from an ovarian teratoma (teratoma hair) was first examined by atomic force microscopy (AFM), Fourier transform infrared (FT-IR), and Raman spectroscopy. The similarities and differences between the teratoma hair and scalp hair were also investigated. Teratoma hair showed a similar morphology and chemical composition to scalp hair. Teratoma hair was covered with a cuticle in the same manner as scalp hair and showed the same amide bonding modes as scalp hair according to FT-IR and Raman spectroscopy. On the other hand, teratoma hair showed different physical properties and cysteic acid bands from scalp hair: the surface was rougher and the adhesive force was lower than the scalp hair. The cystine oxides modes did not change with the position unlike scalp hair. These differences can be understood by environmental effects not by the intrinsic properties of the teratoma hair.

  7. Mesenteric Teratoma in Elderly Female: A Rare Case Report

    PubMed Central

    Surag, KR; Kumar, Yogesh; Lakkanna, S; Raj, Preetham

    2017-01-01

    Dermoid cysts rarely present as mesenteric cysts. Mesenteric cysts are rare pathologic entities, with a reported incidence of approximately 1 of 27 000 to 1 of 100 000 admissions. Mesenteric cyst was first described by Florentine anatomist Benevieni in 1507, while performing an autopsy on an eight-year-old boy. Most commonly, teratoma occurs in the early age group. Mature mesenteric teratoma in adulthood is extremely rare. Teratoma are germ cell tumours commonly composed of multiple cell types derived from one or more of the 3 germ layers. We present the case of a 69-year-old elderly female who presented with abdomen pain for nine months with right lumbar and right iliac fossa mass. Computed Tomography (CT) abdomen revealed bilocular cystic lesion with possibility of mucinous cystadenoma with no definitive organ of origin. She underwent explorative laparotomy and total excision of the cystic mass. Histopathologic examination confirmed diagnosis of mature cystic teratoma of mesentery. This case report highlights the need to maintain high index of suspicion while evaluating abdominal mass. PMID:28274002

  8. Mesenteric Teratoma in Elderly Female: A Rare Case Report.

    PubMed

    Neeralagi, Chandrasekhar Sharanappa; Surag, K R; Kumar, Yogesh; Lakkanna, S; Raj, Preetham

    2017-01-01

    Dermoid cysts rarely present as mesenteric cysts. Mesenteric cysts are rare pathologic entities, with a reported incidence of approximately 1 of 27 000 to 1 of 100 000 admissions. Mesenteric cyst was first described by Florentine anatomist Benevieni in 1507, while performing an autopsy on an eight-year-old boy. Most commonly, teratoma occurs in the early age group. Mature mesenteric teratoma in adulthood is extremely rare. Teratoma are germ cell tumours commonly composed of multiple cell types derived from one or more of the 3 germ layers. We present the case of a 69-year-old elderly female who presented with abdomen pain for nine months with right lumbar and right iliac fossa mass. Computed Tomography (CT) abdomen revealed bilocular cystic lesion with possibility of mucinous cystadenoma with no definitive organ of origin. She underwent explorative laparotomy and total excision of the cystic mass. Histopathologic examination confirmed diagnosis of mature cystic teratoma of mesentery. This case report highlights the need to maintain high index of suspicion while evaluating abdominal mass.

  9. A Benign, Mature, Parapharyngeal Teratoma Presenting in an Adult.

    PubMed

    Punch, Gregory E; Sniezek, Joseph C; Berkey, Bryan D; Petermann, Gregory W

    2007-01-01

    We present a case of an adult female who presented mildly symptomatic and with a history of having a mass removed from her neck as an infant. Radiographic imaging detected the presence of a heterogeneous, encapsulated mass in the parapharyngeal space that was surgically resected, and subsequently pathologically confirmed to be a benign, mature cystic teratoma.

  10. Sacro-coccigeal teratoma: antenatal diagnosis and management.

    PubMed

    Di Mario, M; Morales, V; Gallina, D; Ferrari, A

    1999-05-01

    We report a case of sacro-coccygeal teratoma (ScT) in a 29 years old primigravida at 19 weeks of gestation. Ultrasonographic evaluation in antenatal management of these abnormalities is discussed. The main sonographic criteria appear to be two: dimension and rapid growth of the tumour. Cariotype must be performed to exclude aneuploidy, frequently associated.

  11. Mixed hepatoblastoma and teratoma of the liver in a 3-year-old child: a unique combination and clinical challenge

    PubMed Central

    2009-01-01

    Primary liver tumors in children are rare with malignant hepatoblastoma being the most common neoplasm. In this report, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma. Pathological assessment on a pre-operative bioptical specimen showed an immature teratoid tumor with no area of hepatoblastic differentiation present. Histological and immunhistological examination of the resected tumor specimen additionally showed tumor areas of very different differentiation pattern intermixed with each other, namely areas of hepatoblastoma-typical and neuroblastoma-like morphology as well as areas of rhadomyosarcomatous differentiation. After chemotherapy the tumor size increased and an extended right hemihepatectomy was performed. Post-operatively, the general condition of the child improved and adjuvant chemotherapy was started two weeks later. 36 months after initial diagnosis the patient is healthy, in good general condition, and without any sign of residual tumor disease. Overall, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma and was designated as mixed hepatoblastoma and teratoma. Though mesenchymal tumor portions can occur within hepatoblastomas, most commonly osteoid or chondroid, our case is different as it presents a large spectrum of mesenchymal and epithelial differentiation pattern in most of the lesion. PMID:19909520

  12. Pure Immature Teratoma of the Ovary in Adults

    PubMed Central

    Alwazzan, Ahmad Bakr; Popowich, Shaundra; Dean, Erin; Robinson, Christine; Lotocki, Robert; Altman, Alon D.

    2015-01-01

    Objective The aim of this study was to evaluate clinicopathologic characteristics, treatment outcome, and reproductive function in women diagnosed with ovarian immature teratoma (IT). Our standard chemotherapy regime is currently etoposide/cisplatin (EP), creating a unique opportunity to evaluate this protocol in ovarian ITs. Materials and Methods This study is a retrospective analysis. Twenty-seven women older than 18 years with ovarian IT stages IA to IIIC were identified and included in this study. Patients were treated at 1 institution, Health Sciences Center, Women’s Hospital, Winnipeg, Manitoba, Canada, between 1983 and 2013. Results The median age at diagnosis was 27.0 years (range, 18–36 years). Twenty-two (82%) presented with an International Federation of Gynecology and Obstetrics stage I disease, 3 (11%) had stage II, and 2 patients (7%) had stage III disease. The histologic grade distribution was grade I in 9 patients (33%), grade II in 3 patients (11%), and grade III in 15 patients (56%). Initial management was surgical for all patients: 3 (11%) hysterectomy and bilateral salpingo-oophorectomy, 1 (4%) cystectomy only, and 23 (85%) unilateral salpingo-oophorectomy. Twenty-one patients (78%) received adjuvant therapy. The median follow-up was 60 months (range, 36–72 months). One patient recurred (histological grade III) 6 months after surgery and had a complete clinical response to 4 cycles of EP chemotherapy. Twelve patients reported an attempt to conceive resulting in 10 pregnancies (8 after chemotherapy). Conclusions Ovarian IT is a curable disease. Fertility-sparing surgery should be offered. Adjuvant treatment with cisplatinum-based chemotherapy, typically with bleomycin, etoposide, and cisplatin, is still considered the standard in stages greater than stage IA grade I. Etoposide/cisplatin as a primary chemotherapy regime for early- or advanced-stage disease is an effective treatment with minimal adverse effects and high tolerability. This is

  13. Retroperitoneal abscesses in two western lowland gorillas (Gorilla gorilla gorilla).

    PubMed

    Hahn, Alicia; D'Agostino, Jennifer; Cole, Gretchen A; Raines, Jan

    2014-03-01

    This report describes two cases of retroperitoneal abscesses in female western lowland gorillas (Gorilla gorilla gorilla). Clinical symptoms included perivulvar discharge, lameness, hindlimb paresis, and general malaise. Retroperitoneal abscesses should be considered as part of a complete differential list in female gorillas with similar clinical signs.

  14. Retroperitoneal Tumors in the Pelvis: A Diagnostic Challenge in Gynecology

    PubMed Central

    Wee-Stekly, Wei-Wei; Mueller, Michael David

    2014-01-01

    Retroperitoneal tumors can pose a diagnostic and therapeutic challenge to gynecologists because of their rarity, late presentation, and complex anatomical location in the retroperitoneum. This article reviews the diagnosis and management of retroperitoneal tumors in the pelvis, and highlights the potential pitfalls that may be faced by gynecologists. PMID:25593973

  15. Multimodality Local Therapy for Retroperitoneal Sarcoma

    SciTech Connect

    Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G.; Grobmyer, Stephen R.; Hochwald, Steven N.; Marcus, Robert B.; Indelicato, Daniel J.

    2012-03-01

    Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a

  16. Warm antibody autoimmune haemolytic anaemia associated with ovarian teratoma

    PubMed Central

    Raimundo, Pedro Oliveira; Coelho, Susana; Cabeleira, Alexandra; Dias, Luis; Gonçalves, Manuela; Almeida, Julio

    2010-01-01

    The ovarian cystic teratoma is a rare cause of autoimmune haemolytic anaemia by warm antibodies, resistant to corticotherapy, with few case reports published in the medical literature. We present a case of a 45-year-old woman admitted to hospital due to general weakness. Laboratory studies revealed macrocytic anaemia, biochemical parameters of haemolysis and peripheral spherocytosis. The direct Coombs test was positive. Viral serologies, anti-nuclear antibodies, anti-double-stranded DNA antibodies and β2-microglobulin were negative. CT scan of the thorax, abdomen and pelvis showed a heterogeneous right anexial lesion. The patient was treated with corticotherapy without improvement of anaemia. Regression of extra-vascular haemolysis and normalisation of haemoglobin was obtained only after laparoscopic splenectomy and right ooforectomy, and the histopathology of the right anexial mass revealed a cystic teratoma. Previously published cases controlled the haemolysis by surgically removing the lesion associated with splenectomy. PMID:22750920

  17. Retroperitoneal liposarcoma associated with small plaque parapsoriasis

    PubMed Central

    Tartaglia, Francesco; Blasi, Sara; Sgueglia, Monica; Polichetti, Paolo; Tromba, Luciana; Berni, Alberto

    2007-01-01

    Background Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported. Case presentation A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done. Conclusion Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma. PMID:17620118

  18. Tumor Lysis Syndrome in a Retroperitoneal Sarcoma.

    PubMed

    Zakharia, Yousef; Mansour, Joshua; Vasireddi, Srinivasa; Zakharia, Kais; Fatakhov, Eduard; Koch, Christopher; Hrinczenko, Borys

    2014-01-01

    In the present case, a 49-year-old white female presented to the clinic with a 2-month history of nausea, vomiting, and right upper quadrant pain. On examination a 3-cm mass on the right anterior scalene muscle was noted. A computed tomography scan was performed revealing a 8.7 × 7.7 × 6.1 cm retroperitoneal mass with possible invasion of the inferior vena cava and right renal and left common iliac veins. An excisional biopsy was performed with pathology compatible with spindle cell sarcoma. The patient was then sent for follow-up at the sarcoma clinic as an outpatient. However, before chemotherapy was to be started the patient would be admitted to the hospital with progressively worse nausea and vomiting. At that time the patient's lab work showed lactic acidosis, acute renal failure, hyperuricemia, hyperphosphatemia, and hypocalcemia, which met the Cairo-Bishop criteria for tumor lysis syndrome (TLS). The patient was admitted to the intensive care unit and kidney dialysis initiated. The patient would become progressively obtunded at which time the family opted for hospice care. The patient eventually succumbed peacefully 3 days after her last admission. In this case report, we briefly review the literature on TLS in solid tumors, and we present a rare case of spontaneous TLS in a retroperitoneal sarcoma.

  19. Malignant transformation of a well-organized sacrococcygeal fetiform teratoma in a newborn male.

    PubMed

    Chen, Yi-Hsin; Chang, Chih-Hau; Chen, Ke-Chi; Diau, Guan-Yeu; Loh, Ih-Wei; Chu, Chih-Chun

    2007-05-01

    We report herein a case of a male newborn with a sacrococcygeal fetiform teratoma (FT). The baby presented with a large coccygeal teratoma. The preoperative diagnosis of FT was made by plain radiography, ultrasonography and magnetic resonance imaging. The baby was successfully treated by complete excision and pelvic floor reconstruction. Postoperative follow-up was uneventful until the teratoma recurred 11 months later as a malignancy. After undergoing a second operative procedure accompanied by chemotherapy, he has been doing well for 18 months.

  20. Gastric teratoma in an infant: a rare case report and discussion.

    PubMed

    Yaji, Prabhat Radhakrishna; Joshi, Sanjeev; Kinhal, Vidyadhar; Ravishankar, T H S; Jayaprakasha, G; Melapure, Ashok; Reddy, Ravi; Desai, Mahesh; Venkatesh, B

    2013-06-01

    Gastric teratoma is a rare tumor, accounting for less than 1 % of all teratomas in infants & children. To date, only about 102 cases have been reported in the literature. A 10 month old infant was brought with a history of upper abdominal mass which was otherwise asymptomatic. On evaluation it was diagnosed as gastric teratoma. On laparotomy the mass was found to be originating from lesser curvature of stomach. Mass was excised and histopathologically it was a mature cystic teratoma. No recurrence after 18 months of follow-up.

  1. Characterization of ultrastructure and collagen composition of the teratoma membrane: comparison to the amniotic membrane.

    PubMed

    Kim, Kyung Sook; Cho, Chang-Hoon; Kim, Young-Sun; Yoon, Kyung-Sik; Jung, Min-Hyung; Park, Hun-Kuk

    2013-04-01

    The structural and morphological properties of the teratoma membrane were investigated to better understand the pathogenesis of ovarian teratomas. A mature cystic teratoma and amnion were obtained from patients who underwent laparoscopic cystectomy and uncomplicated delivery, respectively. The teratoma membrane was divided into three layers according to the results of the histological analysis. Each layer showed distinct morphological properties, including an outer layer that was uniformly arranged, a middle layer with an irregular pattern of fibers, and an inner layer that was structurally dense with a wavy pattern of fibers. The morphology of the layers of the amniotic membrane was the reverse that of the teratoma membrane. In the teratoma membrane, the outer layer was primarily composed of type III collagen and the inner layer had a large amount of type III and IV collagen. The amniotic membrane showed a small amount of type III collagen in the outer layer, whereas the inner layer had large amounts of type I, III, and IV collagen. In the teratoma membrane, the collagen fibrils were arranged regularly in the outer layer, but irregularly in the inner layer. In the amniotic membrane, the arrangement of collagen fibrils was the reverse that of the teratoma membrane. Additionally, the collagen fibrils in the teratoma membrane were thinner than those of the amniotic membrane and had slightly shorter d-spacing. Two membranes showed the differences in collagen fibril arrangement, which may caused by the different functional roles.

  2. Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre

    PubMed Central

    Hogg, HDJ; Manas, DM; Lee, D; Dildey, P; Scott, J; Lunec, J; French, JJ

    2016-01-01

    Introduction Retroperitoneal sarcoma is a surgically managed condition that can recur locally following macroscopically complete resection. Owing to the low incidence of the condition, advances in treatment are reported infrequently but complete compartmental resection and adjuvant or neoadjuvant radiotherapy are areas under investigation. Given the practical difficulty of randomised trials, observational data can highlight advantages from progressive treatment approaches. Methods A retrospective database of consecutive retroperitoneal sarcoma resections performed at a single referral centre between March 1997 and March 2013 was interrogated. Histological, radiological and clinical data were collected. Univariate and multivariate analyses for disease free and overall survival were performed to establish independent predictors of disease recurrence and patient survival. Results A total of 79 patients underwent 90 resections (63 primary). The mean five-year overall and disease free survival rates were 55.3% and 24.8% respectively. Higher patient age, high tumour grade, presence of extraretroperitoneal disease and invasive tumour phenotype were found to significantly predict survival following multivariate analysis. Half (50%) of the tumours displayed invasive behaviour on histopathology and 42% of locoregional recurrence was intraperitoneal. Conclusions Retroperitoneal sarcoma is commonly an infiltrative tumour and often recurs outside of the retroperitoneum. These features limit the therapeutic impact of interventions that focus on gaining local control such as complete compartmental resection and radiotherapy. It seems likely that future advances in the management of this cancer will involve new systemic agents to treat this frequently systemic disease. PMID:26876538

  3. Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review

    PubMed Central

    Mohammadzadeh Rezaei, Mohammad Ali; Akhavan Rezayat, Alireza; Ardalan, Shima

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare condition with an unclear etiology, presenting with the development of aberrant chronic nonspecific fibroinflammatory tissue in the retroperitoneal space, which can result in entrapment and obstruction of the retroperitoneal structures. RPF is a subtype of chronic periaortitis, and can be divided into two types: primary (or idiopathic) and secondary. RPF is usually idiopathic, but can also be secondary to malignancies, certain drugs, infections, surgery, and trauma. The systemic clinical manifestations are nonspecific and include low-grade fever, fatigue, anorexia, weight loss, and myalgia. We report five patients admitted to our hospital with clinical, laboratory, imaging, and pathologic findings compatible with RPF, and we describe their treatment and follow-up. We were suspicious that the impurities of some types of opium have an important role in the pathogenesis of RPF. Some of our patients used opium again after the follow-up period; however, they used a different type with a different origin, and we were surprised to see that RPF did not form again. PMID:27896238

  4. Schwannoma Localized Retroperitoneally in a 14-Year-Old Boy

    PubMed Central

    Cayirli, Hasan; Ozguven, Ali Aykan; Gunsar, Cuneyt; Ersoy, Betul; Kandiloglu, Ali Riza

    2016-01-01

    Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children. PMID:28078159

  5. Validation of a prediction model for avoiding post-chemotherapy retroperitoneal lymphadenectomy in patients with metastatic nonseminomatous germ cell cancer

    PubMed Central

    Punjani, Nahid; Power, Nicholas; Vanhie, James J.; Winquist, Eric

    2016-01-01

    Introduction: Post-chemotherapy residual masses (PCRMs) may contain persistent cancer or teratoma in more than 50% of patients with metastatic non-seminomatous germ cell tumours (mNSGCTs). Retroperitoneal lymph node dissection (RPLND) is curative, but controversy exists about selection criteria for surgery. A validated prediction model by Vergouwe et al (2007) based on over 1000 patients was evaluated at our centre. Methods: mNSGCT patients treated with RPLND for PCRMs were identified from an electronic database. Typographical errors in the model were identified and corrected using their 2003 publication, but retaining the 2007 coefficients. Six clinical variables were included in the model and the calculated probability of benign tissue was compared with pathology. “Benign tissue only” was considered a positive test outcome in patients with a predicted probability of “benign tissue only” greater than 70%. Results: Fifty-two (52) mNSGCT patients between 1980 and 2014 were evaluable. Median age was 32 years (range 17–52) and International Germ Cell Consensus Classification (IGCCC) prognostic stages were: good 46.2%, intermediate 32.7%, and poor 21.2%. Most patients received bleomycin/etoposide/cisplatin (BEP) chemotherapy and full bilateral RPLND. Pathology showed residual cancer or teratoma in 31 patients (59.6%) and benign findings in 21 patients (40.6%). Positive and negative predictive values and accuracy were 100%, 69%, and 73%, respectively. Conclusions: “Benign tissue only” was found in 100% of patients in whom this was predicted using our pre-determined criteria. This study involved a limited number of patients, but confirms the potential value of the Vergouwe et al model. Routine use of this prediction model in clinical practice should be tested for mNSGCT patients with PCRMs. PMID:27878048

  6. Coexistence of mature cystic teratoma and adenocarcinoma in situ within atypical proliferative mucinous tumour of ovary--a case report of 35-year-old woman.

    PubMed

    Wincewicz, A; Lewitowicz, P; Adamczyk-Gruszka, O; Sulkowski, S; Kanczuga-Koda, L; Koda, M

    2015-01-01

    Combined ovarian tumors are found in common pathologic practice due to amazing potential of ovarian tissue to copy almost every tissue of human body and imitate many neoplasms of various other organs in a very flexible way. A multicystic tumor is presented in this case report of 35-year-old woman. It consisted of a cyst with sebum and hair and cavities with papillomatous projections and mucus. The ovarian tumor was diagnosed a mature cystic teratoma presenting mainly as dermoid cyst and mucinous adenocarcinoma in situ, arising within atypical proliferative mucinous tumor. This report demonstrates how histoformative properties are reflected in ovarian tumorigenesis. Such a stunning histoformativity makes ovaries the possible site of primary origin for malignant tumors that mimic extra ovarian differentiation. In the authors' point of view, the diagnosis of primary ovarian mucinous tumor within cystic teratoma is firm, whenever simultaneous extraovarian involvement by mucinous neoplasm is excluded.

  7. Surgical anatomy of the retroperitoneal spaces, Part III: Retroperitoneal blood vessels and lymphatics.

    PubMed

    Mirilas, Petros; Skandalakis, John E

    2010-02-01

    In this article, we discuss the surgical anatomy of the blood vessels and the lymphatic vessels and lymph nodes found in the retroperitoneum. Retroperitoneal blood vessels include the aorta and all its branches--parietal and visceral--from the diaphragm to the pelvis, and the inferior vena cava and its tributaries. The retroperitoneal lymphatics form a very rich and extensive chain. As a general rule, lymphatics follow the arteries and named lymph nodes are found at the root of the arteries. Retroperitoneal nodes of the abdomen comprise the inferior diaphragmatic nodes and the lumbar nodes. The latter are classified as left lumbar (aortic), intermediate (interaorticovenous), and right lumbar (caval). These nodes surround the aorta and the inferior vena cava. Around the aorta lie the paraortic nodes, preaortic nodes (include celiac, superior mesenteric, inferior mesenteric nodes collecting lymph from the splanchna supplied by the homonymous arteries), and retroaortic nodes. Similarly, around the vena cava lie the paracaval, precaval, and retrocaval nodes. Pelvic nodes include the common iliac, external and internal iliac, obturator, and sacral nodes.

  8. Gasless laparoscopic removal of retroperitoneal leiomyosarcoma.

    PubMed

    Viani, M P; Poggi, R V; Pinto, A; Maruotti, R A

    1995-02-01

    Leiomyosarcoma is a rare malignant tumor originating from the smooth muscular tissue in any part of the organism. The only therapy is its complete removal. We describe herein the operative treatment of a retroperitoneal leiomyosarcoma with gasless laparoscopic complete removal. The procedure was successfully performed in a consenting woman with an abdominal mass. Gasless laparoscopic removal was performed with a mechanical retractor (Laparolift, Origin Medsystem Inc.), obviating the creation of the pneumoperitoneum and of the sealed environment. The technique is a simple, safe, and effective surgical method. Gasless technique guarantees a clear vision, makes possible continuous suction of smoke and fluids, and allows the use of conventional instruments and easy management of suturing. The present case has proved to be another abdominal procedure that can be carried out with all the advantages of gasless miniinvasive surgery.

  9. Retroperitoneal Necrotizing Fasciitis Masquerading as Perianal Abscess - Rare and Perilous.

    PubMed

    Amaranathan, Anandhi; Sahoo, Ashok Kumar; Barathi, Deepak; Shankar, Gomathi; Sistla, Sarath Chandra

    2017-01-17

    Necrotizing fasciitis is one of the uncommon presentations of a rapidly spreading subcutaneous tissue infection. Although the actual cause is unclear in many cases, most of them are due to the rapid proliferation of microorganisms. Retroperitoneal necrotizing fasciitis is extremely rare. It is a potentially lethal infection that requires immediate and aggressive surgical care. Early diagnosis is the key to a better prognosis. The possibility of retroperitoneal necrotizing fasciitis should be suspected in patients with symptoms of sepsis that are disproportionate to clinical findings. The rapid deterioration of the patient also gives a clue towards the diagnosis. We report a 35-year-old male with perianal abscess who had been progressed to retroperitoneal necrotizing fasciitis. The patient was managed successfully with aggressive debridement and drainage after laparotomy. Appropriate antibiotics were used to combat the sepsis. The patient recovered well at follow up, three months after discharge. Another patient, a 45-year-old male with a retroperitoneal abscess, progressed to retroperitoneal necrotizing fasciitis, and extra peritoneal drainage and debridement was done. Antibiotics depending upon the culture and sensitivity were used to control sepsis. But the patient succumbed to death 45 days after surgery due to uncontrolled sepsis. Necrotizing fasciitis of any anatomical site needs aggressive surgical care with early intervention. But retroperitoneal necrotizing fasciitis needs an extra effort for diagnosis. After diagnosis, it needs timely surgical intervention and appropriate antibiotic therapy for the recovery of the patients.

  10. Intractable yawning associated with mature teratoma of the supramedial cerebellum.

    PubMed

    Saura, Hiroaki; Beppu, Takaaki; Matsuura, Hideki; Asahi, Shigeki; Uesugi, Noriyuki; Sasaki, Makoto; Ogasawara, Kuniaki

    2014-08-01

    Yawning occurs in various conditions such as hypoxia, epilepsy, and sleep disorders including sleep apnea. Intractable yawning associated with a brain tumor has been rarely reported. A 19-year-old woman presented with intractable yawning. Magnetic resonance imaging showed a tumor in the supramedial cerebellum that compressed the dorsal side of the midbrain and upper pons. After subtotal removal of the tumor, the yawning completely disappeared. Postoperative MRI showed resolution of compression of the brainstem. The tumor was histologically diagnosed as a mature teratoma. The present case suggested that the intractable yawning resulted from the tumor compressing the dorsal side of the junction between the midbrain and pons.

  11. Retroperitoneal Lymph Nodes in Transitional Cell Carcinoma of the Kidney and Ureter

    PubMed Central

    Kundu, Shilajit D.; Eggener, Scott E.

    2009-01-01

    The incidence of transitional cell carcinoma of the kidney and ureter is low and for that reason limited data exists regarding the appropriate management of regional retroperitoneal lymph nodes. Lymph node metastases have consistently been associated with an adverse prognosis. However, five-year cancer-specific survival following nephroureterectomy and lymphadenectomy for patients with lymph node involvement ranges from 0–39%, suggesting a therapeutic benefit. This review covers the primary tumor characteristics associated with lymph node involvement, imaging of the lymph nodes, as well as the rationale, role, patient selection, suggested anatomic templates, and technical considerations for lymphadenectomy. PMID:19190765

  12. Adult dermatomyositis associated with benign ovarian teratoma: A case report

    PubMed Central

    YU, XIAOMIN; QIN, DONGYAN; MA, DEHUA; YAO, QIN

    2016-01-01

    A 27-year-old female patient presented with a 3-month history of bilateral orbital and facial edema accompanied by skin erythema and heliotrope rash. The left lesion was more critical than the right. Limb muscles were occasionally sore. On physical examination, scattered hemorrhagic rashes were observed on the patient's face and neck. Upon laboratory testing, creatine kinase (CK) was markedly elevated at 1,543.2 U/l, while CK-MB isoenzyme (27.4 U/l), α-hydroxybutyric dehydrogenase (188.4 U/l) and aspartate aminotransferase (65.3 U/l) were marginally elevated. The patient was diagnosed with dermatomyositis due to the dermatological features and elevated CK. Cancer marker analysis revealed positivity for carbohydrate antigen 125 (68.15 U/ml). Magnetic resonance imaging revealed thickening of the soft tissue of the left eyelid; furthermore, concurrent long T1- and T2-weighted signals (fat saturation) were compatible with inflammatory infiltration. Non-enhanced computed tomography identified that the right accessory region (right ovary and fallopian tube) had a cystic mass with a significant fat component and thickening of the anterior uterine wall. Gynecological ultrasound findings indicated an ovarian teratoma (8.7×7.8×9.3 cm) and uterine myoma (3.6×3.1 cm). The patient's dermatological symptoms and laboratory results were significantly relieved one week after surgical removal of the teratoma with the aid of hydrocortisone, methylprednisolone and methotrexate therapy. PMID:27073526

  13. Teratomas produced from human pluripotent stem cells xenografted into immunodeficient mice - a histopathology atlas

    PubMed Central

    Damjanov, Ivan; Andrews, Peter W.

    2017-01-01

    This atlas illustrates the microscopic features of tumors produced from human pluripotent stem cells (hPSCs) xenografted into immunosuppressed mice, according to the generally accepted protocols for performing this teratoma assay of stem cell pluripotency. Microphotographs depict various hematoxylin and eosin (H&E) stained tissues derived from all three embryonic germ layers (ectoderm, mesoderm and endoderm). The appearance of persistent hPSC in teratomas is also described with special emphasis on the morphogenesis of embryoid bodies and yolk sac components surrounding them. The use of immunohistochemistry for analyzing hPSC-derived teratomas is also illustrated. PMID:28000905

  14. Extremely Late Recurrence 21 Years after Total Removal of Immature Teratoma: A Case Report and Literature Review

    PubMed Central

    MANO, Yui; KANAMORI, Masayuki; KUMABE, Toshihiro; SAITO, Ryuta; WATANABE, Mika; SONODA, Yukihiko; TOMINAGA, Teiji

    2017-01-01

    Immature teratoma (IMT) is normally treated by resection and adjuvant therapy. The present unusual case of recurrent germinoma occurred 21 years after total resection of pineal IMT. A 3-year-old boy presented with headache, disturbance of consciousness, and Parinaud’s syndrome. Magnetic resonance (MR) imaging revealed a pineal mass lesion, and total resection of the tumor was achieved. The histological diagnosis was mature teratoma. He did not receive further treatment, and did well without recurrence for 20 years. However, he suffered headache 21 years after resection, and MR imaging revealed a homogeneously enhanced pineal mass with low minimum apparent diffusion coefficient value and proton MR spectroscopy showed a huge lipid peak. The levels of tumor markers were not elevated. Cerebrospinal fluid (CSF) cytology found atypical cells with large nuclei and irregularly shaped nucleoli. To elucidate the relationship between the primary and recurrent tumors, we reviewed the histological specimens and CSF cytology at the initial treatment and found a subset of incompletely differentiated components resembling fetal tissues in the histological specimen and atypical large cells in the CSF. Based on these radiological and histological findings, we presume that the recurrent disease was disseminated germinoma after the resection of disseminated IMT. He received chemotherapy and craniospinal radiation therapy, and the enhanced lesion and atypical cells in the CSF disappeared. This case demonstrates that disseminated IMT can be controlled for the long term without adjuvant therapy, but may recur as germinoma. Tumor dormancy may account for this unusual course. PMID:27928096

  15. Experience with cardiopulmonary bypass and deep hypothermic circulatory arrest in the management of retroperitoneal tumors with large vena caval thrombi.

    PubMed Central

    Novick, A C; Kaye, M C; Cosgrove, D M; Angermeier, K; Pontes, J E; Montie, J E; Streem, S B; Klein, E; Stewart, R; Goormastic, M

    1990-01-01

    From June 1984 to September 1989, 43 patients with large vena caval tumor thrombi from retroperitoneal malignancies underwent surgical treatment with cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). The primary malignancies were renal cell carcinoma (RCC) (n = 39), renal pelvic transitional cell carcinoma (n = 1), adrenal pheochromocytoma (n = 1), and renal (n = 1) or retroperitoneal (n = 1) sarcoma. The level of the caval thrombus was either suprahepatic (n = 27), intrahepatic (n = 14), or subhepatic (n = 2). In all cases the primary tumor and caval thrombus were completely removed. Concomitant procedures included coronary artery bypass grafting (n = 5), pulmonary resection (n = 2), and hepatic lobectomy (n = 1). The time of circulatory arrest ranged from 10 to 44 minutes (mean, 23.5 minutes). There were two operative deaths (4.7%), neither of them due to to the use of DHCA. Major postoperative complications occurred in 13 patients (30.2%). There were no ischemic or neurologic complications and no cases of perioperative tumor embolization. The median postoperative hospital stay was 9 days. Twenty-two patients (51%) are alive and enjoying a good quality of life. The 3-year patient survival rates in patients with localized (n = 24) versus metastatic (n = 15) RCC are 63.9% and 10.9%, respectively (p = 0.02). We conclude that CPB with DHCA facilities excision of retroperitoneal malignancies with large caval thrombi and provides the potential for cure with low morbidity and mortality rates. PMID:2222013

  16. Laparotomized Direct Puncture for Embolization of a Retroperitoneal Arteriovenous Fistula

    SciTech Connect

    Inagawa, Shoichi; Unno, Naoki; Yamashita, Shuhei; Tanaka, Hiroki; Sakahara, Harumi

    2010-02-15

    A 28-year-old woman was referred to our institution with hope for another child after having an abortion several months previously to avoid a potential risk of catastrophic hemorrhage from a retroperitoneal arteriovenous fistula (AVF) with enlarged and twisted draining veins in the pelvis. Multiple branches coming from the right lumbar arteries and the right iliac arteries fed fistulae converging on an enlarged venous pouch anterior to the psoas major muscle in the right retroperitoneal space. It seemed impossible to achieve complete occlusion of the lesion in a single session by either transarterial or transvenous approach. A laparotomy and direct puncture of the enlarged draining vein immediately downstream of the venous pouch was performed and embolization was done with n-butyl cyanoacrylate and the aid of coils. Complete occlusion of the retroperitoneal AVF was achieved and confirmed in control angiography 5 months later.

  17. Immature ovarian teratoma with hyponatremia and low serum vasopressin level.

    PubMed

    Sakamoto, Yuki; Takei, Yuji; Saga, Yasushi; Machida, Shizuo; Takahashi, Yoshifumi; Fujiwara, Hiroyuki

    2016-10-01

    Hyponatremia is often caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Hypersecretion of vasopressin from malignant tumors can be considered a cause of SIADH. Most of these ectopic productions of vasopressin are complications of small cell lung cancer. Cases concomitant with ovarian tumors are very rare, and a specific causative substance from the ovary is often unknown. A 16-year-old woman was diagnosed with an ovarian tumor. She developed hyponatremia that was resistant to medical treatment, but immediately improved after surgical resection of the tumor. Her diagnosis was SIADH caused by an ovarian tumor; however, her serum vasopressin level was normal. It is possible that a vasopressin-like substance causing SIADH was secreted by either nervous system tissue within an immature teratoma or small cell lung cancer. We should be cautious when SIADH is a complication of an ovarian tumor.

  18. Diagnostic Significance of Cellular Neuroglial Tissue in Ovarian Immature Teratoma

    PubMed Central

    Chai, Yun; Woo, Chang Gok; Kim, Joo-Young; Kim, Chong Jai; Khang, Shin Kwang; Kim, Jiyoon; Park, In Ah; Kim, Eun Na; Kim, Kyu-Rae

    2017-01-01

    Background Immature teratoma (IT) is a tumor containing immature neuroectodermal tissue, primarily in the form of neuroepithelial tubules. However, the diagnosis of tumors containing only cellular neuroglial tissue (CNT) without distinct neuroepithelial tubules is often difficult, since the histological characteristics of immature neuroectodermal tissues remain unclear. Here, we examined the significance of CNT and tried to define immature neuroectodermal tissues by comparing the histological features of neuroglial tissues between mature teratoma (MT) and IT. Methods The histological features of neuroglial tissue, including the cellularity, border between the neuroglial and adjacent tissues, cellular composition, mitotic index, Ki-67 proliferation rate, presence or absence of tissue necrosis, vascularity, and endothelial hyperplasia, were compared between 91 MT and 35 IT cases. Results CNTs with a cellularity grade of ≥ 2 were observed in 96% of IT cases and 4% of MT cases (p < .001); however, CNT with a cellularity grade of 3 in MT cases was confined to the histologically distinct granular layer of mature cerebellar tissue. Moreover, CNT in IT exhibited significantly higher rates of Ki-67 proliferation, mitoses, and necrosis than those in MT (p < .001). Furthermore, an infiltrative border of neuroglial tissue and glomeruloid endothelial hyperplasia were significantly more frequent in IT cases than in MT cases (p < .001). Conclusions Our results suggest that if CNT with a cellularity grade of ≥ 2 is not a component of cerebellar tissue, such cases should be diagnosed as IT containing immature neuroectodermal tissue, particularly if they exhibit an infiltrative border, mitoses, necrosis, and increased Ki-67 proliferation. PMID:27737528

  19. Surgery for retroperitoneal relapse in the setting of a prior retroperitoneal lymph node dissection for germ cell tumor

    PubMed Central

    Gotto, Geoffrey T.; Carver, Brett S.; Sogani, Pramod; Sheinfeld, Joel

    2010-01-01

    Recognition of the therapeutic role of retroperitoneal lymph node dissection (RPLND) in the setting of testicular germ cell tumors (GCTs) is of utmost importance. Although the histologic findings of RPLND provide diagnostic and prognostic information, the adequacy of initial RPLND is an independent predictor of clinical outcome. Despite the advent of effective cisplatin-based chemotherapy for testicular GCTs, patients who have undergone suboptimal surgery at the time of initial RPLND are compromised. Despite the initial enthusiasm surrounding anatomic mapping studies, the use of modified RPLND templates has the potential to leave a significant number of patients with unresected retroperitoneal disease. Teratomatous elements are particularly common. Patients with retroperitoneal relapse following initial RPLND should be treated with reoperative RPLND and chemotherapy and can expect long term survival rates nearing 70% when treated in tertiary centers by experienced surgeons. PMID:20535295

  20. Mutation analysis of the c-mos proto-oncogene in human ovarian teratomas.

    PubMed Central

    de Foy, K. A.; Gayther, S. A.; Colledge, W. H.; Crockett, S.; Scott, I. V.; Evans, M. J.; Ponder, B. A.

    1998-01-01

    Female transgenic mice lacking a functional c-mos proto-oncogene develop ovarian teratomas, indicating that c-mos may behave as a tumour-suppressor gene for this type of tumour. We have analysed the entire coding region of the c-MOS gene in a series of human ovarian teratomas to determine whether there are any cancer-causing alterations. DNA from twenty teratomas was analysed by single-strand conformational analysis (SSCA) and heteroduplex analysis (HA) to screen for somatic and germline mutations. In nine of these tumours the entire gene was also sequenced. A previously reported polymorphism and a single new sequence variant were identified, neither of which we would predict to be disease-causing alterations. These results suggest that mutations in the coding region of the c-MOS gene do not play a significant role in the genesis of human ovarian teratomas. Images Figure 1 PMID:9635841

  1. Adult-onset intradural spinal teratoma in the lumbar spine: A case report.

    PubMed

    Arai, Yasuhisa; Takahashi, Masaki; Takeda, Koutarou; Shitoto, Katsuo

    2000-12-01

    Intradural spinal teratoma is a very rare tumour that can be associated with dysraphic defects. We report a case of adult-onset intradural spinal teratoma in the lumbar spine. The patient was a 54-year-old female who had chief complaints of a gait disturbance. X-rays showed an enlargement of the interpedicular distance at L3/L4 and spina bifida distal to L4. MRI showed a spindle-shaped tumour between L2 and L5. We performed laminotomy using an ultrasonic surgical knife. Pathological diagnosis of the resected tumour was matured teratoma. The diagnosis of matured teratoma was made because the tumour had no epithelium and a layered structure including prostate tissue, matured fat, cartilage and sweat gland.

  2. Sacro-coccygeal teratoma in adult: Two rare case reports and review of literature.

    PubMed

    Kumar, Narendra; Khosla, Divya; Kumar, Ritesh; Saikia, Uma Nahar; Singh, Sk

    2014-07-01

    The sacrococcygeal area is the most common site of extragonadal teratomas in infants, but is a challenge to make clinical as well as radiological diagnosis in adults. We herein describe two cases of sacrococcygeal teratoma (SCT) in adult. The clinical, radiological and histopathological characteristics of both the cases with their outcome are described with review of the literature. The standard care for SCTs is complete surgical resection of the tumor. The presence of malignant transformation is associated with a less favorable outcome.

  3. Identical twins with mature cystic teratomas treated with laparoscopic surgery: Two case reports

    PubMed Central

    Mabuchi, Yasushi; Ota, Nami; Kobayashi, Aya; Tanizaki, Yuko; Minami, Sawako; Ino, Kazuhiko

    2017-01-01

    Mature cystic teratomas are the most common among all ovarian neoplasms, representing 30–40% of the cases. However, to the best of our knowledge, there have been only two reports of mature cystic teratomas occurring in identical twins to date. We herein report a case of identical twins with mature cystic teratomas who were treated with laparoscopic surgery. A 32-year-old woman was referred to our hospital due to a tumor in the right ovary. The patient underwent laparoscopic resection of the ovarian tumor and the pathological diagnosis was benign mature cystic teratoma. Two years later, the identical twin of the abovementioned patient was referred to our hospital also due to a right ovarian tumor. The patient underwent laparoscopic resection of the ovarian tumor and the pathological diagnosis was benign mature cystic teratoma. Therefore, for early diagnosis, it may be important to consider the possibility of mature cystic teratoma in the identical twin of a patient, even in the absence of symptoms.

  4. Demonstration of the route of embryo migration in retroperitoneal ectopic pregnancy using contrast-enhanced computed tomography.

    PubMed

    Liang, Changhu; Li, Xueli; Zhao, Bin; Du, Yinglin; Xu, Shifeng

    2014-03-01

    Retroperitoneal abdominal pregnancy is exceptionally rare. An unusual retroperitoneal pregnancy has a high risk of severe bleeding. Abdominal ultrasonography and whole abdominal computerized tomography scanning should be performed for the early diagnosis of ectopic pregnancy. A case of retroperitoneal ectopic pregnancy located very close to large retroperitoneal blood vessels and treated with resection is presented. Many unusual features of retroperitoneal ectopic pregnancy were highlighted as providing further evidence in support of the main proposed embryo migration mechanism via lymphatic vessels.

  5. A retroperitoneal neuroendocrine tumor in ectopic pancreatic tissue.

    PubMed

    Okasha, Hussein Hassan; Al-Bassiouni, Fahim; El-Ela, Monir Abo; Al-Gemeie, Emad Hamza; Ezzat, Reem

    2013-07-01

    Ectopic pancreas is the relatively uncommon presence of pancreatic tissue outside the normal location of the pancreas. We report a case of abdominal pain due to retroperitoneal neuroendocrine tumor arising from heterotopic pancreatic tissue between the duodenal wall and the head of the pancreas. Patient underwent surgical enucleation of the tumor.

  6. Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge

    PubMed Central

    Gümüştaş, Oguzhan Güven; Sanal, Murat; Güner, Osman; Tümay, Volkan

    2013-01-01

    A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult. PMID:23533897

  7. Retroperitoneal bronchogenic cyst mimicking hydatid liver: a case report.

    PubMed

    Parray, Fazl Q; Sherwani, Afak Yusuf; Dangroo, Sajad Ahmad; Bisati, Rafia Aziz; Malik, Nighat Shaffi

    2012-01-01

    Bronchogenic cysts frequently occur in the mediastinum. They may be rarely encountered in the abdomen and retroperitoneum. Bronchogenic cysts can in fact mimic hydatid cysts. We report a case of retroperitoneal bronchogenic cyst below the right hemidiaphragm mimicking a hydatid cyst of the liver in a 30-year-old female.

  8. Retroperitoneal Bronchogenic Cyst Mimicking Hydatid Liver: A Case Report

    PubMed Central

    Parray, Fazl Q.; Sherwani, Afak Yusuf; Dangroo, Sajad Ahmad; Bisati, Rafia Aziz; Malik, Nighat Shaffi

    2012-01-01

    Bronchogenic cysts frequently occur in the mediastinum. They may be rarely encountered in the abdomen and retroperitoneum. Bronchogenic cysts can in fact mimic hydatid cysts. We report a case of retroperitoneal bronchogenic cyst below the right hemidiaphragm mimicking a hydatid cyst of the liver in a 30-year-old female. PMID:22606600

  9. Postcatheterization Retroperitoneal Hematoma Due to Spontaneous Lumbar Arterial Hemorrhage

    SciTech Connect

    Kalinowski, E. Anthony; Trerotola, Scott O.

    1998-07-15

    A patient developed retroperitoneal hemorrhage after cardiac catheterization, initially thought to be a complication of the puncture. Diagnostic evaluation revealed the source to be spontaneous bleeding from a lumber artery, which was successfully embolized. Spontaneous hemorrhage is a recognized complication of anticoagulation therapy and must be considered in the differential diagnosis even in the face of a seemingly obvious source of bleeding.

  10. Rare Case of Retroperitoneal Lipomatosis in an Indian Woman

    PubMed Central

    Patil, Saurabh S; Shah, Vaibhav R; Choudhary, Vivek A

    2016-01-01

    Retroperitoneal lipomatosis is a rare but distinct clinicopathological entity characterized by non encapsulated lipoma development in the retroperitoneum. Presenting complaints in the early stages are vague, and patients with retroperitoneal lipomatosis are often misdiagnosed because considerable abnormality is not detected by abdomino-pelvic sonography. However, because of the progressive nature of this lesion, most patients eventually present with varying degrees of urinary outflow obstruction and end stage renal disease, or bladder malignancies in few cases. Here we report a case of a 35-year-old Indian woman presenting with complaints of diffuse lumps in the abdomen and constipation. Based on the findings of the imaging [sonography and computed tomography (CT) scan] studies, benign retroperitoneal lipomatosis was preopreratively diagnosed and a confirmatory exploratory laprotomy was performed. Furthermore, we discuss the imaging findings obtained using various radiological modalities such as plain radiographs, intravenous urography, barium enema, sonography, CT and magnetic resonance imaging (MRI). We also discuss the etiopathogenesis, demographics, and various differential diagnoses of retroperitoneal lipomatosis. PMID:27200169

  11. Surgical management of abdominal and retroperitoneal Castleman's disease

    PubMed Central

    Bucher, Pascal; Chassot, Gilles; Zufferey, Guillaume; Ris, Frederic; Huber, Olivier; Morel, Philippe

    2005-01-01

    Background Abdominal and retroperitoneal Castleman's disease could present either as a localized disease or as a systemic disease. Castleman's disease is a lymphoid hyperplasia related to human Herpes virus type 8, which could have an aggressive behavior, similar to that of malignant lymphoid neoplasm mainly with the systemic type, or a benign one in its localized form. Methods The authors report two cases of localized Castleman's disease in the retroperitoneal space and review the current and recent progress in the knowledge of this atypical disease. Cases presentation The two patients were young healthy women presenting with a hyper vascular peri-renal mass suggestive of malignant tumor. Both have been resected in-toto. One of them had an extensive resection with nephrectomy, while the second had a kidney preserving surgery. Pathological examination revealed localized Castleman's disease and surgical margins were free of disease. Postoperative course was uneventful, and after more than 5-years of follow-up no recurrences have been observed. Conclusion Localized Castleman's disease should be considered when facing a solid hypervascular abdominal or retroperitoneal mass. A better knowledge of this disorder and its characteristic would help surgeon to avoid unnecessarily extensive resection for this benign disorder when dealing with abdominal or retroperitoneal tumors. Surgical resection is curative for the localized form, when complete, while splenectomy could be indicated for the systemic form. PMID:15941478

  12. Retroperitoneal hemangiosarcoma in a common carp Cyprinus carpio: a case report.

    PubMed

    Hyatt, Michael W; Clauss, Tonya M; Dennison, Sophie E; Camus, Alvin C

    2013-12-12

    A 7.5 kg common carp Cyprinus carpio presented with prominent localized swelling in the caudal right coelomic area, identified by ultrasound as a fluid filled mass. Fine needle aspirate (FNA) and culture results suggested a sterile seroma. Centesis removed 290 ml of serosanguinous fluid that returned within days. Recheck ultrasonography revealed a solid component within the cavity. Radiography demonstrated irregular lysis and misalignment of vertebrae adjacent to the mass, most suggestive of bacterial osteitis or neoplasia. Treatment with antibiotics followed for 2 mo but failed to resolve the lesion. Repeated radiography and ultrasonography showed progressive enlargement of the mass, with vertebral lysis and invasion characteristic of neoplasia. Ultrasound-guided FNA of the solid component of the mass was non-diagnostic. Euthanasia was elected due to the poor clinical response and primary differential of neoplasia. Post-mortem MRI and CT confirmed a retroperitoneal soft tissue mass, partially surrounded by a fluid-filled cavity, causing vertebral lysis and infiltration of the spinal canal. Expansion of the mass caused severe muscle loss and an associated elevation in creatine kinase (>120000 U l-1). Necropsy results corroborated the MRI and CT findings, revealing a retroperitoneal, multilobular, red and tan mass causing dorsal displacement of the vertebral column, with vertebral lysis, pathologic fracture and invasion of the spinal canal. Histopathologic examination revealed a locally aggressive neoplasm exhibiting multiple patterns of growth, including endothelial lined vascular channels and solid areas formed by more pleomorphic polygonal and spindle cells, consistent with hemangiosarcoma.

  13. Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

    PubMed Central

    Zhang, Youming; Xiao, Desheng; Yin, Hongling; Long, Xueying; Li, Li; Zai, Hongyan; Chen, Minfeng; Li, Wenzheng; Sun, Lunquan

    2017-01-01

    Objectives To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. Methods Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed. Results Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2–65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0–12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease. Conclusions Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis. PMID:28319177

  14. Epithelioid hemangioendothelioma of right innominate vein mimics a teratoma

    PubMed Central

    Wan, Qi; Zhou, Jiaxuan; Yu, Yudong; Sun, Qingyu; Bao, Yingying; Lei, Qiang; Zou, Qiao; Deng, Yingshi; Li, Xinchun

    2017-01-01

    Abstract Rational: Epithelioid hemangioendothelioma (EHE) is a rare neoplasm commonly known to arise from the soft tissue, lung, and liver. EHE arising from right innominate vein (RIV) has scarcely been reported in English literature. Patient concerns: Herein, we present a rare case of EHE of RIV in a 51-year-old woman with right-lower chest pain for 4 days. Computed tomography of the chest revealed a spherical mass with calcification and fatty foci located in the anterior mediastinum, thus a presumptive diagnosis of teratoma was made. Diagnoses, interventions, and outcomes: Video-assisted thoracoscopic explorations and resection of mediastinal tumor were then performed. The pathological examination showed that the tumor was EHE. Postoperative radiotherapy was delivered to the patient. Pulmonary metastases were found by chest CT a year after surgery. Lessons: A diagnosis of EHE might be considered, when a mediastinal tumor closely related to veins showing intratumoral calcification and obvious enhancement, despite the presence of a clear boundary and visible fat content. PMID:28248868

  15. Ovarian Mature Cystic Teratoma: Challenges of Surgical Management

    PubMed Central

    Sinha, Abha; Ewies, Ayman A. A.

    2016-01-01

    Although ovarian mature cystic teratomas are the commonest adnexal masses occurring in premenopausal women, there are many challenges faced by gynecologists on deciding upon the best surgical management. There is uncertainty, lack of consensus, and variation in surgical practices. This paper critically analyzes various surgical approaches and techniques used to treat these cysts in an attempt to outline a unified guidance. MEDLINE and EMBASE databases were searched in January 2015 with no date limit using the key words “ovarian teratoma” and “ovarian dermoid.” The search was limited to articles in English language, humans, and female. The two authors conducted the search independently. The laparoscopic approach is generally considered to be the gold standard for the management. Oophorectomy should be the standard operation except in younger women with a single small cyst. The risk of chemical peritonitis after contents spillage is extremely rare and can certainly be overcome with thorough peritoneal lavage using warmed fluid. There is a place for surveillance in some selected cases. PMID:27110246

  16. Trichoepithelioma Arising in an Ovarian Mature Cystic Teratoma

    PubMed Central

    Nakayama, Satoru; Muto, Haruka; Shimizu, Shinichi; Otsuki, Yoshiro; Adachi, Hiroshi; Murakoshi, Takeshi

    2016-01-01

    Here, we report an extremely rare case of trichoepithelioma (TE)—a benign epithelial tumor originating from the outer root sheath of a hair follicle—arising in an ovarian mature cystic teratoma (MCT) with fluorodeoxyglucose-positron emission tomography (FDG-PET) findings. A 48-year-old Japanese woman presented to our hospital for her annual follow-up of adenomyosis. Ultrasonography and magnetic resonance imaging revealed a left ovarian tumor with irregular-shaped septum, which was suspicious of malignancy. However, tumor marker levels were within normal range. On FDG-PET, the maximum standardized uptake value (SUVmax) of the tumor was 2.9. Laparotomy with left salpingooophorectomy was performed. Pathologic examination revealed the probability of TE, rather than basal cell carcinoma (BCC), arising in an ovarian MCT. After five years of follow-up, the patient had no sign of recurrence. The FDG-PET SUVmax was low in TE, as with other benign tumor. However, future investigation is needed to evaluate the findings of FDG-PET imaging in TE cases. PMID:28127486

  17. A rare case of lethal retroperitoneal abscess caused by Citrobacter koseri.

    PubMed

    Cai, Tommaso; Giubilei, Gianluca; Vichi, Francesca; Farina, Umberto; Costanzi, Antonio; Bartoletti, Riccardo

    2007-01-01

    Retroperitoneal abscesses are very uncommon clinical conditions. The characteristically vague symptomatology of retroperitoneal abscess and the inherent difficulty of identifying retroperitoneal disease by physical examination contributed to these dismal therapeutic outcomes. We present an unusual case of lethal retroperitoneal abscess, caused by Citrobacter diversus(koseri), treated with surgical drainage. Citrobacter species have rarely been involved in deep tissue infection and there is no reported case of lethal retroperitoneal abscess caused by C. koseri. This case is the only reported case of C. koseri as the sole pathogen associated with a lethal retroperitoneal abscess in immunocompetent patient. The case is also notable because it confirms the recent bacterial resistance to beta-lactam antibiotics and to other antimicrobial agents, like chloramphenicol or cotrimoxazol.

  18. Retroperitoneal fibrosis - the state-of-the-art.

    PubMed

    Runowska, Marta; Majewski, Dominik; Puszczewicz, Mariusz

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed.

  19. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor

    PubMed Central

    Prochaska, Erica C.; Sciallis, Andrew P.; Miller, Barbra S.

    2016-01-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor. PMID:27252518

  20. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor.

    PubMed

    Prochaska, Erica C; Sciallis, Andrew P; Miller, Barbra S

    2016-06-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor.

  1. Retroperitoneal fibrosis in three siblings with the sickle cell trait

    PubMed Central

    Phills, James A.; Geggie, Peter; Hidvegi, Robert I.; Oliva, Luis A.

    1973-01-01

    Three West-Indian black siblings with the sickle cell trait developed retroperitoneal fibrosis, a previously unreported association. Other well known renal manifestations associated with the sickle cell trait were also present in some of these cases and included renal medullary necrosis and spontaneous hematuria. It is postulated that the sickling of the erythrocytes in the periureteral vessels resulted in thrombosis, ischemia, reactive scarring and progressive fibrosis indistinguishable from the known histological picture of retroperitoneal fibrosis. The finding of fibrin thrombi in the small veins of the fibrotic tissue of one of these patients would support this explanation. ImagesFIG. 1AFIG. 1BFIG. 2FIG. 3AFIG. 3BFIG. 4AFIG. 4BFIG. 4C PMID:4699274

  2. Pseudomyxoma Retroperitonei: ARare Cause of Retroperitoneal Cystic Mass.

    PubMed

    Al-Qahtani, Hamad Hadi; Asalamah, Saleh; Akeely, Mohammed; Alshakaki, Salman Mohammad

    2016-06-01

    Pseudomyxoma retroperitonei is a rare condition, characterized by accumulation of mucinous material in the retroperitoneal space, originating predominantly from the appendiceal mucinous neoplasms. A72-year-old male patient presented with a history of progressive right side abdominal pain for 5 months with a palpable abdominal mass. Ultrasound, computerized tomography, and magnetic resonance imaging showed large right abdominal multiloculated cystic lesion with heterogeneous echoic contents. Colonoscopy revealed normal mucosa with extramural pressure on the right colon and cecum. He underwent complete excision of the mass along with right hemicolectomy. The cystic mass was containing gelatinous material. Histopathology revealed low grade mucinous neoplasm. Pseudomyxoma retroperitonei should be considered in the differential diagnosis of patient presenting with progressive right side abdominal pain and retroperitoneal cystic mass.

  3. [Aspergillosis located on polycystic kidney treated with retroperitoneal nephrectomy].

    PubMed

    Rabii, R; Hoznek, A; Salomon, L; Bourg, S; Chopin, D K; Abbou, C C

    2001-03-01

    We reported an uncommon case of 40 years old man, cardiac transplant recipient with chronic renal faillure who consulted for infected left polycystic renal. The serum creatinine level was 750 mmol/L, and urine culture isolated a E. Ecol germe. The abdominopelvic computed tomography showed a bilateral large polycystic renal cortex and suspected the infected cyst in lower pole of left kidney. The retroperitoneal laparoscopic nephrectomy was performed confirming a renal invasive aspergillosa. About this case we should have a high index of suspicion for fungal aetiology in kidney infection in transplant patients and the management of non functioning infected polycystic kidney can use laparoscopic retroperitoneal nephrectomy. This approach can offers a minimal morbidity and alternative to open surgery.

  4. Spontaneous retroperitoneal hematoma in a patient under anticoagulant agents presenting as upper gastrointestinal bleeding.

    PubMed

    Carrilero Zaragoza, Gabriel; Egea Valenzuela, Juan; Moya Arnao, María; Muñoz Tornero, María; Jijón Crespín, Roxanna; Tomas Pujante, Paula; Iglesias Jorquera, Elena; Parra García, Josefa; Sánchez Velasco, Eduardo; Pereñíguez López, Ana; Miras López, Manuel; Fuster Quiñonero, Matilde; Carballo Álvarez, Fernando

    2016-12-01

    We present the case of a 44-year-old woman with past history of repeated miscarriage and Budd-Chiari syndrome secondary to primary myelofibrosis. Because of this she was under treatment with oral anticoagulant agents. The patient was admitted in hospital as she presented with gastrointestinal bleeding (melena), asthenia and progressive anemia. In an initial upper endoscopy an extrinsic duodenal compression associated with an ulcer on the posterior face of the first portion of duodenum and upper duodenal knee was observed. In the following days a huge spontaneous retroperitoneal hematoma due to anticoagulation was diagnosed by computed tomography. This was treated with a percutaneous drainage and withdrawal of the antithrombotic drugs. The evolution of the patient was initially satisfactory but she suffered subclavian and jugular vein thrombosis, and reintroduction of anticoagulant agents at the lowest therapeutic doses was required.

  5. Retroperitoneal Extraskeletal Osteosarcoma: Imaging Findings and Transarterial Chemoembolization

    SciTech Connect

    Zhang Huojun Yang Jijin Lu Jianping; Sheng Jin; Yuan Min; Jiang Xu; Li Yuxiao; Gupta, Sanjay

    2010-04-15

    Extraskeletal osteosarcoma (EOS) is an uncommon and usually highly aggressive mesenchymal tumor. Retroperitoneal extraskeletal osteosarma (REOS) is exceedingly rare. Due to the rare nature of the disease, both the diagnosis and the management of REOS can be challenging. We present the clinical history, CT findings, angiographic manifestations, and use of transarterial chemoembolization for treatment in a case of REOS. To our knowledge, the angiographic features of and attempt at transarterial treatment of REOS have not been reported in the literature.

  6. Femoral Nerve Palsy due to Anticoagulant Induced Retroperitoneal Hematoma

    PubMed Central

    Gurbuz, Orcun; Ercan, Abdulkadir; Kumtepe, Gencehan; Karal, İlker Hasan; Velioglu, Yusuf; Ener, Serdar

    2014-01-01

    A forty-one-year-old man who, sought evaluation for a sudden hip flexion contracture and groin pain with a history of mechanical mitral valve replacement, had been misdiagnosed and treated as having lumbar discopathy for two days. This patient finally was diagnosed with compressive femoral neuropathy due to warfarin-induced retroperitoneal hematoma and successfully managed nonoperatively. This case is reported in order to draw attention to this rare presentation. PMID:25386195

  7. A Huge Cystic Retroperitoneal Lymphangioma Presenting with Back Pain

    PubMed Central

    Kubachev, Kubach; Abdullaev, Elbrus; Babyshin, Valentin; Neronov, Dmitriy; Abdullaev, Abakar

    2016-01-01

    Retroperitoneal lymphangioma is a rare location and type of benign abdominal tumors. The clinical presentation of this rare disease is nonspecific, ranging from abdominal distention to sepsis. Here we present a 73-year-old female patient with 3-month history of back pain. USG and CT revealed a huge cystic mass which was surgically excised and appeared to be lymphangioma on histopathology. PMID:27843456

  8. Retroperitoneal Liposarcoma: Current Insights in Diagnosis and Treatment

    PubMed Central

    Matthyssens, Lucas E.; Creytens, David; Ceelen, Wim P.

    2015-01-01

    Retroperitoneal liposarcoma (RLS) is a rare, biologically heterogeneous tumor that present considerable challenges due to its size and deep location. As a consequence, the majority of patients with high-grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS. PMID:25713799

  9. Gallium-67 imaging in retroperitoneal fibrosis: Significance of a negative result

    SciTech Connect

    Jacobson, A.F. )

    1991-03-01

    A patient with retroperitoneal fibrosis and right peritracheal and hilar lymphadenopathy was studied using gallium-67-citrate. No abnormal uptake was seen in the regions of retroperitoneal fibrosis, while there was avid uptake in chest lesions later shown to represent small cell lung carcinoma. Retroperitoneal fibrosis which does not show gallium uptake is most likely mature, with few inflammatory elements. In patients with multiple retroperitoneal and/or mediastinal masses, gallium imaging may be useful in identifying the most active sites of disease for possible biopsy and for subsequent monitoring of response to therapy.

  10. De novo spinal teratoma after treatment of an intracranial germ cell tumor.

    PubMed

    Tohma, Y; Kaneko, T; Kita, D; Iwato, M; Hayashi, Y; Tachibana, O; Hasegawa, M; Yamashita, J

    2000-11-01

    The authors report an extremely rare case of de novo spinal teratoma after treatment for intracranial germ cell tumor. A 17-year-old male developed pain of bilateral lower extremities and urinary retention 18 months after complete remission of intracranial mixed germ cell tumor. Magnetic resonance imaging revealed a huge spinal tumor associated with spina bifida occulta. Total resection was performed, and histogenetical findings led to the diagnosis of a mature teratoma with normal p16 gene, whereas analysis of intracranial tumor showed p16 deletion. The spinal anomaly and genetic analysis strongly suggest that the spinal teratoma was a de novo tumor rather than a metastasis or dissemination of the original intracranial germ cell tumor.

  11. Photodynamic Approach for Teratoma-Free Pluripotent Stem Cell Therapy Using CDy1 and Visible Light

    PubMed Central

    2016-01-01

    Pluripotent stem cells (PSC) are promising resources for regeneration therapy, but teratoma formation is one of the critical problems for safe clinical application. After differentiation, the precise detection and subsequent elimination of undifferentiated PSC is essential for teratoma-free stem cell therapy, but a practical procedure is yet to be developed. CDy1, a PSC specific fluorescent probe, was investigated for the generation of reactive oxygen species (ROS) and demonstrated to induce selective death of PSC upon visible light irradiation. Importantly, the CDy1 and/or light irradiation did not negatively affect differentiated endothelial cells. The photodynamic treatment of PSC with CDy1 and visible light irradiation confirmed the inhibition of teratoma formation in mice, and suggests a promising new approach to safe PSC-based cell therapy. PMID:27725957

  12. Type IV Sacrococcygeal Teratoma Displacing the Urinary Bladder: Unique Magnetic Resonance Imaging

    PubMed Central

    Eftekharzadeh, Sahar; Keihani, Sorena; Fareghi, Mehdi; Alamsahebpour, Alireza; Kajbafzadeh, Abdol-Mohammad

    2016-01-01

    Type IV sacrococcygeal teratoma is a rare pediatric tumor that is confined to the presacral area with no external component. The signs and symptoms often arise due to mass effect and compression of adjacent organs. Urinary retention is an uncommon presenting symptom in these patients. A wide spectrum of imaging findings may be encountered in cases with sacrococcygeal teratoma because of variability of tumor size and components. We hereby present a unique magnetic resonance urography finding in a type IV sacrococcygeal teratoma which caused bladder displacement. A meticulous and complete resection of tumor with special attention to the pelvic plexus led to preservation of normal voiding function and normal bowel function in this patient. PMID:27413571

  13. Molecular Genetic Analysis of a Suprasellar Immature Teratoma : Mutation of Exon 4 P53 Gene

    PubMed Central

    Udin, Nujaimin; Ahmad, Ku Asmarina Ku; Ahmad, Farizan; Omar, Effat; Aziz, Mohd Ezanee; Kumar, Raj; Abdullah, Jafri Malin

    2008-01-01

    We described an intracranial immature teratoma in a 13 year old Malay boy who presented with history of chronic headache and blurring of vision. Physical findings revealed bilateral papilloedema but no other localizing sign. A Magnetic Resonance Imaging of the brain revealed a suprasellar well defined lobulated midline heterogenous mass which was intraoperatively described as mainly solid tumour with multiple small cystic component filled with yellowish jelly like material. Histopathological finding confirmed the case as immature teratoma. Molecular genetic analysis of p53 and p27 genes revealed substitution of nucleotide G to C at location nucleotide 12139, exon 4 of gene p53. No alteration was detected at exon 5–6 and 8 of p53 gene and exon 1 and 2 of p27 gene. This is the first case report of an intracranial immature teratoma with genetic mutation occuring in a Malay boy. PMID:22589625

  14. TeratoScore: Assessing the Differentiation Potential of Human Pluripotent Stem Cells by Quantitative Expression Analysis of Teratomas

    PubMed Central

    Avior, Yishai; Biancotti, Juan Carlos; Benvenisty, Nissim

    2015-01-01

    Summary Teratoma formation is the gold standard assay for testing the capacity of human pluripotent stem cells to differentiate into all embryonic germ layers. Although widely used, little effort has been made to transform this qualitative assay into a quantitative one. Using gene expression data from a wide variety of cells, we created a scorecard representing tissues from all germ layers and extraembryonic tissues. TeratoScore, an online, open-source platform based on this scorecard, distinguishes pluripotent stem cell-derived teratomas from malignant tumors, translating cell potency into a quantitative measure (http://benvenisty.huji.ac.il/teratoscore.php). The teratomas used for the algorithm also allowed us to examine gene expression differences between tumors with a diploid karyotype and those initiated by aneuploid cells. Chromosomally aberrant teratomas show a significantly different gene expression signature from that of teratomas originating from diploid cells, particularly in central nervous system-specific genes, congruent with human chromosomal syndromes. PMID:26070610

  15. Teratoma formation of human embryonic stem cells in three-dimensional perfusion culture bioreactors.

    PubMed

    Stachelscheid, H; Wulf-Goldenberg, A; Eckert, K; Jensen, J; Edsbagge, J; Björquist, P; Rivero, M; Strehl, R; Jozefczuk, J; Prigione, A; Adjaye, J; Urbaniak, T; Bussmann, P; Zeilinger, K; Gerlach, J C

    2013-09-01

    Teratoma formation in mice is today the most stringent test for pluripotency that is available for human pluripotent cells, as chimera formation and tetraploid complementation cannot be performed with human cells. The teratoma assay could also be applied for assessing the safety of human pluripotent cell-derived cell populations intended for therapeutic applications. In our study we examined the spontaneous differentiation behaviour of human embryonic stem cells (hESCs) in a perfused 3D multi-compartment bioreactor system and compared it with differentiation of hESCs and human induced pluripotent cells (hiPSCs) cultured in vitro as embryoid bodies and in vivo in an experimental mouse model of teratoma formation. Results from biochemical, histological/immunohistological and ultrastuctural analyses revealed that hESCs cultured in bioreactors formed tissue-like structures containing derivatives of all three germ layers. Comparison with embryoid bodies and the teratomas revealed a high degree of similarity of the tissues formed in the bioreactor to these in the teratomas at the histological as well as transcriptional level, as detected by comparative whole-genome RNA expression profiling. The 3D culture system represents a novel in vitro model that permits stable long-term cultivation, spontaneous multi-lineage differentiation and tissue formation of pluripotent cells that is comparable to in vivo differentiation. Such a model is of interest, e.g. for the development of novel cell differentiation strategies. In addition, the 3D in vitro model could be used for teratoma studies and pluripotency assays in a fully defined, controlled environment, alternatively to in vivo mouse models.

  16. [Ovarian teratomas as the cause of diseases of the nervous, endocrine and haematology system].

    PubMed

    Ziober-Malinowska, Patrycja; Kułak, Krzysztof; Witt, Elżbieta; Korell, Matthias; Kotarski, Jan; Tarkowski, Rafał

    2016-01-01

    Teratomas are the most common tumors of the ovary occurring in girls and young women. Derived from primordial germ cell and embryonic gonads have the ability to differentiate into the three germ layers. In about 95% of cases are benign, and their most common form is a dermoid cyst of the ovary. This paper aims to present the relationship described tumors of autoimmune hemolytic anemia, anti-NMDA-dependent autoimmune inflammation of the brain and thyroid disease. It reminds us that teratomas are not always insulated disease entities and may have a significant impact on the course of coexisting diseases and their treatment.

  17. Differential routes of carboplatin administration influence lymphocyte apoptosis in retroperitoneal lymph nodes.

    PubMed

    Huang, Yong-Wen; Zeng, Zheng; Li, Su; Liu, Ji-Hong

    2012-12-01

    We aimed to investigate carboplatin distribution in retroperitoneal lymph nodes and its effect on lymphocyte apoptosis following intravenous (IV), intra-arterial (IA), and retroperitoneal (RP) administration. Sixty-three healthy female canines were randomly assigned as IV, IA, or RP administration of carboplatin. At 0.5, 1, 2, 4, 8, 24, and 72 h after carboplatin treatment, retroperitoneal lymph nodes (n = 6 at each time point) were collected and high-performance liquid chromatography was employed to measure the carboplatin content. The differences in carboplatin pharmacokinetics of the three administration routes were compared. Terminal deoxynucleotidyl transferase mediated dUTP nick end labeling (TUNEL) was carried out to measure the lymphocyte apoptosis of the retroperitoneal lymphocytes. The peak concentration of carboplatin in plasma following IV administration was the highest among all approaches; as to the peak time, RP administration was longer than the other two administrations. Concentration for carboplatin in the retroperitoneal lymph node was highest following IA administration at early time points, but at higher time points, concentration was significantly higher following RP administration. Penetration of carboplatin into the retroperitoneal space was higher following RP administration. Following RP administration, the level of apoptotic lymphocytes in the retroperitoneal lymph nodes was significantly greater than either IV or IA. Following RP administration of carboplatin, the concentration, area under the curve of carboplatin and the number of apoptotic lymphocytes were significantly higher than those following IV and IA administration. This suggests that RP administration of carboplatin is beneficial for the treatment of retroperitoneal lymph node metastasis.

  18. Retroperitoneal Haematoma in a Patient with Dengue Haemorrhagic Fever: A Rare Case Report

    PubMed Central

    Singh, Jasminder; Singh, Harpreet; Jagota, Ruchi; Bala, Saroj

    2016-01-01

    Dengue Haemorrhagic Fever (DHF) has diverse manifestations ranging from asymptomatic petechial skin haemorrhages to life threatening cerebral, pulmonary, gastrointestinal and genitourinary haemorrhages. However, the association of spontaneous retroperitoneal haematomas with DHF is not well documented in literature. We report a rare case of spontaneous retroperitoneal haematoma complicating DHF. PMID:28050423

  19. Hypopituitarism due to lymphocytic hypophysitis in a patient with retroperitoneal fibrosis.

    PubMed Central

    Alvarez, A.; Cordido, F.; Sacristán, F.

    1997-01-01

    We present a 78-year-old woman with clinical acute hypopituitarism in whom pathologic findings showed lymphocytic hypophysitis and retroperitoneal fibrosis. Lymphocytic hypophysitis should be included in the differential diagnosis of hypopituitarism at any age. The association with idiopathic retroperitoneal fibrosis suggest an autoimmune origin for this entity. Images Figure 1 Figure 2 PMID:9519190

  20. OpenTein: a database of digital whole-slide images of stem cell-derived teratomas

    PubMed Central

    Park, Sung-Joon; Komiyama, Yusuke; Suemori, Hirofumi; Umezawa, Akihiro; Nakai, Kenta

    2016-01-01

    Human stem cells are promising sources for regenerative therapy. To ensure safety of future therapeutic applications, the differentiation potency of stem cells has to be tested and be widely opened to the public. The potency is generally assessed by teratoma formation comprising differentiated cells from all three germ layers, and the teratomas can be inspected through high-quality digital images. The teratoma assay, however, lacks consistency in transplantation protocols and even in interpretation, which needs community-based efforts for improving the assay quality. Here, we have developed a novel database OpenTein (Open Teratoma Investigation, http://opentein.hgc.jp/) to archive and freely distribute high-resolution whole-slide images and relevant records. OpenTein has been designed as a searchable, zoomable and annotatable web-based repository system. We have deposited 468 images of teratomas derived by our transplantation of human stem cells, and users can freely access and process such digital teratoma images. Approximately, the current version of OpenTein responds within 11.2 min for processing 2.03 gigapixel teratoma images. Our system offers valuable tools and resources in the new era of stem cell biology. PMID:26496950

  1. [Intrapartum management strategies for congenital cervical teratoma: the EXIT procedure (ex utero intrapartum treatment)].

    PubMed

    Nascimento, Glaucius C; De Souza, Alex S Rolland; Lima, Marcelo M S; Guerra, Gláucia V; Meneses, Jucille A; Cardoso, Alexandre S; Azevedo, Katarinna S

    2007-01-01

    Cervical teratomas are 3 to 5% of all teratomas and have an incidence of 1:20,000 to 1:40,000 live-borns. M.J.F., 31-years-old, had done a routine scan during pregnancy that evidenced a large fetal neck mass with teratoma cervical sonographic aspect. An interdisciplinary reunion decided for expectant management until pregnancy term when C section with EXIT procedure would be performed. At 34 weeks occurred spontaneous preterm labor and the interdisciplinary team was contacted. After head exteriorization a large neck mass was noticed. The EXIT procedure was performed allowing a tranquil orotracheal intubation while placental circulation was sustained. At third day the patient presented hypoxia, decreased heart rate and central cyanosis developing cardiorespiratory failure with unsuccessful response to resuscitation. Cervical teratoma is a rare fetal condition that is very important an interdisciplinary approach performing EXIT procedure to allow an adequate respiratory assistance at delivery. Intensive neonatal care is essential to improve patient outcome.

  2. [Mediastinal teratoma with malignant transformation of the somatic component. Clinical report].

    PubMed

    Gerardo, Rita; Morgado, Carolina; Calvo, Dolores; Pinto, Eugénia; Bravio, Ivan; Castelão, Nelson; Martelo, Fernando

    2009-01-01

    Mediastinal germ cell tumours (M-GCT) are rare forms of neoplasms compared with other tumours of the same location. They are classified in seminomas, malignant non-seminomatous GCT and teratomas. The malignant transformation of the somatic component of the teratoma, with sarcomatous or carcinomatous degeneration, is even more uncommon. We report the clinical case of a 32 year old man who presented with severe chest pain on the right hemithorax. The image exams revealed the existence of a large heterogeneous lesion with a diameter of 7.7 cm, with areas of lipomatous density and a calcic image with the appearance of a tooth, in the right projection of the anterior mediastinum, in the vicinity of the large vessels, compatible with teratoma. The transthoracic biopsy (CT guided) showed morphologic aspects of sarcoma. The patient was operated on with the en bloc resection of the mediastinal mass, right lung, a segment of the pericardium and the thymus. The pathological studies showed a teratoma with malignant transformation of the mesenquimatous component, with muscular differentiation into leiomiosarcoma and rabdomiosarcoma. After surgery, the patient was treated with a scheme of doxorubicin and ifosfamide. The most prominent concepts related to this clinical entity, as well as its treatment, are debated in this article, based on the most recent publications dedicated to the subject.

  3. Subtotal resection of an intradural mature teratoma in an adult presenting with difficulty initiating micturition

    PubMed Central

    Vanguardia, Maria Kristina; Honeybul, Stephen; Robbins, Peter

    2014-01-01

    Background: Teratomas are tumors comprised of tissues from all three germ layers. Teratomas within the spine are exceedingly rare especially in the absence of either spinal dysraphism, congenital abnormalities of the spine, spinal surgery, or history of lumbar punctures. Virchow was the first to describe this occurrence in the spine in 1863 and since then, only a handful of cases have been reported. Case description: A 41-year-old male presented with a longstanding history of difficulty initiating micturition and lower back pain with recent onset of saddle paraesthesia and bilateral leg pain. He did not have a history suggestive of spinal trauma nor congenital abnormalities. Neurological examination was unremarkable. Magnetic resonance imaging (MRI) confirmed the presence of an intradural extra axial lesion in the region of the cauda equine. At surgery, the lesion was found to be densely adherent to the conus and a subtotal resection was performed. Histological examination confirmed the lesion to be a mature teratoma. Postoperatively, he made a good recovery and there is no evidence of recurrence at one year follow-up. Conclusion: This case demonstrates that a teratoma without immature elements can be subtotally excised to reduce the risk of neurological morbidity. PMID:24778911

  4. Retroperitoneal fibrosis and obstructive uropathy due to actinomycosis: case report of a treatment approach.

    PubMed

    Yagmurdur, Mahmut Can; Akbulut, Sami; Colak, Aysel; Aygun, Cem; Haberal, Mehmet

    2009-01-01

    An actinomycotic retroperitoneal infection usually occurs in the presence of an intrauterine device (IUD). It can result in pelvic inflammatory disease and diffuse retroperitoneal fibrosis. A 39-year-old patient was admitted to the emergency unit with left flank pain. A computed tomography scan of the abdomen showed bilateral hydroureteronephrosis and a retroperitoneal malignant mass. Other tumors were excluded with a colonoscopy and an upper gastrointestinal endoscopy. Results of a fine needle aspiration biopsy showed fibrosis compatible with retroperitoneal mesenteritis. Double-J stents were placed in both ureters, and immunosuppressive therapy was started. The patient had clinical and radiologic responses to the therapy. A bilateral ureterolysis and sigmoid colon resection were done. The pathology report showed fibrosis and Actinomyces israelii infection. Parenteral and oral penicillins were administered. The probability of an Actinomyces infection in patients with retroperitoneal fibrosis should be kept in mind, especially in cases in which the patient has an intrauterine device.

  5. Retroperitoneal fibrosis: case series of five patients and review of the literature.

    PubMed

    Shiber, Shacahf; Eliakim-Raz, Noa; Yair, Molad

    2016-01-01

    Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow-up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature.

  6. Gastroesophageal cancer and retroperitoneal fibrosis: Two case reports and review of the literature

    PubMed Central

    Peixoto, Renata D’Alpino; Al-Barrak, Jasem; Lim, Howard; Renouf, Daniel

    2013-01-01

    Retroperitoneal fibrosis secondary to malignant disease is a rare condition associated with a dismal prognosis. We herein present the first ever reported case of retroperitoneal fibrosis related to esophageal adenocarcinoma in a 63-year-old patient who developed bilateral ureteral obstruction due to extensive retroperitoneal fibrosis 18 mo after having completed neoadjuvant chemoradation followed by surgery for a pT3N0 adenocarcinoma of the distal esophagus. We also report the case of a previously healthy woman who presented with bilateral ureteral obstruction and diffuse narrowing of the common biliary duct and was found to have extensive retroperitoneal fibrosis as a consequence of metastatic gastric adenocarcinoma. Both patients had poor performance status and were unsuitable for palliative chemotherapy. This paper shows that urinary and biliary obstructive symptoms might represent retroperitoneal fibrosis as a consequence of gastroesophageal malignancy. PMID:23671733

  7. Gastroesophageal cancer and retroperitoneal fibrosis: Two case reports and review of the literature.

    PubMed

    Peixoto, Renata D'Alpino; Al-Barrak, Jasem; Lim, Howard; Renouf, Daniel

    2013-03-15

    Retroperitoneal fibrosis secondary to malignant disease is a rare condition associated with a dismal prognosis. We herein present the first ever reported case of retroperitoneal fibrosis related to esophageal adenocarcinoma in a 63-year-old patient who developed bilateral ureteral obstruction due to extensive retroperitoneal fibrosis 18 mo after having completed neoadjuvant chemoradation followed by surgery for a pT3N0 adenocarcinoma of the distal esophagus. We also report the case of a previously healthy woman who presented with bilateral ureteral obstruction and diffuse narrowing of the common biliary duct and was found to have extensive retroperitoneal fibrosis as a consequence of metastatic gastric adenocarcinoma. Both patients had poor performance status and were unsuitable for palliative chemotherapy. This paper shows that urinary and biliary obstructive symptoms might represent retroperitoneal fibrosis as a consequence of gastroesophageal malignancy.

  8. Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management

    PubMed Central

    Laroche, Ann-Sophie; Bell, Robert Z; Bezzaoucha, Sarah; Földes, Eva; Lamarche, Caroline; Vallée, Michel

    2016-01-01

    Introduction Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function. Methods We conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital. Results We identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes. Conclusion Most of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not. PMID:27822461

  9. Positive psoas sign in presentation of retroperitoneal malignant triton tumour

    PubMed Central

    Winterbottom, Christopher Toby

    2010-01-01

    This article describes a case of a rare malignant neoplasm presenting to the emergency department with common symptomatology and its subsequent identification using a simple physical examination technique. Discussion includes a description of this rare soft tissue sarcoma and a consideration of the value of the psoas sign as a part of the routine abdominal exam to detect intra-abdominal and retroperitoneal pathology. In conclusion, this article acts as a reminder to all clinicians that uncommon and significant pathology may present to the emergency department masquerading as a common, seemingly benign, complaint, but can be clinically identified using simple techniques available to all and rapidly investigated using appropriate special investigations. PMID:22479296

  10. Benign sacrococcygeal teratoma incidentally found on routine scoliosis radiographs in a 12-year-old female: a case report

    PubMed Central

    Cunningham, Kelly M.; Chavhan, Govind B.; Ainsworth, Kelly E.

    2016-01-01

    Objective: To describe the imaging characteristics of sacrococcygeal teratomas and to review appropriate diagnostic evaluation and management. Clinical Features: 12-year-old otherwise healthy, asymptomatic female with an incidental pelvic mass found on routine scoliosis radiographs. Intervention and Outcome: The pelvic mass was further evaluated by MRI and CT scan. Management consisted of successful surgical resection with no postoperative complications. Pathology confirmed a mature, benign, sacrococcygeal teratoma. Conclusions: Chiropractors manage patients with scoliosis, which may include radiographic surveillance. Familiarity with the radiographic features of masses such as sacrococcygeal teratomas is important for prompt diagnosis and management. PMID:27069263

  11. Preventing Pluripotent Cell Teratoma in Regenerative Medicine Applied to Hematology Disorders.

    PubMed

    Bedel, Aurelie; Beliveau, François; Lamrissi-Garcia, Isabelle; Rousseau, Benoit; Moranvillier, Isabelle; Rucheton, Benoit; Guyonnet-Dupérat, Veronique; Cardinaud, Bruno; de Verneuil, Hubert; Moreau-Gaudry, François; Dabernat, Sandrine

    2016-09-07

    : Iatrogenic tumorigenesis is a major limitation for the use of human induced pluripotent stem cells (hiPSCs) in hematology. The teratoma risk comes from the persistence of hiPSCs in differentiated cell populations. Our goal was to evaluate the best system to purge residual hiPSCs before graft without compromising hematopoietic repopulation capability. Teratoma risk after systemic injection of hiPSCs expressing the reporter gene luciferase was assessed for the first time. Teratoma formation in immune-deficient mice was tracked by in vivo bioimaging. We observed that systemic injection of hiPSCs produced multisite teratoma as soon as 5 weeks after injection. To eliminate hiPSCs before grafting, we tested the embryonic-specific expression of suicide genes under the control of the pmiR-302/367 promoter. This promoter was highly active in hiPSCs but not in differentiated cells. The gene/prodrug inducible Caspase-9 (iCaspase-9)/AP20187 was more efficient and rapid than thymidine kinase/ganciclovir, fully specific, and without bystander effect. We observed that iCaspase-9-expressing hiPSCs died in a dose-dependent manner with AP20187, without reaching full eradication in vitro. Unexpectedly, nonspecific toxicity of AP20187 on iCaspase-9-negative hiPSCs and on CD34(+) cells was evidenced in vitro. This toxic effect strongly impaired CD34(+)-derived human hematopoiesis in adoptive transfers. Survivin inhibition is an alternative to the suicide gene approach because hiPSCs fully rely on survivin for survival. Survivin inhibitor YM155 was more efficient than AP20187/iCaspase-9 for killing hiPSCs, without toxicity on CD34(+) cells, in vitro and in adoptive transfers. hiPSC purge by survivin inhibitor fully eradicated teratoma formation in immune-deficient mice. This will be useful to improve the safety management for hiPSC-based medicine.

  12. Retroperitoneal fibrosis – the state-of-the-art

    PubMed Central

    Majewski, Dominik; Puszczewicz, Mariusz

    2016-01-01

    Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis – IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed. PMID:27994271

  13. Retroperitoneal Laparoscopy in Dogs: Access Technique, Working Space, and Surgical Anatomy

    PubMed Central

    Jeong, Junemoe; Ko, Jonghyeok; Lim, Hyunjoo; Kweon, Oh‐Kyeong

    2016-01-01

    Objective To develop and describe a laparoscopic retroperitoneal access technique, investigate working space establishment, and describe the surgical anatomy in the retroperitoneal space as an initial step for clinical application of retroperitoneal laparoscopy in dogs. Study Design Cadaveric and experimental study. Animals Cadaveric (n=8) and healthy (n=6) adult dogs. Methods The retroperitoneal access technique was developed in 3 cadavers based on the human technique and transperitoneal observation. Its application and working space establishment with carbon dioxide (CO2) insufflation alone was evaluated in 5 cadavers by observing with a transperitoneal telescope and in 6 live dogs by repeated computed tomography (CT) scans at pressure of 0, 5, 10, and 15 mmHg. Recordings of retroperitoneoscopy as well as working space volume and linear dimensions measured on CT images were analyzed. Results Retroperitoneal access and working space establishment with CO2 insufflation alone were successfully performed in all 6 live dogs. The only complication observed was in 1 dog that developed subclinical pneumomediastinum. As pressure increased, working space was established from the ipsilateral to the contralateral side, and peritoneal tearing eventually developed. Working space volume increased significantly from 5 mmHg and linear dimensions increased significantly from 0 to 10 mmHg. With pneumo‐retroperitoneum above 5 mmHg, retroperitoneal organs, including kidneys and adrenal glands, were easily visualized. Conclusion The retroperitoneal access technique and working space establishment with CO2 insufflation starting with 5 mmHg and increasing to 10 mmHg provided adequate working space and visualization of retroperitoneal organs, which may allow direct access for retroperitoneal laparoscopy in dogs. PMID:27731512

  14. Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report

    PubMed Central

    Minocha, Priyanka; Setia, Ankur

    2016-01-01

    Summary Abdominal pain is a very common symptom in all age groups but retroperitoneal fibrosis is a rare differential diagnosis suspected in young patients presenting with nonspecific abdominal pain and symptoms of obstructive uropathy. Presented here is a case of a 16-year-old boy who presented with symptoms of persistent abdominal pain and a previous history of swelling in the left leg. A computed tomography (CT) scan suggested retroperitoneal fibrosis and an exploratory laparotomy and histopathological examination were performed for definitive diagnosis. This case report is intended to promote awareness of retroperitoneal fibrosis in young patients among health care providers. PMID:27904827

  15. Retroperitoneal tumor: giant cavernous hemangioma – case presentation and literature review

    PubMed Central

    Haponiuk, Ireneusz; Jaworski, Radoslaw; Peksa, Rafal; Irga-Jaworska, Ninela; Jaskiewicz, Janusz

    2016-01-01

    Retroperitoneal hemangiomas are very rare. This paper presents the case of a 71-year-old female patient with giant cavernous hemangioma of the retroperitoneum who underwent surgical treatment for abdominal pain and left lower limb edema. Interventional staged treatment with percutaneous transcatheter arterial embolization prior to surgery was considered. Radical resection of the tumor was performed, which caused the symptoms to abate. Additionally a literature review of cases involving cavernous hemangioma in the retroperitoneal space is presented. No description of retroperitoneal cavernous hemangioma originating from the bowel was found in the analyzed reports. PMID:28096841

  16. Femoral nerve dysfunction after retroperitoneal hemorrhage: pathophysiology revealed by computed tomography.

    PubMed

    Reinstein, L; Alevizatos, A C; Twardzik, F G; DeMarco, S J

    1984-01-01

    In three patients receiving anticoagulation therapy who developed retroperitoneal hemorrhage computed tomography (CT) clearly localized the resulting hematoma in each case. Three distinct syndromes are described. A hemorrhage within the iliacus muscle resulted in femoral nerve dysfunction. A large hemorrhage within the iliacus muscle which extended into the psoas muscle produced both femoral and obturator nerve dysfunction. A retroperitoneal hemorrhage extrinsic to both the iliacus and psoas muscles did not produce peripheral nerve dysfunction. The pathophysiology of peripheral nerve dysfunction in retroperitoneal hemorrhage is reviewed in detail.

  17. Antenatal diagnosis of large sacro-coccygeal teratoma with foetal cardiomegaly and hydrops.

    PubMed

    Gupta, N; Shah, D; Singh, U; Tiwari, A

    2008-01-01

    We report the case of a young primigravida who presented with a 20 weeks pregnancy with a previous diagnosis of uterine fibroid. However, ultrasound evaluation revealed the presence of a large sacro-coccygeal teratoma in the foetus which was heterogeneous with cystic areas and calcific foci, and showed increased vascularity. There was also evidence of foetal cardiomegaly and hydrops foetalis, indicated by subcutaneous oedema in the foetus,thickened placenta and polyhydramnios. The presence of hydrops with sacro-coccygeal teratoma has a grave prognosis for the mother and child; hence termination of pregnancy was done. A 20 weeks old foetus with a huge sacro-coccygeal mass, which was ruptured at many places and showed areas of active bleed, was expelled.

  18. Unusual liver locations of growing teratoma syndrome in ovarian malignant germ cell tumors.

    PubMed

    Lorusso, Domenica; Malaguti, Paola; Trivellizzi, Ilaria Nausica; Scambia, Giovanni

    2011-01-01

    ► Growing teratoma syndrome (GTS) with unusual liver locations are described after fertility preserving surgery and chemotherapy treatment for mixed malignant ovarian germ cell tumors (MGCT). ► It's a rare syndrome of mixed malignant ovarian germ cell tumors and in both cases enlarged and growing liver masses appeared during cisplatin-etoposide-bleomicin (BEP) chemotherapy. ► Radiological exams (CT scan and MRI) were suggestive for secondary metastasis and serum markers became negative during chemotherapy.

  19. Differentiating retroperitoneal liposarcoma tumors with optical coherence tomography

    NASA Astrophysics Data System (ADS)

    Lev, Dina; Baranov, Stepan A.; Carbajal, Esteban F.; Young, Eric D.; Pollock, Raphael E.; Larin, Kirill V.

    2011-03-01

    Liposarcoma (LS) is a rare and heterogeneous group of malignant mesenchymal neoplasms exhibiting characteristics of adipocytic differentiation. Currently, radical surgical resection represents the most effective and widely used therapy for patients with abdominal/retroperitoneal LS, but the presence of contiguous essential organs, such as the kidney, pancreas, spleen, adrenal glands, esophagus or colon, as well as often reoccurrence of LS in A/RP calls for the enhancement of surgical techniques to minimize resection and avoid LS reoccurrences. Difficulty in detecting the margins of neoplasms due to their affinity to healthy fat tissue accounts for the high reoccurrence of LS within A/RP. Nowadays, the microscopic detection of margins is possible only by use of biopsy, and the minimization of surgical resection of healthy tissues is challenging. In this presentation we'll demonstrate the initial OCT results for the imaging and distinction of LS and normal human fat tissues and clear detection of tumor boundaries.

  20. Pancreatic Pseudocyst with Splenic Artery Erosion, Retroperitoneal and Splenic Hematoma

    PubMed Central

    Botianu, Petre V. H.; Dobre, Adrian S.; Botianu, Ana-Maria V.; Onisor, Danusia

    2015-01-01

    The erosion of the peripancreatic vascular structures is a rare but life-endangering complication of pancreatic diseases. We report a female patient with a multicompartmentalized pancreatic pseudocyst that eroded the splenic artery resulting in a retroperitoneal and splenic hematoma with hemodynamic instability which required emergency laparotomy with splenectomy, partial cystectomy, ligation of the splenic artery at the level of the vascular erosion, cholecystectomy (lithiasis), and multiple drainage. The postoperative course was difficult (elevated level of platelets, pancreatic fistula) but eventually favourable, with no abdominal complaints and no recurrence at 2-year follow-up. The case shows that the pancreatic pseudocysts may present with acute hemorrhagic complications with life-endangering potential and significant postoperative morbidity. PMID:26783490

  1. Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction

    PubMed Central

    Yan, Ting; Wang, Yujuan; Liu, Zhijun; Zhang, Xiaolei; Wu, Qian; Xi, Mingrong

    2017-01-01

    Abstract Objective: The present report aimed to present a unique case of idiopathic retroperitoneal fibrosis (RPF) presenting features of unilateral ureteral and sigmoid colon obstruction. RPF is a rare disorder with unclear etiology. Case report: A 43-year-old female had a 10-day history of lower right abdominal and lumbar pain. Gynecological examination, ultrasound, and computed tomography (CT) were all suggestive of right ovarian tumor. An enhanced CT showed right-sided hydronephrosis. The patient was diagnosed as having ovarian cancer. Ten days after hospitalization, a right intraureteral stent with a double-J catheter was inserted. Upon exploring the abdomen, unyielding RPF was encountered. A partial sigmoidectomy and colostomy were performed. Postoperative pathological results suggested idiopathic RPF. She received steroid treatments. Conclusion: RPF is a rare disease that can be misdiagnosed. Our understanding about its presentation has to be improved and it should be considered as a differential diagnosis for patients presenting with abdominal diseases. PMID:28207528

  2. Hepatic splenosis mimicking liver metastases in a patient with history of childhood immature teratoma

    PubMed Central

    Trotovsek, Blaz; Skrbinc, Breda

    2016-01-01

    Abstract Background Hepatic splenosis is rare condition, preceded by splenectomy or spleen trauma, the term refers to nodular implantation of normal splenic tissue in the liver. In patients with history of malignancy in particular, it can be mistaken for metastases and can lead to unnecessary diagnostic procedures or inappropriate treatment. Case report Twenty-two-year old male was treated for immature teratoma linked to undescended right testicle after birth. On regular follow-up examinations no signs of disease relapse or long-term consequences were observed. He was presented with incidental finding of mature cystic teratoma after elective surgery for what appeared to be left-sided inguinal hernia. The tumour was most likely a metastasis of childhood teratoma. Origin within remaining left testicle was not found. Upon further imaging diagnostics, several intrahepatic lesions were revealed. Based on radiologic appearance they were suspicious to be metastases. The patient underwent two ultrasound guided fine-needle aspiration biopsies. Cytologic diagnosis was inconclusive. Histology of laparoscopically obtained tissue disclosed presence of normal splenic tissue and led to diagnosis of hepatic splenosis. Conclusions Though hepatic splenosis is rare, it needs to be included in differential diagnosis of nodular hepatic lesions. Accurate interpretation of those lesions is crucial for appropriate management of the patient. If diagnosis eludes after cytologic diagnostics alone, laparoscopic excision of nodular lesion is warranted before considering more extensive liver resection. PMID:27247554

  3. Teratoma with Malignant Transformation: A Case Report with Pathological, Cytogenetic, and Immunohistochemistry Analysis

    PubMed Central

    Wang, Jue; Kazmi, Syed A. Jaffar

    2011-01-01

    Background. Teratoma with malignant transformation (TMT) is rare and most commonly encountered in adult patient with germ cell tumor (GCT). Method. We report a rare case of testicular teratoma with metastatic TMT/embryonal rhabdomyosarcoma (ERMS). A 44-year-old man underwent right orchiectomy which revealed a malignant teratoma, he subsequently had right pneumonectomy with two pulmonary masses containing a high-grade embryonal rhabdomyosarcoma. The patient developed liver metastasis three months after initial diagnosis. He was treated with a chemotherapy regimen with vincristine, dactinomycin, and cyclophosphamide (VAC) alternating with vincristine and irinotecan (VI) with complete resolution of his liver lesion. The tumors were examined with a battery of cytogenetic, immunohistochemical, and molecular assays. Results. The malignant cells were immunohistochemically positive for desmin, myogenin, and MyoD1. Molecular cytogenetics of embryonal rhabdomyosarcoma tissue revealed the presence of i(12p). The tumor expressed high level of TOPO2A, TOPO1, MRP1, MGMT, BCRP, ERCC1, RRM1, and TS. Conclusion. The activity of topoisomerase inhibitors and the potential usefulness of topoisomerase expression as biomarkers should be further tested in aprospective study. PMID:21776193

  4. Retroperitoneal Approach in Single-Port Laparoscopic Hysterectomy

    PubMed Central

    Kim, Tae-Hyun; Kim, Chul Jung; Lee, Yoo-Young; Choi, Chel Hun; Lee, Jeong-Won; Bae, Duk-Soo; Kim, Byoung-Gie

    2016-01-01

    Background and Objectives: In single-port laparoscopic hysterectomy(SP-LH), ligation of the uterine artery is a fundamental step. We analyzed the effectiveness and safety of 2 different surgical approaches to ligate the uterine artery in SP-LH for women with uterine myomas or adenomyosis. Methods: A single surgeon (TJ Kim) performed 36 retroperitoneal single-port laparoscopic hysterectomies (SP-rH) from September 1st 2012 to April 30th 2013. We compared these cases with 36 cases of conventional single-port laparoscopic abdominal hysterectomy (SP-aH) performed by the same surgeon from November 1st 2011 to July 31th 2012 (historic control). In the SP-rH cases, the retroperitoneal space was developed to identify the uterine artery; then, it was ligated where it originates from the internal iliac artery. Results: Estimated blood loss (EBL) was decreased in the SP-rH group compared with the SP-aH group (100 mL vs 200 mL; P = .023). The median total operative time was shorter in the SP-rH group (75 minutes vs 93 minutes; P < .05). The operative time of the Scope I phase, including ligation of the utero-ovarian (or infundibulopelvic) ligament, round ligament, uterine artery, and detachment of the bladder, was longer in the SP-rH group compared with that in the SP-aH group (26.0 minutes vs 24 minutes; P = .043). However, the operative time of the Scope II phase, including detachment of the uterosacral-cardinal ligament, vaginal cutting, and uterus removal, was shorter in the SP-rH group (19.5 minutes vs 30 minutes; P < .05). Operative complications were not significantly different between the groups (P = .374). Conclusion: Although SP-rH may be considered technically difficult, it can be performed safely and efficiently with surgical outcomes comparable to those of SP-aH. PMID:27186067

  5. Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography

    PubMed Central

    Zhang, Shuai; Chen, Min; Li, Chun-Mei; Song, Guo-Dong; Liu, Ying

    2017-01-01

    Background: Retroperitoneal fibrosis (RPF) and lymphoma presenting as retroperitoneal mass may closely resemble each other and misdiagnosis may occur. This study investigated the differential imaging features of RPF and lymphoma which presented as a retroperitoneal soft tissue using multidetector-row computed tomography (MDCT). Methods: The 42 consecutive patients were included in this retrospective review, including 19 RPF patients (45.2%; including 13 males and 6 females; mean age: 56.7 ± 6.2 years) and 23 patients with lymphoma (54.8%; including 14 males and 9 females; mean age: 57.4 ± 12.3 years). An array of qualitative computed tomography (CT) features of lesions in 42 consecutive patients with newly diagnosed untreated RPF and lymphoma were retrospectively analyzed. The quantitative size of the lesion at the para-aortic region and attenuation in the precontrast, arterial, and portal phases were calculated in regions of interest and compared between the patients with newly diagnosed untreated RPF and with lymphoma. Receiver operating characteristic curve analysis was used to assess the potential diagnostic value of each quantitative parameter. Inter-reader concordance was also calculated. Results: Mean ages between patients with RPF and lymphoma were not significantly different (56.7 ± 6.2 years vs. 57.4 ± 12.3 years P = 0.595). Compared to those in patients with lymphoma, homogeneous enhancement (65.2% vs. 94.7%, P = 0.027) and pelvic extension (52.2% vs. 89.5%, P = 0.017) were significantly more common while the involvement of additional nodes (78.3% vs. 5.3%, P < 0.001), suprarenal extension (60.9% vs. 15.8%, P = 0.004), and aortic displacement (43.5% vs. 5.3%, P = 0.006) were significantly less common in patients with RPF. Lesion size at the para-aorta was significantly greater in patients with lymphoma, compared with RPF patients (3.9 ± 1.2 cm vs. 1.8 ± 0.6 cm; P < 0.001). The attenuation values in three phases were not significantly different

  6. Thoraco-abdominal wall reconstruction after surgical debulking of a giant retroperitoneal liposarcoma: a case report.

    PubMed

    Colebunders, B; Colpaert, S D M; Mertens, M; Willemsen, P

    2011-01-01

    A case of a patient with a recurrent dedifferentiated retroperitoneal liposarcoma with extensive invasion of the thoraco-abdominal wall including the skin, requiring reconstructive surgery after debulking of the tumor is reported.

  7. Organ Preservation in a Case of Retroperitoneal Ganglioneuroma: A Case Report and Review of Literature

    PubMed Central

    2016-01-01

    The retroperitoneum is a closed space harbouring vital organs including the great vessels, kidneys and adrenal glands, ureters, and the ascending and descending colon. Surgical management of retroperitoneal pathologies may need multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs should be attempted, especially in case of benign tumors. We present a case of 15-year-old girl with an 11 × 6 × 5 cm retroperitoneal ganglioneuroma displacing the right kidney, renal vein, and ureter and abutting the IVC which was excised in toto preserving the right kidney and ureter with careful dissection around the great vessels. We also attempt to review the various surgical options available while dealing with these benign retroperitoneal tumors which are often detected incidentally and usually surround important retroperitoneal organs and vessels. PMID:27668117

  8. Laparoscopic excision of a large retroperitoneal lymphovascular malformation in an adult

    PubMed Central

    Suryawanshi, Pravin R.; Agrawal, Mohit M.; Rathod, Mukesh D.; Mandhane, Anirudha M.

    2017-01-01

    Retroperitoneal lymphangioma is a rare benign tumour of the retroperitoneal lymphatics that usually manifests in infancy. It is worth reporting of an unexpected presentation, especially in an adult. They frequently affect the neck (75%) and the axilla (20%). Intra-abdominal lymphangiomas (<5%) have been reported in the mesentery, gastrointestinal tract, spleen, liver and pancreas. Retroperitoneal lymphangiomas account for nearly 1% of all lymphangiomas and are uncommon incidental findings usually at surgery, autopsy or lymphography. Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone is often inconclusive, and surgery is frequently required for definitive diagnosis. An interesting and rare case of a retroperitoneal lymphangioma in an adult patient which was removed laparoscopically is described here. PMID:27251838

  9. Retroperitoneal Necrotizing Fasciitis Masquerading as Perianal Abscess – Rare and Perilous

    PubMed Central

    Amaranathan, Anandhi; Barathi, Deepak; Shankar, Gomathi; Sistla, Sarath Chandra

    2017-01-01

    Necrotizing fasciitis is one of the uncommon presentations of a rapidly spreading subcutaneous tissue infection. Although the actual cause is unclear in many cases, most of them are due to the rapid proliferation of microorganisms. Retroperitoneal necrotizing fasciitis is extremely rare. It is a potentially lethal infection that requires immediate and aggressive surgical care. Early diagnosis is the key to a better prognosis. The possibility of retroperitoneal necrotizing fasciitis should be suspected in patients with symptoms of sepsis that are disproportionate to clinical findings. The rapid deterioration of the patient also gives a clue towards the diagnosis. We report a 35-year-old male with perianal abscess who had been progressed to retroperitoneal necrotizing fasciitis. The patient was managed successfully with aggressive debridement and drainage after laparotomy. Appropriate antibiotics were used to combat the sepsis. The patient recovered well at follow up, three months after discharge. Another patient, a 45-year-old male with a retroperitoneal abscess, progressed to retroperitoneal necrotizing fasciitis, and extra peritoneal drainage and debridement was done. Antibiotics depending upon the culture and sensitivity were used to control sepsis. But the patient succumbed to death 45 days after surgery due to uncontrolled sepsis. Necrotizing fasciitis of any anatomical site needs aggressive surgical care with early intervention. But retroperitoneal necrotizing fasciitis needs an extra effort for diagnosis. After diagnosis, it needs timely surgical intervention and appropriate antibiotic therapy for the recovery of the patients. PMID:28229030

  10. Preventing Pluripotent Cell Teratoma in Regenerative Medicine Applied to Hematology Disorders.

    PubMed

    Bedel, Aurelie; Beliveau, François; Lamrissi-Garcia, Isabelle; Rousseau, Benoit; Moranvillier, Isabelle; Rucheton, Benoit; Guyonnet-Dupérat, Veronique; Cardinaud, Bruno; de Verneuil, Hubert; Moreau-Gaudry, François; Dabernat, Sandrine

    2017-02-01

    Iatrogenic tumorigenesis is a major limitation for the use of human induced pluripotent stem cells (hiPSCs) in hematology. The teratoma risk comes from the persistence of hiPSCs in differentiated cell populations. Our goal was to evaluate the best system to purge residual hiPSCs before graft without compromising hematopoietic repopulation capability. Teratoma risk after systemic injection of hiPSCs expressing the reporter gene luciferase was assessed for the first time. Teratoma formation in immune-deficient mice was tracked by in vivo bioimaging. We observed that systemic injection of hiPSCs produced multisite teratoma as soon as 5 weeks after injection. To eliminate hiPSCs before grafting, we tested the embryonic-specific expression of suicide genes under the control of the pmiR-302/367 promoter. This promoter was highly active in hiPSCs but not in differentiated cells. The gene/prodrug inducible Caspase-9 (iCaspase-9)/AP20187 was more efficient and rapid than thymidine kinase/ganciclovir, fully specific, and without bystander effect. We observed that iCaspase-9-expressing hiPSCs died in a dose-dependent manner with AP20187, without reaching full eradication in vitro. Unexpectedly, nonspecific toxicity of AP20187 on iCaspase-9-negative hiPSCs and on CD34(+) cells was evidenced in vitro. This toxic effect strongly impaired CD34(+) -derived human hematopoiesis in adoptive transfers. Survivin inhibition is an alternative to the suicide gene approach because hiPSCs fully rely on survivin for survival. Survivin inhibitor YM155 was more efficient than AP20187/iCaspase-9 for killing hiPSCs, without toxicity on CD34(+) cells, in vitro and in adoptive transfers. hiPSC purge by survivin inhibitor fully eradicated teratoma formation in immune-deficient mice. This will be useful to improve the safety management for hiPSC-based medicine. Stem Cells Translational Medicine 2017;6:382-393.

  11. Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland.

    PubMed

    Maeda, Yujiro; Suenaga, Hideyuki; Sugiyama, Madoka; Saijo, Hideto; Hoshi, Kazuto; Mori, Yoshiyuki; Takato, Tsuyoshi

    2013-07-01

    A 2-day-old girl was diagnosed with an oral epignathus teratoma and an uncommon combination of orofacial malformations including cleft palate; tongue, mandible, cranial base, cervical vertebrae, lower lip, and pituitary gland duplications; and fistula of the glabella and lower lip. Computed tomography revealed that the mass within the nasal cavity had tooth-like calcifications and protruded into the nasopharynx and oral cavity. It was implanted on the anterior wall of the body of the sphenoid bone and was accompanied with mandibular duplication. Magnetic resonance imaging detected duplication of the pituitary gland and confirmed the absence of intracranial communication of the nasopharyngeal mass. The teratoma did not cause respiratory obstruction; however, the patient required continuous nasogastric tube feeding. Usually, an epignathus teratoma is associated with few midline defects and can be corrected with multiple interventions at different time points. The current study describes the surgical procedure comprising excision of the tumor along with reconstructive surgeries of the mandible, tongue, and fistulae undertaken when the infant reached 7 months of age. The cleft palate was repaired at 18 months of age using the Kaplan buccal flap method. Histopathologic examination confirmed a grade 0 teratoma covered with keratinized skin and containing pilosebaceous and sweat glands, adipose tissue, and smooth muscle. The long-term success of this intervention was determined at the follow-up examination conducted at 3 years of age, with no signs of the teratoma recurrence observed.

  12. Cervical cystic swelling in an adolescent: unusual association of a cervical mature teratoma with vertebral anomalies and a history of gastric duplication cyst.

    PubMed

    Hartog, Hermien; Dikkers, Freek G; Veldhuizen, Albert G; Coppes, Maarten H; Sleeboom, Christien; de Langen, Zacharias Jan

    2011-06-01

    A 14-year-old girl presented with a cervical cystic swelling in association with deformity of cervical vertebrae. As a child, she had been treated for gastric duplication. Pathologic examination of the resected cervical swelling revealed a mature teratoma. We discuss possible embryologic associations, which could explain the unusual combination of a mature teratoma with vertebral anomalies and gastric duplication.

  13. 18F-Fluorodeoxyglucose Uptake in Anti-N-Methyl-D-Aspartate-Receptor Encephalitis Associated With an Immature Teratoma.

    PubMed

    Nakamoto, Ryusuke; Nakamoto, Yuji; Ishimori, Takayoshi; Fushimi, Yasutaka; Togashi, Kaori

    2017-02-01

    We report a case of anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis associated with an immature teratoma developed in a 38-year-old woman. Positron emission tomography (PET) revealed focal intense uptake of F-fluorodeoxyglucose in an area of the brain corresponding to the right medial temporal lobe as well as an intrapelvic tumor. After the PET examination, the patient complained of disorientation and short-term memory loss. The ovarian tumor was resected and diagnosed as an immature teratoma. The cerebrospinal fluid analysis was positive for anti-NMDAR antibody. After surgery, the patient's neurologic symptoms improved. The PET finding of encephalitis associated with an immature teratoma was unexpected.

  14. Antenatal Sonographic Diagnosis of Pharyngeal Teratoma: Our Experience of a Rare Case with Review of the Literature

    PubMed Central

    Varras, M.; Akrivis, Ch.; Plis, Ch.; Tsoukalos, G.

    2009-01-01

    Background. Teratomas are the most common tumors. They are usually localized in the sacrococcygeal area, while the pharyngeal localization is very rare. The number of cases of stomatopharyngeal teratomas detected prenatally via sonography is very small. Case Report. We present the case of a 24-year-old primipara at 18 weeks' gestation, that at the routine ultrasound scan, the fetus was found with an echogenic mass, filling the stomatopharyngeal cavity and protruding from the mouth. Other abnormalities were not found. Termination of pregnancy was achieved using misoprostol. A female stillborn fetus with a weight of 250 g and length of 25.5 cm was delivered. The postmortem and pathologic examination confirmed the diagnosis. Conclusion. Pharyngeal teratomas can be diagnosed with the use of ultrasounds in utero facilitating parents' counseling in early time. PMID:19936119

  15. Risk predictors of retroperitoneal hemorrhage following percutaneous coronary intervention.

    PubMed

    Tiroch, Klaus A; Arora, Nipun; Matheny, Michael E; Liu, Christopher; Lee, Timothy C; Resnic, Frederic S

    2008-12-01

    Retroperitoneal hemorrhage (RPH) is a potentially catastrophic complication after percutaneous coronary intervention (PCI). Previous studies identified female gender, body surface area, and high arterial puncture location as independent risk factors for RPH. There have been conflicting reports regarding the association with vascular closure devices (VCDs). Chronic renal insufficiency (CRI) and diabetes mellitus have been associated with both peripheral vascular disease and vascular access-site complications. The putative association of VCDs, CRI, and diabetes mellitus with RPH in the contemporary PCI era was investigated. A total of 3,062 consecutive patients undergoing 3,482 PCIs at Brigham and Women's Hospital from January 2005 to April 2007 were evaluated for the study. All 3,311 patients with femoral angiography underwent hand-caliper-based quantitative vascular analysis and were included in this analysis. Multivariate analysis was performed using a backwards selection algorithm, and a propensity adjustment was developed to control for possible confounding variables regarding VCD use. The incidence of RPH was 0.49% (17 of 3,482 patients). After multivariate and propensity analyses, covariates that significantly influenced the risk of RPH were CRI, glycoprotein IIb/IIIa inhibitors, and high arterial puncture (p < or =0.007). VCD use was not independently associated with the development of RPH (p = 0.74). In conclusion, this large prospective cohort study identified CRI, but not VCD use, as an independent predictor for RPH and peripheral vascular disease.

  16. Endovascular Embolization of Spontaneous Retroperitoneal Hemorrhage Secondary to Anticoagulant Treatment

    SciTech Connect

    Isokangas, Juha-Matti Peraelae, Jukka M.

    2004-11-15

    The purpose of this study was to report a single hospital's experience of endovascular treatment of patients with retroperitoneal hemorrhage (RPH) secondary to anticoagulant treatment. Ten consecutive patients treated in an intensive care unit and needing blood transfusions due to RPH secondary to anticoagulation were referred for digital subtraction angiography (DSA) to detect the bleeding site(s) and to evaluate the possibilities of treating them by transcatheter embolization. DSA revealed bleeding site(s) in all 10 patients: 1 lumbar artery in 4 patients, 1 branch of internal iliac artery in 3 patients and multiple bleeding sites in 3 patients. Embolization could be performed in 9 of them. Coils, gelatin and/or polyvinyl alcohol were used as embolic agents. Bleeding stopped or markedly decreased after embolization in 8 of the 9 (89%) patients. Four patients were operated on prior to embolization, but surgery failed to control the bleeding in any of these cases. Abdominal compartment syndrome requiring surgical or radiological intervention after embolization developed in 5 patients. One patient died, and 2 had sequelae due to RPH. All 7 patients whose bleeding stopped after embolization had a good clinical outcome. Embolization seems to be an effective and safe method to control the bleeding in patients with RPH secondary to anticoagulant treatment when conservative treatment is insufficient.

  17. [A case of seronegative spondylarthropathy with iritis and retroperitoneal fibrosis].

    PubMed

    Takahashi, M; Ishikawa, A; Kondo, H

    1999-02-01

    In 1985 a 41-year old male visited a local hospital because of congestion in the bulbar conjunctiva, which was diagnosed as iritis. In August 1990, right coxalgia and arthralgia of metatarsophalangeal joints appeared, with recurrence of iritis. In October, stiffness in the hands and arthralgia of proximal interphalangeal joints also started. In July 1991, the right coxalgia worsened, resulting in walking difficulty. He was admitted to the Kitasato University Hospital. He presented with bilateral iritis, polyarthritis with limited ranges of motion and sacroilitis. The Schober's test was positive at 3 cm. Serological tests for rheumatoid factor and HLA-B 27 were negative. Abdominal computer tomographic scan revealed low density lesion around the aorta. PSL 10 mg was initiated, and iritis and arthritis remitted. Progression of the periaortic lesion was not observed during the subsequent 5 years. In this case, iritis preceded limited ranges of motion in the vertebrae and sacroilitis. From these findings, seronegative spondylarthropathy with peripheral arthritis was diagnosed. The periaortic lesion seen in this case probably corresponds to chronic periaortitis recently reported as a subset of idiopathic retroperitoneal fibrosis. The two lesions observed in the present case may be interpreted as caused by inflammation of the connective tissue initially either at the vertebrae or around the aorta, which had advanced to involve the other lesion.

  18. Laparoendoscopic single-site versus conventional laparoscopic surgery for ovarian mature cystic teratoma

    PubMed Central

    Park, Jeong-Yeol; Kim, Dae-Yeon; Suh, Dae-Shik; Kim, Jong-Hyeok

    2015-01-01

    Objective To compare the intraoperative and postoperative outcomes of laparoendoscopic single-site surgery (LESS) versus conventional laparoscopic surgery in women with ovarian mature cystic teratoma. Methods A retrospective review of 303 women who underwent LESS (n=139) or conventional laparoscopic surgery (n=164) due to ovarian mature cystic teratoma was performed. Intra- and postoperative outcomes were compared between the two groups. Results There was no intergroup difference in age, body weight, height, body mass index, comorbidities, tumor size, bilaterality of tumor, or the type of surgery. However, more patients in the LESS group had a history of previous abdominal surgery (19.4% vs. 6.7%, P=0.001). Surgical outcomes including operating time (89 vs. 87.8 minutes, P=0.734), estimated blood loss (69.4 vs. 68.4 mL, P=0.842), transfusion requirement (2.2% vs. 0.6%, P=0.336), perioperative hemoglobin level change (1.3 vs. 1.2 g/dL, P=0.593), postoperative hospital stay (2.0 vs. 2.1 days, P=0.119), and complication rate (1.4% vs. 1.8%, P=0.999) did not differ between LESS and conventional groups. Postoperative pain scores measured using a visual analogue scale were significantly lower in the LESS group at 8 hours (P=0.021), 16 hours (P=0.034), and 32 hours (P=0.004) after surgery, and 32 of 139 patients (23%) in the LESS group and 78 of 164 patients (47.6%) in the conventional group required at least one additional analgesic (P<0.001). Conclusion LESS was feasible and showed comparable surgical outcomes with conventional laparoscopic surgery for women with ovarian mature cystic teratoma. LESS was associated with less postoperative pain and required less analgesia. PMID:26217600

  19. Chromosome 12p abnormalities and IMP3 expression in prepubertal pure testicular teratomas.

    PubMed

    Cornejo, Kristine M; Cheng, Liang; Church, Alanna; Wang, Mingsheng; Jiang, Zhong

    2016-03-01

    Although the histologic appearance of pure testicular teratomas (PTTs) is similar in children and adults, the prognosis is dramatically different. Prepubertal PTTs are rare, with a benign clinical course, whereas the adult cases typically have malignant outcomes. Chromosome 12p abnormalities are seen in most adult testicular germ cell tumors but have not been found in prepubertal PTTs. IMP3 is an oncofetal protein that is highly expressed in many malignancies. Recently, we demonstrated IMP3 is expressed in adult mature testicular teratomas but not in mature ovarian teratomas. The aim of this study was to evaluate prepubertal PTTs for chromosome 12p abnormalities and expression of IMP3. A total of 11 cases (excision, n=1; orchiectomy, n=10) were obtained from the surgical pathology archives of 2 large medical centers (1957-2013). All 11 cases were investigated for isochromosome 12p and 12p copy number gain using interphase fluorescence in situ hybridization analysis and were examined by immunohistochemistry for IMP3 expression. Patients ranged in age from 0.9 to 7.0 (mean, 2.4) years. A positive immunohistochemical stain for IMP3 (cytoplasmic staining) was identified in 5 (46%) of 11 cases. Isochromosome 12p was detected in 2 cases (18%) that also expressed IMP3. Somatic copy number alterations of 12p were not observed (0%). We are the first to describe 12p abnormalities and IMP3 expression in prepubertal PTTs. Our data demonstrate a small subset of PTTs harbor typical molecular alterations observed in adult testicular germ cell tumors. Although prepubertal PTTs are considered to be benign neoplasms, it may be a heterogeneous group.

  20. Mediastinal teratoma in a free-ranging American black bear (Ursus americanus).

    PubMed

    Munk, Brandon A; Turner, J Chris; Keel, M Kevin

    2013-12-01

    A 2.75-yr-old female American black bear (Ursus americanus) was found emaciated and unable to rise. It was euthanized due to the perceived poor prognosis. An approximately 18 x 8 x 10-cm, multiloculated, well-demarcated mass that extended from the bifurcation of the trachea, laterally displacing the lung lobes and caudally displacing the heart. The mass contained fibrous connective tissue, cartilage, bone, nervous tissue, smooth muscle, and a variety of epithelial structures. A mediastinal teratoma was diagnosed based on the microscopic features of the neoplasm.

  1. [Benign pre-sacral teratoma and vestigial retrorectal cysts in the adult].

    PubMed

    Chêne, G; Voitellier, M

    2006-01-01

    Benign mature presacral teratomas or dermoid cysts occur rarely in the adult; the incidence is estimated at less than 1/40,000. They are considered to be retrorectal vestigial growths from embryonal rests; they are usually asymptomatic and discovered by chance. However, since they have a 20-30% risk of secondary infection and a 1-12.5% risk of malignant degeneration, they should undergo complete surgical resection. We present a case of presacral dermoid cyst in a young male and summarize the diagnostic and therapeutic management.

  2. An Uncommon Case of Adolescent Ovarian Teratoma Incarcerated in Inguinal Hernia.

    PubMed

    Pensabene, Marco; Girgenti, Vincenza; Cimador, Marcello; Li Voti, Giuseppe; Rodolico, Vito; Siracusa, Fortunato

    2016-01-01

    Mature cystic teratoma is the most frequent benign ovarian neoplastic lesion in adolescents and is generally composed of fully differentiated tissue arising from multipotential three germinal layers. It accounts for approximately 50% of benign ovarian tumors in childhood. Rarely, a bilateral, synchronous, or metachronous presentation can be observed, supporting a conservative approach as the first surgical approach. We report a case of an ovarian mature cystic incarcerated in indirect inguinal hernia in a 15-year-old girl undergoing ovary-sparing surgery. To our knowledge this is the first case of such lesion incarcerated in an inguinal hernia reported in literature.

  3. TERATOMA OF THE OVARY IN A FREE-RANGING JAPANESE RACCOON DOG (NYCTEREUTES PROCYONOIDES VIVERRINUS).

    PubMed

    Yoshimura, Hisashi; Yamamoto, Masami; Moriya, Maiko; Endo, Tomohiko; Sugiura, Natsuko; Kato, Takuya; Matsuda, Yoko; Ishiwata, Toshiyuki; Kajigaya, Hiroshi; Kamiya, Shinji

    2017-03-01

    A young adult, female, free-ranging Japanese raccoon dog ( Nyctereutes procyonoides viverrinus) with scabies infection was found dead as a result of traumatic injuries presumed to reflect vehicular trauma. Necropsy showed a large solid mass located on the left ovarian region, occupying a third of the abdominal cavity. Histologically, the mass contained complex tissues derived from three germinal layers, with areas of cuboidal or columnar epithelium, keratinized squamous epithelium, bone, cartilage, and adipose tissue. This paper presents the first morphologic description of ovarian teratoma in a raccoon dog.

  4. An Uncommon Case of Adolescent Ovarian Teratoma Incarcerated in Inguinal Hernia

    PubMed Central

    Girgenti, Vincenza; Cimador, Marcello; Li Voti, Giuseppe

    2016-01-01

    Mature cystic teratoma is the most frequent benign ovarian neoplastic lesion in adolescents and is generally composed of fully differentiated tissue arising from multipotential three germinal layers. It accounts for approximately 50% of benign ovarian tumors in childhood. Rarely, a bilateral, synchronous, or metachronous presentation can be observed, supporting a conservative approach as the first surgical approach. We report a case of an ovarian mature cystic incarcerated in indirect inguinal hernia in a 15-year-old girl undergoing ovary-sparing surgery. To our knowledge this is the first case of such lesion incarcerated in an inguinal hernia reported in literature. PMID:27525145

  5. Increased 99mTc-MDP Activity in a Partially Calcified Malignant Mediastinal Teratoma.

    PubMed

    Li, Wei; Zhang, Linqi; Zhang, Rusen

    2016-02-01

    A 41-year-old woman presented with cough and shortness of breath for 3 weeks. Chest x-ray and CT showed a large, partially calcified soft tissue mass adjacent to the right side of the heart. Whole-body bone was acquired to evaluate possible metastases, which showed abnormal accumulation of Tc-MDP in the right chest. Further SPECT/CT imaging that demonstrated intense Tc-MDP activity was mainly in the calcification portion of mass. Histopathological examination from biopsy specimen of the lesion was consistent with malignant teratoma.

  6. [Retroperitoneal fibrosis and arthritis--a manifestation of the same illness].

    PubMed

    Thiele, A; Störkel, S; Stierle, H E

    1998-10-01

    Retroperitoneal fibrosis is a disorder in which the retroperitoneal fat is the site of a subacute and chronic inflammatory reaction and is subsequently replaced by dense fibrotic tissue. Rheumatoid nodules are chronic granulomata occurring at sites of pressure and movement, both near the body surface and internally. A 55-year-old sales-manager was admitted to radiation synovectomy after a 5 year history of excessive right and left knee effusions. There were no other clinical or laboratory abnormalities. The patient did not respond to either radioisotope synoviorthesis using radioactive Yttrium (90 Y), or to open synovectomy and prostetic surgery of the right knee. One year later, surgery of left ureter was necessary. Histological findings revealed the diagnosis of Ormond's disease. Comparative histological studies of synovial membrane of knee and retroperitoneal tissues showed local necrosis, fibrin deposition, lining cell proliferation, and infiltration by lymphocytes. Diagnosis of arthritis complicated by retroperitoneal rheumatoid nodules and retroperitoneal fibrosis was made. Serum rheumatoid factor has been negative. For the last 3 years, the patient has been on successful therapy with azathioprine. Rheumatoid nodules of the retroperitoneum have vanished completely and frequency of knee effusions decreased.

  7. Effect of Cross-Sex Hormonal Replacement on Antioxidant Enzymes in Rat Retroperitoneal Fat Adipocytes

    PubMed Central

    Velázquez Espejel, Rodrigo; Cabrera-Orefice, Alfredo; Uribe-Carvajal, Salvador; Pavón, Natalia

    2016-01-01

    We report the effect of cross-sex hormonal replacement on antioxidant enzymes from rat retroperitoneal fat adipocytes. Eight rats of each gender were assigned to each of the following groups: control groups were intact female or male (F and M, resp.). Experimental groups were ovariectomized F (OvxF), castrated M (CasM), OvxF plus testosterone (OvxF + T), and CasM plus estradiol (CasM + E2) groups. After sacrifice, retroperitoneal fat was dissected and processed for histology. Adipocytes were isolated and the following enzymatic activities were determined: Cu-Zn superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione-S-transferase (GST), and glutathione reductase (GR). Also, glutathione (GSH) and lipid peroxidation (LPO) were measured. In OvxF, retroperitoneal fat increased and adipocytes were enlarged, while in CasM rats a decrease in retroperitoneal fat and small adipocytes are observed. The cross-sex hormonal replacement in F rats was associated with larger adipocytes and a further decreased activity of Cu-Zn SOD, CAT, GPx, GST, GR, and GSH, in addition to an increase in LPO. CasM + E2 exhibited the opposite effects showing further activation antioxidant enzymes and decreases in LPO. In conclusion, E2 deficiency favors an increase in retroperitoneal fat and large adipocytes. Cross-sex hormonal replacement in F rats aggravates the condition by inhibiting antioxidant enzymes. PMID:27630756

  8. Type IV Ehlers-Danlos Syndrome: A Surgical Emergency? A Case of Massive Retroperitoneal Hemorrhage.

    PubMed

    Chun, Stephen G; Pedro, Patrick; Yu, Mihae; Takanishi, Danny M

    2011-01-01

    Retroperitoneal hemorrhagic bleeding is a known manifestation of Type-IV Ehlers-Danlos Syndrome that is caused by loss-of-function mutations of the pro-alpha-1 chains of type III pro-collagen (COL3A1) resulting in vascular fragility. A number of previous reports describe futile surgical intervention for retroperitoneal bleeding in Type-IV Ehlers-Danlos Syndrome with high post-operative mortality, although the rarity of retroperitoneal bleeding associated with Type-IV Ehlers-Danlos Syndrome precludes an evidence-based approach to clinical management. We report a 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome who presented with severe abdominal pain and tachycardia following an episode of vomiting. Further work-up of his abdominal pain revealed massive retroperitoneal bleeding by CT-scan of the abdomen. Given numerous cases of catastrophic injury caused by surgical intervention in Type-IV Ehlers-Danlos Syndrome, the patient was treated non-operatively, and the patient made a full recovery. This case suggests that even in cases of large retroperitoneal hemorrhages associated with Ehlers-Danlos Syndrome, it may not truly represent a surgical emergency.

  9. A Squamous Cell Carcinoma and a Malignant Fibrous Histiocytoma Developing in a Patient with a Mature Cystic Teratoma

    PubMed Central

    Tahaoglu, Ali Emre; Ege, Serhat; Bakir, Mehmet Sait; Saruhan, Gülbin; Aksin, Serif; Gul, Talip

    2017-01-01

    We present a very rare case of malignant transformation of a benign mature cystic teratoma. The pathology report revealed malignant transformation of both the epithelial and sarcomatous elements of a benign dermoid cyst. To the best of our knowledge, this appears to be the third case of a malignant fibrous histiocytoma and a squamous cell carcinoma developing from a mature cystic teratoma. Malignant transformation of a dermoid cyst is usually diagnosed postmenopausally, but our patient was premenopausal. The etiology and prognosis of malignant transformation of this benign condition remain unknown. PMID:28203171

  10. The unexpected finding of a benign mature teratoma in a forensic pathology autopsy: a rare cause for sudden, unexpected death.

    PubMed

    Høyer, Christian Bjerre; Ulhøi, Benedicte Parm; Charles, Annie Vesterby

    2013-12-01

    Intracranial teratomas are rare tumors that are usually discovered in infancy due to progressive symptoms. We describe a case of a 38-year-old man who was found dead 9 hours after the last sign of life. The deceased's medical history could not explain the sudden, unexpected death. A forensic autopsy revealed an asymptomatic, mature teratoma in the left frontal and temporal lobes. We concluded that the cause of death must have been a generalized epileptiform seizure originating in the tumor site(s) leading to aspiration of the stomach contents and unfavorable positioning, resulting in asphyxia.

  11. Traumatic retroperitoneal hematoma illustrated on Tc-99m methylene diphosphonate bone scintigraphy in a patient presenting with a fall.

    PubMed

    Nguyen, Huong Van; Chiam, Quee Li; Dixson, Hugh; Goddard, Kim A

    2007-08-01

    Retroperitoneal haematoma is rarely described in the context of bone scintigraphy, as it is usually an incidental discovery on bone scan carried out for another purpose. We report a case of a right retroperitoneal haematoma detected on Tc-99m-methylene diphosphonate bone scintigraphy taken in an elderly patient presenting with a mechanical fall and a right acetabular fracture.

  12. An unusual sarcomatous retroperitoneal metastasis. A rare case report with a brief literature review.

    PubMed

    Lazar, A M; Straja, N D; Brătucu, E

    2012-01-01

    Retroperitoneal sarcomas are rare tumors associated with a high rate of recurrence and very bad prognosis. Their only efficient treatment is a negative- margin surgical resection that is extremely difficult to achieve. Retroperitoneal metastases from extremity sarcomas are considered unusual. In literature, such a metastatic pattern is described extremely rarely. In this paper we report a case of a very aggressive extremity chondrosarcoma, associated with local recurrence, multiple distant metastases, that finally led to a retroperitoneal metastasis. The recurrence and progression of the sarcoma in this localization were impressive, with a fast overcome of therapeutic options. Chemo- and radiotherapy have not proved to be efficacious in this context and they could have had a role in the deterioration of patient state of health. New tumor markers for the detection and follow-up of these tumors should be considered.

  13. Retroperitoneal liposarcomas: a representative literature review occasioned by a rare case of laterelapse abdominal liposarcoma.

    PubMed

    Pisani, Michela; Al-Buheissi, Salah; Whittlestone, Tim

    2016-05-24

    Soft tissue sarcomas (STS) are rare and heterogeneous tumours representing approximately 0.7%-1% of all adult tumours. In the adults and among the retroperitoneal sarcomas (RPS), Liposarcoma (LS) is the most common variant accounting for 12% -20% of all sarcomas and up to 45% of sarcomas at retroperitoneal localization. A rare case of LS relapsed after 15 years is giving the occasion to review the published literature and emphasise the followings concepts: 1) Despite extensive surgery remains the mainstay of treatment for localized STS at present, anatomical complexity and occult localization result in local recurrence in the majority of patients; 2) The role of imaging and tumour markers is still limited; 3) Indefinite prolonged surveillance is a key point of treatment; 4) Referral to tertiary centres with dedicated Retroperitonal Surgeons and Oncology expertise is mandatory.

  14. An unusual case of retroperitoneal accessory spleen with vascular supply directly from the aorta.

    PubMed

    Souparis, A; Papaziogas, B; Alexandrakis, A; Koutelidakis, J; Paraskevas, G; Papaziogas, T

    2002-08-01

    We report on a case of a retroperitoneal accessory spleen with vascular supply directly from the aorta. A 47-year old woman presented with a six-month history of epigastric pain, intermittent nausea and vomiting. The CT scan of the abdomen revealed the presence of a retroperitoneal tumor between the spleen, left kidney and pancreas. The MR-tomography confirmed the presence of a solid tumor, with vascular supply directly from the aorta. The exploratory laparotomy showed the presence of a well-shaped accessory spleen, which was confirmed by the histological examination of the specimen. The surgeon should be aware of the possible existence of accessory spleens for the differential diagnosis of retroperitoneal tumors.

  15. Anesthetic management of a patient with giant retroperitoneal liposarcoma: case report with literature review

    PubMed Central

    Feng, Dandan; Xu, Fangxia; Wang, Meng; Gu, Xiaoping; Ma, Zhengliang

    2015-01-01

    Patients with giant retroperitoneal liposarcomas are considered at great risks of perioperative complications and require meticulous anaesthetic management. There have been few reports about anaesthetic management of giant retroperitoneal liposarcoma. We present the case of a 66-year-old patient who suffered from a giant retroperitoneal liposarcoma (diameter 45 cm and weigh 4.5 kg), needing a resection surgery under general anesthesia. We successfully managed anesthesia procedures in this patient using FloTrac/VigileoTM monitoring system without major perioperative complications. The surgery was completed uneventfully and the patient recovered smoothly. After reviewing the literature, we summarize FloTrac/VigileoTM monitoring system is useful to help anaesthesiologists adjust infusion rate to maintain the stability of circulatory state. Anesthetic monitoring, fluid management and temperature control need to be focused in the anesthetic management. PMID:26770605

  16. Intrapericardial teratoma in a low birth weight preterm infant: a successful multidisciplinary approach.

    PubMed

    Iacona, Gabriele M; Barber, Miguel A; Medina, Margarita; Abella, Raul

    2011-02-01

    We report the case of an intrapericardial teratoma, a rare tumour in infants, prenatally diagnosed and successfully treated thanks to a multidisciplinary approach. An intrapericardial cystic mass (2.5 cm) with pericardial effusion was identified in a foetus at 32 weeks gestational age (GA). Intrauterine pericardiocentesis was performed immediately (40 ml) and repeated at 33 weeks (25 ml) and then at 34 weeks GA, just before birth (36 ml). Considering the rapid growth of the mass and the risk of hydrops, vaginal delivery was induced. A baby girl weighing 1.98 kg was born without cardiorespiratory compromise. Echocardiography and thoracic CT-scan located the 4.0×3.0 cm cystic mass between the left atrial appendage and the left superior pulmonary vein. At three days of life, the mass was completely removed without cardiobypass. It arose from the ascending aorta. Postoperative course was uneventful. Pathology diagnosed an immature intrapericardial teratoma. As long-term follow-up is required, alpha-fetoprotein can be a valid tool to monitor a possible recurrence. A multidisciplinary approach allows successful prenatal management and postnatal tumour surgery.

  17. Large Gastric Teratoma: A Rare Intra-abdominal Mass of Infancy

    PubMed Central

    Hasan, Roumina; Monappa, Vidya; Kumar, Sandeep; Kumar, Vijay

    2016-01-01

    Amongst the varied, diverse causes of intraabdominal masses in infancy and early childhood, gastric teratomas (GTs) account for a very small proportion. A worldwide literature search reveals only around one hundred cases of GT and also supports the fact that its preoperative diagnosis remains elusive. Here we report the case of a two-month-old male who presented to the pediatric surgery outpatient department of Kasturba Medical College and Hospital, Karnataka, India, with progressive distension of abdomen since birth. Clinically, a large firm, non-mobile and non-tender mass involving all four quadrants of the abdomen was seen. Ultrasound revealed a large solid-cystic mass with internal septations extending from the epigastrium up to the pelvis. Computed tomography revealed a large intraperitoneal fat containing solid-cystic mass lesion showing curvilinear and chunky areas of calcification, with the mass focally indenting the posterior gastric wall and showing focal polypoidal intragastric extension. Exploratory laparotomy revealed a large cystic tumor with a solid component, arising from lesser curvature of the stomach, showing focal intraluminal extension across the posterior gastric wall, and occupying the whole lesser sac and abdominal cavity. The tumor was excised in toto along with the body of the stomach. Histopathological examination showed mature tissue derived from all three germ cell layers and confirmed the diagnosis of mature gastric teratoma. The patient was disease free at one-year follow-up. PMID:27162596

  18. CREPT expression correlates with poor prognosis in patients with retroperitoneal leiomyosarcoma

    PubMed Central

    She, Yaoguang; Liang, Jiao; Chen, Lin; Qiu, Ying; Liu, Na; Zhao, Xudong; Huang, Xiaohui; Wang, Yinyin; Ren, Fangli; Chang, Zhijie; Li, Peiyu

    2014-01-01

    Retroperitoneal leiomyosarcomas (LMSs) are rare gynecological malignancies that display poor prognosis and high mortality. Cell cycle-related and expression-elevated protein in tumor (CREPT) is an oncogene that is involved in the regulation of many cell cycle-related proteins. However, its distribution and clinical significance in retroperitoneal LMS remains poorly understood. This study assessed the histological classifications of postoperative tumor samples from 71 cases of retroperitoneal LMS that were collected at The General Hospital of the People’s Liberation Army from January 1998 to December 2012. We found that more than half of the patients displayed positive expressions of CREPT, Ki-67 and PCNA via immunohistochemical analysis. The expression of CREPT correlated with histological grade (P = 0.044), and the PCNA expression level correlated with the differentiation of tumor cells and histological grade (P < 0.001 and P = 0.009, respectively). Multivariate analysis showed that survival was associated with histological grade and the expression level of CREPT (P = 0.011 and P = 0.012, respectively). Kaplan-Meier analysis showed that the patients lacking CREPT expression exhibited significantly longer overall postoperative survival (median, 60.0 months) than the patients displaying CREPT expression (median, 33.0 months), and CREPT expression correlated with distant recurrence within 5 years after surgery (P = 0.004). Western blot analyses showed that CREPT was more strongly expressed in the retroperitoneal LMS tumor tissue than in paired control tissue. Based on the above data, we concluded that CREPT displays unique immunostaining for retroperitoneal LMS tissue and can be used to supplement other currently available retroperitoneal LMS markers. PMID:25400738

  19. [Septic portal vein thrombosis as a rare complication of Crohn disease with retroperitoneal abscess].

    PubMed

    Diehl, S J; Lehmann, K J; Manthe, S; Georgi, M

    1996-03-01

    Portal vein thrombosis is a rare complication of inflammatory bowel disease and occurs particularly in patients with ulcerative colitis. This report describes a patient with known Crohn's disease since 1980 who developed retroperitoneal abscesses and subsequently a septic portal vein thrombosis. After 10 years of remission, clinical deterioration, jaundice, and fever occurred. An abdominal CT-study confirmed the ultrasonic presumptive diagnosis of two retroperitoneal abscesses close to the pancreas tail. Another CT, one week later, showed furthermore a portal vein thrombosis. Only by intensive care treatment was it possible to control the critical situation.

  20. Challenges in the management of a rare case of extensive retroperitoneal haemangioma in a pregnant woman

    PubMed Central

    Tan, Shu-Qi; Lim, Jason Shau Khng; Tan, Yin Ru; Tan, Hak Koon

    2014-01-01

    Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient’s delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature. PMID:25631977

  1. Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

    PubMed Central

    Chen, Fan; Wang, Xiangyu; Wang, Yang; Meng, Hui; Hou, Xinguo; Zhu, Yaofeng; Gao, Wei; Jiang, Xuewen; Chen, Shouzhen; Zhang, Zhaocun; Zou, Zhichuan; He, Tianyi; Yang, Yue; Zhu, Kejia; Wang, Yong; Liu, Yaxiao; Cui, Jianfeng; Shi, Benkang; Yin, Gang

    2016-01-01

    Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing’s syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup. PMID:27695589

  2. Management of a large retroperitoneal primitive neuroectodermal tumour: 'a multimodal approach'.

    PubMed

    Sable, Shailesh; Gandhi, Vidhyachandra; Nagral, Aabha; Nagral, Sanjay

    2012-02-25

    Retroperitoneal primitive neuroectodermal tumour (PNET) is a rare disease having poor prognosis. Treatment mainly consists of en block resection of the tumour to achieve RO resection, however multimodal approach has also been used with improved survival. The authors report a 40-year-old male with large retroperitoneal PNET adherent to aorta and compressing the inferior vena cava. He was subjected to P6 protocol chemotherapy (cyclophosphamide, adriamycin, vincristine, ifosfamide and etoposide) in view of borderline operability. Postchemotherapy contrast enhanced CT revealed significant reduction in size of the lesion. He underwent complete resection of the tumour followed by concurrent chemoradiation. He remains asymptomatic on follow-up over a period of 2 years.

  3. Challenges in the management of a rare case of extensive retroperitoneal haemangioma in a pregnant woman.

    PubMed

    Tan, Shu-Qi; Lim, Jason Shau Khng; Tan, Yin Ru; Tan, Hak Koon

    2014-11-01

    Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient's delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature.

  4. Prenatal diagnosis of anasarca in an end-second trimester fetus presenting with sacrococcygeal teratoma by magnetic resonance imaging.

    PubMed

    Nassenstein, Kai; Schweiger, Bernd; Barkhausen, Joerg

    2006-09-01

    Fetal hydrops is a known complication of sacrococcygeal teratoma (SCT) due to arteriovenous shunts within the tumor, which is clinically important and influence patients' prognosis. We present unique MRI morphological findings of a marked fetal anasarca in a case of an end-second trimester fetus presenting with SCT.

  5. Endoscopic Resection of Skull Base Teratoma in Klippel-Feil Syndrome through Use of Combined Ultrasonic and Bipolar Diathermy Platforms.

    PubMed

    Edward, Justin A; Psaltis, Alkis J; Williams, Ryan A; Charville, Gregory W; Dodd, Robert L; Nayak, Jayakar V

    2017-01-01

    Klippel-Feil syndrome (KFS) is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken. Out of concern for vascular control of the fibrofatty dense tumor stalk at the skull base and need for complete teratoma resection, we successfully employed a tissue resection tool with combined ultrasonic and bipolar diathermy to the tumor pedicle at the sphenoid/clivus junction. No CSF leak or major hemorrhage was noted using this endonasal approach, and no concerning postoperative sequelae were encountered. The patient continues to do well now 3 years after tumor extirpation, with resolution of all preoperative symptoms and absence of teratoma recurrence. KFS, teratoma biology, endocrine gland duplication, and the complex considerations required for successfully addressing this type of advanced skull base pathology are all reviewed herein.

  6. Endoscopic Resection of Skull Base Teratoma in Klippel-Feil Syndrome through Use of Combined Ultrasonic and Bipolar Diathermy Platforms

    PubMed Central

    Psaltis, Alkis J.; Williams, Ryan A.; Charville, Gregory W.; Dodd, Robert L.

    2017-01-01

    Klippel-Feil syndrome (KFS) is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken. Out of concern for vascular control of the fibrofatty dense tumor stalk at the skull base and need for complete teratoma resection, we successfully employed a tissue resection tool with combined ultrasonic and bipolar diathermy to the tumor pedicle at the sphenoid/clivus junction. No CSF leak or major hemorrhage was noted using this endonasal approach, and no concerning postoperative sequelae were encountered. The patient continues to do well now 3 years after tumor extirpation, with resolution of all preoperative symptoms and absence of teratoma recurrence. KFS, teratoma biology, endocrine gland duplication, and the complex considerations required for successfully addressing this type of advanced skull base pathology are all reviewed herein. PMID:28133560

  7. An unusual case of Cat-Eye syndrome phenotype and extragonadal mature teratoma: review of the literature.

    PubMed

    Tzetis, Maria; Stefanaki, Kalliopi; Syrmou, Areti; Kosma, Konstantina; Leze, Eleni; Giannikou, Krinio; Oikonomakis, Vasilis; Sofocleous, Christalena; Choulakis, Michael; Kolialexi, Aggeliki; Makrythanasis, Periklis; Kitsiou-Tzeli, Sophia

    2012-07-01

    BACKGROUND Cat-Eye syndrome (CES) with teratoma has not been previously reported. We present the clinical and molecular findings of a 9-month-old girl with features of CES and also a palpable midline neck mass proved to be an extragonadal mature teratoma, additionally characterized by array comparative genomic hybridization (aCGH). RESULTS High resolution oligonucleotide-based aCGH confirmed that the supernumerary marker chromosome (SMC) derived from chromosome 22, as was indicated by molecular cytogenetic analysis with fluorescence in situ hybridization (FISH). Additionally, aCGH clarified the size, breakpoints, and gene content of the duplication (dup 22q11.1q11.21; size:1.6 Mb; breakpoints: 15,438,946-17,041,773; hg18). The teratoma tissue was also tested with aCGH, in which the CES duplication was not found, but the analysis revealed three aberrations: del Xp22.3 (108,864-2788,689; 2.7 Mb hg18), dup Yp11.2 (6688,491-7340,982; 0.65 Mb, hg18), and dup Yq11.2q11.23 (12,570,853-27,177,133; 14.61 Mb, hg18). These results indicated 46 XY (male) karyotype of the teratoma tissue, making this the second report of mature extragonadal teratoma in a female neonate, probably deriving from an included dizygotic twin of opposite sex (fetus in fetu). CONCLUSIONS Our findings extend the phenotypic spectrum of CES syndrome, a disorder with clinical variability, pointing out specific dosage-sensitive genes that might contribute to specific phenotypic features.

  8. [Factors predictive of retroperitoneal lymph node metastasis in endometrial cancer].

    PubMed

    Urzal, Cecília; Sousa, Rita; Baltar, Vítor; Correia, Paulo; Cruz, Eugénia; Pereira da Silva, Daniel

    2014-01-01

    Introdução: Tem sido sugerido que o estadiamento completo possa ser omitido com segurança em doentes com carcinoma do endométrio e baixo risco de metástases ganglionares. Os objectivos do nosso trabalho foram a exploração do significado prognóstico de factores patológicos para disseminação ganglionar pélvica e para-aórtica e a validação do algoritmo da Clínica Mayo, de forma a identificar as doentes em que a linfadenectomia possa ser dispensada.Material e Métodos: Efectuámos um estudo retrospectivo incluindo 208 doentes, para a avaliação de variáveis tumorais patológicas e metástases ganglionares. A análise estatística foi realizada através dos testes qui-quadrado, exacto de Fisher e t de Student.Resultados: A invasão miometrial > 50% (p < 0,001), a invasão do colo (p = 0,001), a invasão dos espaços linfovasculares (p = 0,003) e a citologia peritoneal positiva (p = 0,03) constituíram preditores significativos de disseminação ganglionar retroperitoneal. A metastizaçãoganglionar pélvica foi preditiva de metastização ganglionar para-aórtica (p < 0,001).Discussão: O algoritmo da Clínica Mayo identificou as doentes sem metástases nos gânglios pélvicos ou para-aórticos com um valor preditivo negativo de 98,4% (61/62). A combinação de invasão miometrial ≤ 50% e ausência de invasão cervical ou linfovascular apresentou um valor preditivo negativo de 98,8% (79/80).Conclusão: Apesar de os critérios da Clínica Mayo predizerem uma probabilidade muito reduzida de metástases ganglionares retroperitoneais, a combinação de invasão miometrial ≤ 50% e ausência de invasão cervical ou linfovascular teria evitado a linfadenectomia num número superior de mulheres.

  9. Surgical ‘damage control’ treatment of a large retroperitoneal liposarcoma encasing a horseshoe kidney

    PubMed Central

    Andreoni, B; Chiappa, A; Pace, U; Bertani, E; Verweij, F; Orsi, F; Petralia, G; Tullii, M; Venturino, M; Pelosi, G

    2008-01-01

    Damage control is a surgical strategy for severely compromised trauma patients based on speed control of life-threatening injuries that aims to rapidly resuscitate patients in an intensive care unit (ICU). We report on the use of such therapeutic strategy in a patient affected by a retroperitoneal sarcoma concomitant to a horseshoe kidney, a relatively rare anatomical malformation. PMID:22275967

  10. Liver transplantation surgical techniques for extensive retroperitoneal tumor with major blood vessel involvement: a case report.

    PubMed

    Miura, K; Sato, Y; Kokai, H; Hara, Y; Kobayashi, T; Oya, H; Yamamoto, S; Hatakeyama, K

    2012-03-01

    A case of a 71-year-old man with a huge retroperitoneal tumor situated behind the liver, which strongly compressed the liver inferior vena cava (IVC), and gastrointestinal tract is described. With the techniques of whole liver extraction and autologous orthotopic liver transplantation, we successfully removed the tumor. We have the surgical techniques, essential elements, and indications for this procedure.

  11. [Retroperitoneal liposarcoma as etiology of abdominal pain. Case report and literature review].

    PubMed

    Pérez-Ponce, Yisvanth; Castellanos-Alejandre, Raúl; Guerrero-Romero, J Francisco; Estrada-León, Felipe; Torres-Lobatón, Alfonso

    2008-01-01

    Soft tissue sarcomas are very uncommon types of tumors, with their embryological origin in the mesoderm and in nerve structures of the neuroectodermic layer. They represent only 1.5% of cases in the National Registry of Malignant Tumors in Mexico. They can be encountered anywhere connective soft tissue is found. Because of their specialized localization, retroperitoneal soft tissue sarcomas have a propensity to remain asymptomatic for long periods of time and reach a large size before being diagnosed. The only accepted treatment is wide surgical excision with clear margins, without a clear benefit for adjuvant treatment. The very uncommon nature of these tumors and their varied histopathology, site and behavior classify them as a difficult entity in terms of treatment. We present here the case of a 66-year-old female with a left-side retroperitoneal tumor, complaining only of vague abdominal pain as the presenting symptom. A CT-guided needle biopsy reported a sarcoma and the patient was subjected to laparatomy with complete resection of the tumor (30 x 13 x 10 cm). Histopathological report demonstrated a low-grade retroperitoneal sarcoma and free macroscopic and microscopic borders, without obvious invasion except for left kidney and ureter. The patient refused adjuvant treatment, and she is disease-free 7 years after treatment. Retroperitoneal sarcomas can cause pain and reach very large sizes. The best treatment available is wide surgical resection with clear margins. The most important prognostic factors are free margins, type of resection, age of patient and tumor histology.

  12. Systemic immunologic and inflammatory response after transperitoneal versus retroperitoneal laparoscopic donor nephrectomy: A prospective observational study.

    PubMed

    Gogoi, Debojit; Pal, Dilip Kumar; Bera, Malay K

    2016-01-01

    Laparoscopic donor nephrectomy (LDN) can be performed via either transperitoneal or retroperitoneal approach. Very few studies have been carried out till now, comparing immunologic and inflammatory responses in donors after these two approaches. This is a prospective observational study. Selection of approach was decided by the operating surgeon. All patients underwent peripheral venous blood sampling preoperatively and 24 h postoperatively for the measurement of C-reactive protein (CRP), interleukein-6 (IL-6), total leukocyte count (TLC), blood urea nitrogen (BUN), and serum creatinine (SCr). Operative time, warm ischemia time, hospital stay, requirement of analgesia, and complications were also recorded. From February 2013 to January 2015, we performed 54 LDNs (38 transperitoneal and 16 retroperitoneal). There were 49 females and five males. Mean operative time was not significantly different in these two approaches, but warm ischemia time was significantly less in the retroperitoneal laparoscopic donor nephrectomy (RLN) group. Postoperative inflammatory markers' (IL-6, CRP, and TLC) levels, BUN, and SCr rise in both of these approaches, but there was no significant difference observed between these two approaches. RLN is a safe and effective approach to preserve a longer right renal vein. It combines the benefit of both hand assistance and retroperitoneal approach. Warm ischemic time is significantly less in RLN group.

  13. [Secondary retroperitoneal fibrosis in a 39-year-old man after rectal cancer].

    PubMed

    Jarosch, A; Tiller, M; Rohrbach, H; Leimbach, T; Schepp, W

    2016-05-01

    A 39-year-old man had been treated for rectal cancer 6 years ago by lower anterior resection of the rectum and perioperative radiochemotherapy. Since then follow-up had been unremarkable but now the patient presented with unspecific lower abdominal pain. The cause of the pain was identified as paraneoplastic retroperitoneal fibrosis secondary to metachronous pulmonary metastases of the rectal cancer.

  14. Desperation Postchemotherapy Retroperitoneal Lymph Node Dissection for Metastatic Germ Cell Tumors.

    PubMed

    Carver, Brett S

    2015-08-01

    Patients with persistently elevated serum tumor markers should be monitored for marker kinetics and evaluated for nonviable cancer causes of marker elevation. Desperation postchemotherapy retroperitoneal lymph node dissection is performed in select patients following second-line chemotherapy. Adjuvant postoperative chemotherapy is not indicated in patients following second-line chemotherapy.

  15. Prognostic factors predictive of survival for truncal and retroperitoneal soft-tissue sarcoma.

    PubMed Central

    Singer, S; Corson, J M; Demetri, G D; Healey, E A; Marcus, K; Eberlein, T J

    1995-01-01

    OBJECTIVE: The authors identified prognostic factors relevant to clinical outcomes (especially survival) in truncal and retroperitoneal soft-tissue sarcoma. SUMMARY BACKGROUND DATA: These results can be used to optimize surgical management and select patients most likely to benefit from novel therapeutic strategies in future trials. METHODS: A retrospective analysis was performed of a prospectively compiled database of 183 consecutive patients with truncal and retroperitoneal sarcomas seen at the Brigham and Women's Hospital and the Dana Farber Cancer Institute between 1970 to 1994. RESULTS: For truncal sarcoma, multivariate analysis showed that high-grade histology was associated with an eightfold increased risk of death compared with low-grade histology (p = 0.001). In addition to grade, gross positive margin of resection (p = 0.001), microscopic positive margin (p = 0.023), and tumors greater than 5 cm in size (p = 0.018) were important independent prognostic factors for survival. In this series, postoperative radiation therapy for truncal sarcoma was associated with a 2.4-fold decreased risk of death compared with truncal sarcoma patients receiving no adjuvant radiation therapy, having adjusted for the other prognostic factors (p = 0.030). In contrast, for retroperitoneal sarcoma, multivariate analysis showed that high-grade and intermediate-grade histology were associated with a five- to sixfold increased risk of death compared with low-grade histology (p = 0.009). In addition to grade, gross positive margin of resection (p = 0.001) and microscopic positive margin (p = 0.004) were important independent prognostic factors for survival in retroperitoneal sarcoma. Patients who received either preoperative or postoperative chemotherapy for retroperitoneal sarcoma had a 4.6-fold (p = 0.002) and 3-fold (p = 0.010) increased risk of death, respectively, compared with patients receiving no adjuvant chemotherapy, having adjusted for the other prognostic factors

  16. Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.

    PubMed Central

    Lee, Chang Hun; Kim, Keun Il; Kim, Young Dae; Lee, Min Ki; Kim, Jee Yeon; Park, Do Youn; Sol, Mee Young; Suh, Kang Suek

    2003-01-01

    Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult. PMID:12923341

  17. Repeated 131I treatment of a residual ovarian teratoma containing malignant thyroid tissue.

    PubMed

    Suga, K; Hirabayashi, A; Motoyama, K; Kume, N; Matsunaga, N; Tamura, H; Kato, H

    1999-11-01

    A 49-year-old woman with ovarian teratoma received 131I treatment three times for an unresectable mass containing malignant thyroid tissue after surgery. Repeated 131I treatment effectively reduced serum thyroglobulin (Tg) level and tumour uptake of 131I, despite absence of any change in size of the treated tumour. Treatment did not inhibit the increase of serum CA-125 and tumour 201Tl uptake, associated with progression of a radioresistant intratumoral hyper-perfused tissue component, detected by colour Doppler ultrasound. Serum CA-125 level and tumour 201Tl uptake were not significantly changed despite temporary increases in serum Tg level after each 131I treatment. These observations indicate the importance of diagnostic measures using combined functional imaging and tumour markers in managing this rare tumour.

  18. Robot-assisted laparoscopic retroperitoneal lymph node dissection for stage IIIb mixed germ cell testicular cancer after chemotherapy.

    PubMed

    Lee, Sang Hyub; Kim, Dong Soo; Chang, Sung-Goo; Jeon, Seung Hyun

    2015-07-01

    Laparoscopic retroperitoneal lymph node dissection, especially when performed with the da Vinci Surgical System (Intuitive Surgical), has shown excellent cosmetic results with similar oncologic outcomes to those of open surgery. In this study, we present a case of robot-assisted retroperitoneal lymph node dissection performed in an 18-year-old man who was diagnosed with a stage IIIb mixed germ cell tumor and who was initially treated with radical orchiectomy, followed by chemotherapy. This case shows that robot-assisted retroperitoneal lymph node dissection is technically feasible, safe, and cosmetically favorable, even when performed on patients with high-stage disease or after chemotherapy.

  19. Retroperitoneal Laparoendoscopic Single-Site Ureterolithotomy: A Comparison with Conventional Laparoscopic Surgery

    PubMed Central

    Liu, Xiaopeng; Huang, Huaiqiu; Wu, Jieying; Huang, Wentao; Cai, Songwang; Li, Xiaojuan; Ye, Chunwei; Zhu, Baoyi; Cai, Yi; Gao, Xin

    2012-01-01

    Abstract Background and Purpose Laparoendoscopic single-site (LESS) surgery through the retroperitoneal approach has been seldom reported. We aimed to compare the feasibility and outcomes of LESS and conventional laparoscopic surgery via the retroperitoneal approach in the management of large, impacted ureteral stones. Patients and Methods From June 2010 to May 2011, LESS ureterolithotomy through the retroperitoneal approach was performed in 10 patients (the LESS group). Another 15 patients who underwent conventional retroperitoneal laparoscopic ureterolithotomy (the conventional laparoscopic group) by the same surgeon were involved and compared. The operative time, complications, and surgical outcomes were evaluated. Results All the operations were completed successfully, without conversion to conventional laparoscopic or open surgeries. The operative time of the LESS group and of the conventional laparoscopic group were 132.7±16.3 and 128.1±20.1 minutes, respectively (P=0.782). The estimated blood loss were 30.7±5.9 vs 28.0±4.5 mL (P=0.620). Duration of analgesia postoperatively was 2.0±0.8 vs 3.5±0.5 days (P=0.005). All targeted stones were successfully extracted without major complications. Postoperative urine leakage was noted in one patient in each group. Cosmetic results were superior in the LESS group according to both the study nurse's and the patients' assessments (8.5 vs 5.3; P=0.012, and 8.3 vs 5.6; P=0.025, respectively). All patients showed no obstructions or stricture formations on postoperative follow-up. Conclusions In experienced hands, LESS for ureterolithotomy through the retroperitoneal approach is feasible and can acquire outcomes equal to those of conventional multiport laparoscopic surgery. Prospective long-term follow-up studies with a larger number of patients are needed to further evaluate its benefits. PMID:22103789

  20. Hydrocephalus secondary to chemotherapy in a case of prenatally diagnosed giant immature grade 3 sacrococcygeal teratoma

    PubMed Central

    Sarbu, Ioan; Socolov, Demetra; Socolov, Razvan; Miron, Ingrith; Trandafirescu, Mioara; Diaconescu, Smaranda; Ciongradi, Carmen Iulia

    2016-01-01

    Abstract Introduction: Sacrococcygeal teratoma (SCT) is a rare tumor in the general population, arising from multipotent stem cells. Whereas most of the cases diagnosed postnatally have good prognosis, the rate of mortality and morbidities associated with prenatally diagnosed SCT remain high, with a reported mortality rate of 30% to 50%. The outcome of fetal SCT can be unpredictable, with some cases with slow growth during fetal life, whereas others grow rapidly, causing multiple complications; also, some of these tumor will develop triggering fetal (preterm delivery, high-output cardiac failure, hydrops fetalis, intrauterine death) or maternal complications (distocia, placentomegaly, maternal mirror syndrome—preeclampsia). Even if prenatal criteria seem to define tumors at risk, it can not totally predict postnatal outcome as treatment-related complications can occur. We present a case of giant prenatally detected SCT. The case was diagnosed at 24th week of gestation, and was closely monitored by serial ultrasound. The morphology of the lesion was defined by fetal MRI performed at 25th week of gestation. A baby girl with a huge sacrococcygeal tumor was born and surgery was performed 48 hours later. Pathological examination revealed a grade 3 immature teratoma. Because of the tumor size and pathological aspect, adjuvant chemotherapy was considered. The outcome was complicated by wound infection, sepsis, and subsequent hydrocephalus, induced by chemotherapy-induced immunosuppression. Conclusion: Our case emphasizes not only the importance of prenatal monitoring of these cases but also the importance of individualized postnatal management, as unusual and unpredictable complications can occur and affect outcome. PMID:27787388

  1. Genetics and biology of human ovarian teratomas. II. Molecular analysis of origin of nondisjunction and gene-centromere mapping of chromosome I markers.

    PubMed Central

    Deka, R; Chakravarti, A; Surti, U; Hauselman, E; Reefer, J; Majumder, P P; Ferrell, R E

    1990-01-01

    Chromosomal heteromorphisms and DNA polymorphisms have been utilized to identify the mechanisms that lead to formation of human ovarian teratomas and to construct a gene-centromere map of chromosome 1 by using those teratomas that arise by meiotic nondisjunction. Of 61 genetically informative ovarian teratomas, 21.3% arose by nondisjunction at meiosis I, and 39.3% arose by meiosis II nondisjunction. Eight polymorphic marker loci on chromosome 1p and one marker on 1q were used to estimate a gene-centromere map. The results show clear linkage of the most proximal 1p marker (NRAS) and the most proximal 1q marker (D1S61) to the centromere at a distance of 14 cM and 20 cM, respectively. Estimated gene-centromere distances suggest that, while recombination occurs normally in ovarian teratomas arising by meiosis II errors, ovarian teratomas arising by meiosis I nondisjunction have altered patterns of recombination. Furthermore, the estimated map demonstrates clear evidence of chiasma interference. Our results suggest that ovarian teratomas can provide a rapid method for mapping genes relative to the centromere. Images Figure 1 Figure 2 PMID:1977308

  2. Diagnosis and Management of Spontaneous Lumbar Venous Retroperitoneal Hematoma in Setting of Deep Venous Thrombosis: A Case Report and Algorithm

    PubMed Central

    Tseng, Joseph; Chapman, Todd; Scott, Ryan; Kalinkin, Olga

    2016-01-01

    Retroperitoneal hematoma is rare and benefits from a systematic approach to prevent morbidity and mortality. Management of such bleeds is based upon patient stability, the cause (spontaneous or posttraumatic), and source (arterial or venous). Herein, the authors describe a diagnostic and management algorithm for retroperitoneal hemorrhage with an example of a rare lumbar venous bleed under the complicated clinical setting of deep venous thrombosis. PMID:27795865

  3. Benign cystic mediastinal teratoma presenting as a massive pleural effusion in a 17-year-old boy.

    PubMed

    Montebello, Annalisa; Mizzi, Adrian; Cassar, Paul John; Cassar, Karen

    2017-01-10

    Mediastinal teratomas are a rare, albeit an important differential diagnosis of anterior/middle mediastinal masses in young adults and various atypical presentations have been reported. The authors report a case of a 17-year-old boy who presented with a 2-month history of worsening shortness of breath and pleuritic chest pain. A massive left-sided pleural fluid collection was seen on a chest radiograph (CXR). The pleural fluid was drained and a CT Thorax confirmed the presence of a cystic mass. Following re-accumulation of the fluid, thoracotomy was performed and a benign mediastinal teratoma excised. The patient remained well with no evidence of recurrence on follow-up CXRs a year post operatively.

  4. A novel association of an uncommon pigmentation pattern: coexistence of cutis tricolor with intracranial teratoma and holoprosencephaly.

    PubMed

    Tekin, Burak; Yucelten, Ayse Deniz; Bayri, Yasar

    2014-10-15

    Cutis tricolor was first described in a 17-year-old male patient by Happle et al. as a rare coexistence of circumscribed hyperpigmentation and hypopigmentation close to each other on a background of normally pigmented skin. Cutis tricolor has been reported as an isolated cutaneous finding or in various associations. To the best of our knowledge, cutis tricolor in association with teratoma and holoprosencephaly has not been reported in the literature. Herein, we report a male patient who presented with a teratoma and a combination of whorl-like hypopigmentation together with hyperpigmented patches adjacent to each other on intermediately pigmented skin. This case report supports the view that cutis tricolor may be a marker of an underlying neurological abnormality.

  5. Tropism of the in situ growth from biopsies of childhood neuroectodermal tumors following transplantation into experimental teratoma.

    PubMed

    Jamil, Seema; Hultman, Isabell; Cedervall, Jessica; Ali, Rouknuddin Q; Fuchs, Gabriel; Gustavsson, Bengt; Asmundsson, Jurate; Sandstedt, Bengt; Kogner, Per; Ährlund-Richter, Lars

    2014-04-01

    Experimental teratoma induced from human pluripotent stem cells with normal karyotype can be described as a failed embryonic process and includes besides advanced organoid development also large elements of tissue with a prolonged occurrence of immature neural components. Such immature components, although benign, exhibit strong morphological resemblance with tumors of embryonic neuroectodermal origin. Here, we demonstrate that biopsy material from childhood tumors of neural embryonic origin transplanted to mature experimental teratoma can show an exclusive preference for matching tissue. Tumor specimens from five children with; Supratentorial primitive neuroectodermal tumor (sPNET); Pilocytic astrocytoma of the brainstem; Classic medulloblastoma; peripheral primitive neuroectodermal tumor (pPNET) or neuroblastoma (NB), respectively, were transplanted. Analysis of up to 120 sections of each tumor revealed an engraftment for three of the transplanted tumors: pPNET, sPNET, and NB, with a protruding growth from the latter two that were selected for detailed examination. The histology revealed a strict tropism with a non-random integration into what morphologically appeared as matched embryonic microenvironment recuperating the patient tumor histology. The findings suggest specific advantages over xenotransplantation and lead us to propose that transplantation to the human embryonic microenvironment in experimental teratoma can be a well-needed complement for preclinical in vivo studies of childhood neuroectodermal tumors.

  6. Retroperitoneal pararenal isolated neurofibroma: report of a case and review of literature

    PubMed Central

    Corbellini, C; Vingiani, A; Maffini, F; Chiappa, A; Bertani, E; Andreoni, B

    2012-01-01

    The neurofibroma is a tumour of neural origin. This kind of neoplasm, though, is generally skin located. Rare cases in deep organs or in the peritoneal cavity are also reported in the literature. There are two types of neurofibromas, localized and diffuse; the latter is associated with von Recklinghausen disease and always occurs together with skin neurofibromas. Here we report the case of a 47-year-old man affected by retroperitoneal neurofibroma, but not associated with von Recklinghausen disease. A computed tomography (CT) scan described a retroperitoneal pararenal lesion with no clear involvement of adjacent viscera. We describe the diagnostic modality, treatment planning and the timing of treatment of this neoplasm, reviewing also the literature. PMID:22654960

  7. Retroperitoneal abscess and acute acalculous cholecystitis after iatrogenic colon injury: report of a case

    PubMed Central

    Dong, Chengwei; Wang, Yuxu; Hu, Sanyuan; Du, Futian; Ding, Wei

    2015-01-01

    Acute acalculous cholecystitis has a high mortality rate due to the difficulties in early diagnosis and high rate of complications like empyema, gangrene and perforation. We report a case of 20-year-old male with acute severe pancreatitis, acute renal failure and acute peripancreatic fluid collection who was transferred to our department after blood filtration treatment in ICU. After percutaneous catheter drainage for 20 hours, the patient got a high fever. Computed tomography revealed retroperitoneal colon injury. In this case, percutaneous catheter drainage was performed again and the pus cavity was flushed regularly, after which the patient’s state gradually improved. Unpredictably, septic shock appeared on the 51st day. Repeated computed tomography revealed acute acalculous cholecystitis and abscess formation. After percutaneous transhepatic gallbladder catheterization and drainage, the patient got better gradually. Three months later the retroperitoneal catheter was removed. Four months later, ultrasound examination showed normal gallbladder and the catheter was removed. PMID:26131252

  8. Immunoglobulin G4-Related Retroperitoneal Fibrosis Treated with Hochuekkito, a Kampo Medicine, following Steroid Treatment.

    PubMed

    Fukuchi, Minoru; Sakurai, Shinji; Kogure, Toshiaki; Naitoh, Hiroshi; Kuwano, Hiroyuki

    2014-05-01

    We report a case of immunoglobulin G4 (IgG4)-related retroperitoneal fibrosis (RF) with complete remission and no relapses after therapy with steroids and Hochuekkito, a Kampo (i.e. traditional Japanese herbal) medicine. A 62-year-old Japanese man was admitted to our hospital for treatment of a retroperitoneal mass detected by computed tomography. The mass had a maximum diameter of 11.0 cm; it involved the left ureter and was associated with left hydronephrosis. After inserting a ureteral stent, we performed a biopsy by laparotomy. Histopathology revealed IgG4-related RF. The lesion disappeared after 7 months of steroid therapy. We subsequently used Hochuekkito as an alternative maintenance treatment because of steroid-related complications. The patient has not relapsed in the 3 years since starting the medication. To the best of our knowledge, this is the first case of IgG4-related RF treated with Hochuekkito as a maintenance treatment.

  9. Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature

    PubMed Central

    Menenakos, Charalambos; Braumann, Chris; Hartmann, Jens; Jacobi, Christoph A

    2007-01-01

    Background Castleman's disease is a rare lymphoproliferative syndrome. Its etiology and pathogenesis are unclear. The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin. Case presentation We present a case of a 63-year old male patient who was referred for surgical treatment of a lately diagnosed retroperitoneal pelvic mass. The patient had been already treated for two years due to progressive diffuse cutaneous lesions histologically consistent with lichen ruber verucosus and pemphigus vulgaris. Intraoperatively a highly vascularized solid mass occupying the small pelvis was resected after meticulous vascular ligation and hemostasis. After surgery and following immunosuppressive treatment a clear remission of the skin lesions was observed. Conclusion Castleman's tumor should be always suspected when a retroperitoneal mass is combined with PNP. In a review of the literature we found 37 additional cases. Complete surgical resection of the tumor can be curative in most of the cases. PMID:17466075

  10. Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases

    PubMed Central

    Ben Salha, Imen; Zaidi, Shane; Noujaim, Jonathan; Miah, Aisha B.; Fisher, Cyril; Jones, Robin L.; Thway, Khin

    2016-01-01

    Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2-amplified retroperitoneal liposarcoma. PMID:27746879

  11. Fatal Retroperitoneal Bleeding Caused by Neurofibromatosis: A Case Report and Review of the Literature

    PubMed Central

    Moerbeek, Patrick R.; van Buijtenen, Jesse M.; van den Heuvel, Baukje; Hoksbergen, Arjan W. J.

    2015-01-01

    A young female was brought into the emergency department with pulseless electrical activity (PEA) after local resection of neurofibromateous lesions. Chest ultrasonography was normal. Abdominal ultrasonography was not performed. After successful resuscitation a total body CT-scan was performed to rule out potential bleeding sources. However, haemodynamic instability reoccurred and the scan had to be aborted at the thoracoabdominal level. No thoracic abnormalities were found. Resuscitation was reinitiated and abdominal ultrasonography was performed, showing a large amount of abdominal fluid. A progressive fall in haemoglobin was noted. Emergency laparotomy was performed, revealing a large retroperitoneal haematoma. Despite ligation and packing, bleeding continued. Postoperative angiography showed active bleeding from a branch of the left internal iliac artery, which could be successfully coiled. Unfortunately, the patient died five days later due to irreversible brain damage. Revision of an MRI scan made one year earlier showed a 10 cm large retroperitoneal neurofibromatous lesion exactly at the location of the current bleeding. This case shows that patients with neurofibromatosis might develop spontaneous life-threatening bleeding from retroperitoneal located lesions. Furthermore, it points out the necessity of focused assessment with ultrasonography of the abdomen in all patients with PEA of unknown origin. PMID:25688270

  12. Intra- and retroperitoneal irrigation liquid after arthroscopy of the hip joint.

    PubMed

    Haupt, Ulrich; Völkle, Daniela; Waldherr, Christian; Beck, Martin

    2008-08-01

    The case of intra- and retroperitoneal irrigation solution after hip arthroscopy of a 15-year-old girl is presented. She underwent hip arthroscopy for intra-articular adhesiolysis after previous surgical dislocation of the hip for the treatment of femoroacetabular impingement. Arthroscopy was performed in the lateral decubitus position without traction to debride the peripheral joint compartment. The irrigation pressure was set at 40 mm Hg. There were no intraoperative complications. By the end of surgery, the anesthesiologist reported a drop in the patient's body temperature from 36.3 degrees to 34.5 degrees C. Postoperatively, she complained about abdominal swelling and discomfort. Abdominal sonography revealed approximately 2 to 3 L of intra- and retroperitoneal liquid, which was considered to be irrigation fluid. The irrigation fluid was absorbed within 16 hours without further treatment. The only possible way the irrigation fluid could have flown was a retroperitoneal course along the iliopsoas muscle and the iliac vessels with intraperitoneal perforation along their course. We observed at arthroscopies that irrigation pressure incidentally can rise to 140 mm Hg when leaking of fluid through the portals occurs. Intra-abdominal fluid is a potentially devastating complication. A sudden drop of body temperature has to raise suspicion for intra-abdominal leaking of irrigation fluid.

  13. [Two cases of retroperitoneal liposarcoma arisen from perirenal fat tissue, which could not be diagnosed preoperatively].

    PubMed

    Hamano, Atsushi; Yamashita, Yuzoh; Katoh, Yoshitake; Yumura, Yasushi; Mikata, Kunihisa; Takase, Kazunori; Ohgo, Yoshiharu; Noguchi, Sumio; Nagashima, Yohji

    2004-12-01

    We report two cases of retroperitoneal liposarcoma arisen from the perirenal fat tissue, which could not be diagnosed preoperatively. Case 1 is a 58-year-old male. He complained of left flank tumor. Computed tomography and magnetic resonance image showed a mass over 10 cm that contained fat components in the retroperitoneal space. The tumor was resected with left nephrectomy and histological examination revealed well differentiated liposarcoma. As adjuvant therapy, he received chemotherapy and 30 months has passed uneventfully. Case 2 is a 70-year-old male. Screening ultrasonography revealed incidental retroperitoneal tumor. With clinical diagnosis as non-functioning adrenal tumor, he received left nephrectomy. The pathological diagnosis was well differentiated liposarcoma, sclerosing type. No adjuvant therapy was performed. He has stopped visiting our clinic due to aggravation of heart disease. The characteristics of the images of the two cases were different despite the histological resemblance. This difference was considered to be due to the difference in the distribution of lipomatous tissue in each patient.

  14. Reprogramming in vivo produces teratomas and iPS cells with totipotency features.

    PubMed

    Abad, María; Mosteiro, Lluc; Pantoja, Cristina; Cañamero, Marta; Rayon, Teresa; Ors, Inmaculada; Graña, Osvaldo; Megías, Diego; Domínguez, Orlando; Martínez, Dolores; Manzanares, Miguel; Ortega, Sagrario; Serrano, Manuel

    2013-10-17

    Reprogramming of adult cells to generate induced pluripotent stem cells (iPS cells) has opened new therapeutic opportunities; however, little is known about the possibility of in vivo reprogramming within tissues. Here we show that transitory induction of the four factors Oct4, Sox2, Klf4 and c-Myc in mice results in teratomas emerging from multiple organs, implying that full reprogramming can occur in vivo. Analyses of the stomach, intestine, pancreas and kidney reveal groups of dedifferentiated cells that express the pluripotency marker NANOG, indicative of in situ reprogramming. By bone marrow transplantation, we demonstrate that haematopoietic cells can also be reprogrammed in vivo. Notably, reprogrammable mice present circulating iPS cells in the blood and, at the transcriptome level, these in vivo generated iPS cells are closer to embryonic stem cells (ES cells) than standard in vitro generated iPS cells. Moreover, in vivo iPS cells efficiently contribute to the trophectoderm lineage, suggesting that they achieve a more plastic or primitive state than ES cells. Finally, intraperitoneal injection of in vivo iPS cells generates embryo-like structures that express embryonic and extraembryonic markers. We conclude that reprogramming in vivo is feasible and confers totipotency features absent in standard iPS or ES cells. These discoveries could be relevant for future applications of reprogramming in regenerative medicine.

  15. [From human andro- and parthenogenesis (hydatidiform moles and benign ovarian teratomas) to cancer].

    PubMed

    Coullin, P

    2005-01-01

    Genomic imprinting is a process that appeared in mammals. This phenomenon blocks the normal development of parthenogenic and androgenic conceptuses, that is to say benign ovarian teratomas and hydatidiform moles respectively. Pathological modifications of these conceptuses depend on whether the chromosomes come from the mother or father. These pathologies are associated with an accidental anomaly during gametogenesis and/or fertilizing. These reproductive anomalies are sporadic and some familial cases may exist suggesting a genetic control of such diseases. The human andro- and parthenogenetic conceptuses, but more frequently the moles, may be invasive (choriocarcinoma). An imbalance of the imprinting genes may initiate the deregulation of other genes, including oncogenes and anti-oncogenes, which can explain the cancerous modification. Immunological and environmental factors must be also considered (presence of the only paternal chromosomes in the choriocarcinoma). Numerous works on this subject are published and some recent important discoveries underline the roles of genes HOX, Tim P3, E-cad and p-16, and the recurrent chromosome anomalies 7q21+and 8p21- in the mole to choriocarcinoma processing. Although these phenomena are complex and heterogeneous, the andro- and parthenogenote conceptuses are particularly interesting models with which to understand developmental disorders and cancerous progression.

  16. Primitive neuroectodermal tumor in an ovarian cystic teratoma: natural killer and neuroblastoma cell analysis.

    PubMed

    Tabellini, Giovanna; Benassi, Marzia; Marcenaro, Emanuela; Coltrini, Daniela; Patrizi, Ornella; Ricotta, Doris; Rampinelli, Fabio; Moretta, Alessandro; Parolini, Silvia

    2014-01-01

    In the present study, we report an extremely rare case of a 31-year-old woman with neuroblastoma arising in an ovarian cystic teratoma. We analyzed the expression of activating receptors on natural killer (NK) cells derived from the patient's peripheral blood and peritoneal fluid. In addition, we investigated the presence of specific ligands recognized by different NK cell receptors on tumor cells. We show that NK cells isolated from peritoneal fluid expressed certain triggering receptors including DNAM-1 (CD226) and CD16 with lower intensity as compared to peripheral blood NK cells. Remarkably, at variance with most cases of childhood neuroblastoma, the tumor cells from this patient expressed substantial amounts of HLA class-I molecules. These molecules are known to be protective against NK cell-mediated lysis. In addition, neuroblastoma cells expressed B7-H3 (CD276), another surface molecule that inhibits NK cell function. Finally, this tumor did not express the PVR (CD155) and nectin-2 (CD112) ligands for the DNAM-1 activating NK receptor, which plays a crucial role in NK/neuroblastoma interactions. Altogether, these findings indicate that the neuroblastoma cells of this patient express an NK-resistant surface phenotype, which is at least in part similar to that previously described in a fraction of childhood neuroblastoma.

  17. Immature teratoma of the tectum mesencephali with histopathological detection of rudimentary eye anlage in a 3-year-old boy: Report of a rare case.

    PubMed

    Dubinski, Daniel; Mittelbronn, Michel; Marquardt, Gerhard; Tews, Dominique S; Noack, Anika; Behmanesh, Bedjan; Seifert, Volker; Freiman, Thomas M

    2016-12-01

    Intracranial teratoma is a rare neoplasm derived from omnipotent germinal cells that can contain mesoderm, endoderm and/or ectoderm layer tissue. Histologically teratomas are characterized by abnormal structures like teeth or bone that can be further subdivided into mature and immature according to the presence of incompletely differentiated tissue. Characteristic intracranial teratomas are space-occupying lesions in the pineal region and often present with hydrocephalic symptoms due to aqueduct stenosis. A 3-year-old boy presented with a peracute hemiparesis, fatigue and speech deficit. MRI diagnostics showed a cystic, partially solid, inhomogeneous contrast-enhancing formation at the top of the tectum mesencephali with consecutive aqueduct compression. The patient underwent a sub-occipital craniotomy via a supracerebellar approach and complete resection was achieved. The histopathological examination mainly showed mature tissue of ectodermal, mesodermal and endodermal origin. However, small areas of undifferentiated neuroectodermal tissue within an optic vesicle formation were detected, leading to the diagnosis of an immature teratoma. In due course, the patient was discharged in good health without neurological deficits. To our knowledge, optic vesicle-containing intracranial germ cell tumors are extremely rare. Here we report a unique case with immature neuroectodermal tissue within an optic vesicle formation in an otherwise mature teratoma.

  18. BAX-mediated cell death affects early germ cell loss and incidence of testicular teratomas in Dnd1(Ter/Ter) mice.

    PubMed

    Cook, Matthew S; Coveney, Douglas; Batchvarov, Iordan; Nadeau, Joseph H; Capel, Blanche

    2009-04-15

    A homozygous nonsense mutation (Ter) in murine Dnd1 (Dnd1(Ter/Ter)) results in a significant early loss of primordial germ cells (PGCs) prior to colonization of the gonad in both sexes and all genetic backgrounds tested. The same mutation also leads to testicular teratomas only on the 129Sv/J background. Male mutants on other genetic backgrounds ultimately lose all PGCs with no incidence of teratoma formation. It is not clear how these PGCs are lost or what factors directly control the strain-specific phenotype variation. To determine the mechanism underlying early PGC loss we crossed Dnd1(Ter/Ter) embryos to a Bax-null background and found that germ cells were partially rescued. Surprisingly, on a mixed genetic background, rescued male germ cells also generated fully developed teratomas at a high rate. Double-mutant females on a mixed background did not develop teratomas, but were fertile and produced viable off-spring. However, when Dnd1(Ter/Ter) XX germ cells developed in a testicular environment they gave rise to the same neoplastic clusters as mutant XY germ cells in a testis. We conclude that BAX-mediated apoptosis plays a role in early germ cell loss and protects from testicular teratoma formation on a mixed genetic background.

  19. Ovarian teratoma displaying a wide variety of tissue components in a broiler chicken (Gallus Domesticus): morphological heterogeneity of pluripotential germ cell during tumorigenesis

    PubMed Central

    Ohfuji, S.

    2016-01-01

    Spontaneous ovarian teratoma was found in a seven-week-old female Chunky broiler chicken that was slaughtered for food. On post-mortem inspection, a spherical tumor mass attaching to a juvenile ovary was found in the abdominal cavity. Histopathologically, the tumor was comprised of immature mesenchymal stroma and a variety of mature tissue elements of mesodermal and ectodermal origin. In addition, there were multiple indistinguishable tissue elements, which showed no malignant cytological features but were unidentifiable as to corresponding embryological layer of origin. These heterogeneous teratoma tissues consisted of a variety of glandular, cystic, duct-like, and tubular structures, some of which exhibited a lining by a mixture of both keratinizing/non-keratinizing stratified squamous epithelial cells and cuboidal/columnar epithelial cells. The ovarian tetatoma was considered a benign and congenital one. The highly diverse differentiation of the teratoma might have manifested a morphological aspect of intrinsic character of the pluripotential germ cells during tumorigenesis. PMID:27303655

  20. Step-up approach and video assisted retroperitoneal debridement in infected necrotizing pancreatitis: A case complicated by retroperitoneal bleeding and colonic fistula

    PubMed Central

    Lim, Eugene; Sundaraamoorthy, R.S.; Tan, David; Teh, Hui-Seong; Tan, Tzu-Jen; Cheng, Anton

    2015-01-01

    Introduction Infected Necrotizing Pancreatitis carries a high mortality and necessitates intervention to achieve sepsis control. The surgical strategy for proven infected necrosis has evolved, with abandonment of open necrosectomy to a step-up approach consisting of percutaneous drains and Video-assisted retroperitoneal debridement (VARD). We present a case that underwent VARD complicated by bleeding and colonic perforation and describe its management. Presentation of case A 38 year-old male with acute pancreatitis developed infected necrotizing pancreatitis. Initial treatment was by percutaneous drainage under radiological guidance and intravenous antibiotics. The infected retroperitoneal necrosis was then debrided using gasless laparoscopy through a mini-incision. Post-operatively, he developed peripancreatic bleeding which was controlled with angioembolisation. He also developed a descending colon fistula which was treated with laparotomy and defunctioning loop ileostomy. He recovered and subsequently had his ileostomy closed twelve months later. The colonic fistula recurred and was treated with endoscopic clips and histoacryl glue injection and finally closed. Discussion Step-up approach consists of the 3 D's: Delay, drain and debride. VARD is recommended as it is replicable in general surgical units using standard laparoscopic instruments. Bleeding and colon perforation are potential complications which must have multi-disciplinary input, aggressive resuscitation and timely radiologic intervention. Defunctioning ileostomy is recommended to control sepsis in colonic fistulation. Novel fistula closing methods using endoscopic clips and histoacryl glue are potential treatment options. Conclusion Step-up approach and VARD is the new paradigm to treat necrotizing pancreatitis. Complications of bleeding and colon fistula are uncommon and require multi-disciplinary management. PMID:26587229

  1. Sacrococcygeal teratoma in a female newborn with clinical features of trisomy 13: a case report from Central Africa

    PubMed Central

    Lubala, Toni Kasole; Mukuku, Olivier; Shongo, Mick Pongombo; Mutombo, Augustin Mulangu; Lubala, Nina; Luboya, Oscar Numbi; Lukusa-Tshilobo, Prosper

    2015-01-01

    Introduction The objective of this report is to describe the first patient presenting clinical features of trisomy 13 in association with a sacrococcygeal teratoma. Case presentation We present the case of a Congolese female infant born with bilateral cleft lip and palate, hypotelorism, microcephaly, and capillary hemangioma on her face. She presented with a large sacrococcygeal mass (15.0 cm ×12.0 cm ×5.0 cm) with a cystic consistency and a positive transillumination. Conclusion This observation suggests that overexpression of certain genes on chromosome 13 may lead to tumor formation from remnant cells of Hensen’s node. PMID:26715863

  2. Teratoma formation assays with human embryonic stem cells: a rationale for one type of human-animal chimera.

    PubMed

    Lensch, M William; Schlaeger, Thorsten M; Zon, Leonard I; Daley, George Q

    2007-09-13

    Despite a long and valuable history, human-animal chimera research has often been questioned. Among the moral issues raised by chimeras is the concept that integration of human cells into anatomical locations such as the brain might endow animals with "human-like" capacities including self-awareness. We present a justification for one type of human-animal chimera experiment: the evaluation of hES cell developmental potency via teratoma formation in immunodeficient mice. We argue that this experiment raises no significant moral concerns and should be the jurisdiction of animal care and use committees and exempt from formal review by the stem cell research oversight process.

  3. Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: (18)F-FDG PET/CT findings.

    PubMed

    Dhull, V S; Passah, A; Rana, N; Arora, S; Mallick, S; Kumar, R

    2016-01-01

    A 30-year-old female presented with a 3-month history of erosive stomatitis and bullous lesions, along with recurrent episodes of abdominal pain. She was found to have a retroperitoneal lump in left lumbar region. Skin biopsy revealed bullous disorder. CT guided biopsy of the retroperitoneal mass was suggestive of inflammatory myofibroblastic tumor (IMT). She was started on oral steroids and supportive care, and surgery was being planned when she developed respiratory failure. CT chest revealed vertebral metastases. PET/CT for whole body work up revealed a left para-aortic mass along with multiple skeletal metastases. The patient was kept on conservative management. After 3 months, the patient has shown clinical improvement, and an exploratory laparotomy is now being planned for the excision of the tumor, followed by chemotherapy. This case of retroperitoneal IMT is rare in terms of skeletal metastases with paraneoplastic pemphigus.

  4. 18F-FDG PET/CT Brain Imaging on a Patient With Paraneoplastic Opsoclonus-Myoclonus Syndrome Arising out of a Mature Cystic Teratoma.

    PubMed

    Na, Chang Ju; Jeong, Young Jin; Lim, Seok Tae; Sohn, Myung-Hee; Jeong, Hwan-Jeong

    2016-02-01

    Opsoclonus-myoclonus syndrome (OMS) is an involuntary multidirectional eye movement accompanied by myoclonic jerks and a subtype of paraneoplastic neurological syndromes. Clinical features of OMS include opsoclonus with myoclonic jerks and cerebellar ataxia. Although there have been a few studies on brain FDG PET in paraneoplastic neurological syndrome associated with some kinds of malignancies such as lung and gastric cancer, brain FDG PET of patients with OMS caused by a mature cystic teratoma has not been reported. Here, we described a case of brain FDG PET/CT studies performed in a woman with OMS provoked from a mature cystic teratoma.

  5. Caveolin expression and activation in retroperitoneal and subcutaneous adipocytes: influence of a high-fat diet.

    PubMed

    Gómez-Ruiz, Ana; Milagro, Fermín I; Campión, Javier; Martínez, J Alfredo; de Miguel, Carlos

    2010-10-01

    The effect of a high-fat diet on the expression of the three main isoforms of caveolins in adipocytes isolated from rat retroperitoneal and subcutaneous white adipose tissue was investigated. Two distinct phases can be distinguished on a time-dependent response in adipocytes from both locations. The early stage affects only to retroperitoneal adipocytes and implies caveolin-1 activation and caveolin-2 inactivation, together with increased expression of insulin signaling intermediaries. This initial response would be aimed to counterbalance the energy overload. Continued exposure to the high-fat diet produces an increase in circulating glucose and insulin levels, inducing a late stage in which adipocytes from both locations are affected. This late stage is characterized by general increased caveolin-1 and caveolin-2 expression; while on the other hand, the insulin signaling intermediaries are downregulated, with the noticeable exception of GLUT-4, whose expression remains high. Therefore, it seems that at this stage caveolins and GLUT-4 are regulated independently of the insulin pathway, through a mechanism that could be mediated by inflammation and oxidative stress associated with obesity. Although this GLUT-4 upregulation suggests a response against the raise in circulating glucose, this might not be the case, since the developing insulin resistance at this stage indicates a prediabetic state. We have also found that the high-fat diet is able to induce the expression of muscle-specific caveolin-3 in retroperitoneal adipocytes since the initial phase. This observation is similar to what we reported previously in skeletal muscle (Gómez-Ruiz et al., 2009, FEBS Lett 583:3259-3264), suggesting a similar regulatory mechanism for this isoform.

  6. Retroperitoneal abscess with retained gall-stones as a late complication of laparoscopic cholecystectomy.

    PubMed

    Kamiński, Mateusz; Nowicki, Michał

    2016-01-01

    Laparoscopic cholecystectomy is the golden standard, considering treatment of cholelithiasis. During the laparoscopic procedure one may often observe damage to the gall-bladder wall, as well as presence of gall-stones in the peritoneal cavity, as compared to classical surgery. These gall-stones may be associated with the occurrence of various complications following surgery. The study presented a rare case of a retroperitoneal abscess, as a consequence of retained gall-stones, in a female patient who was subject to laparoscopic cholecystectomy two years earlier.

  7. Spontaneous retroperitoneal and subcapsular liver hematoma. The diagnostic contribution of CT, US and CEUS. Case report.

    PubMed

    Badea, Radu; Chiorean, Liliana; Mitre, Calin; Botar-Jid, Carolina; Caraiani, Cosmin

    2013-06-01

    We present the case of a patient undergoing long-time treatment with oral anticoagulants for a severe vascular pathology who had a major subcapsular liver bleeding as well as retroperitoneal bleeding. The purpose of the paper is to present the specific characteristics of this case, to review the literature that concentrates on spontaneous bleedings and to show the role of contrast-enhanced ultrasonography as a useful, non invasive imaging method in excluding active hemorrhage and its value in the non-surgical management of the case.

  8. Retroperitoneal migration of a self-inflicted ballpoint pen via the urethra.

    PubMed

    Cury, Jose; Coelho, Rafael F; Srougi, Miguel

    2006-01-01

    Numerous accounts documenting the introduction of foreign bodies into the urinary bladder have been reported. These foreign bodies are typically self-inserted via urethral but migration from adjacent organs by an ulcerative process and penetrating injuries are also reported. However, ''contrary'' migration of a self-inflicted vesical foreign body to the retroperitoneum was not previously reported in literature. We report here a case of a ballpoint pen self-inserted via urethral by a female patient, which was identified in retroperitoneal position years later.

  9. Nitrogenous subcutaneous emphysema caused by spray application of fibrin glue during retroperitoneal laparoscopic surgery.

    PubMed

    Matsuse, Shinji; Maruyama, Atsushi; Hara, Yoshiki

    2011-06-01

    We report a case of a patient treated by retroperitoneoscopic partial nephrectomy who developed nitrogenous subcutaneous emphysema (SCE) as a complication. The use of a nitrogen gas-pressured fibrin tissue adhesive applied as a spray caused excessively increased pressure in the closed retroperitoneal space and resulted in widespread SCE with protracted clinical course. To the best of our knowledge, this is the first report of nitrogenous SCE associated with pneumoperitoneum. The clinical significance of nitrogenous SCE is emphasized, and the risks associated with the use of fibrin glue as a spray during laparoscopic surgery are discussed.

  10. Embryonic pig liver, pancreas, and lung as a source for transplantation: optimal organogenesis without teratoma depends on distinct time windows.

    PubMed

    Eventov-Friedman, Smadar; Katchman, Helena; Shezen, Elias; Aronovich, Anna; Tchorsh, Dalit; Dekel, Benjamin; Freud, Enrique; Reisner, Yair

    2005-02-22

    Pig embryonic tissues represent an attractive option for organ transplantation. However, the achievement of optimal organogenesis after transplantation, namely, maximal organ growth and function without teratoma development, represents a major challenge. In this study, we determined distinct gestational time windows for the growth of pig embryonic liver, pancreas, and lung precursors. Transplantation of embryonic-tissue precursors at various gestational ages [from E (embryonic day) 21 to E100] revealed a unique pattern of growth and differentiation for each embryonic organ. Maximal liver growth and function were achieved at the earliest teratoma-free gestational age (E28), whereas the growth and functional potential of the pancreas gradually increased toward E42 and E56 followed by a marked decline in insulin-secreting capacity at E80 and E100. Development of mature lung tissue containing essential respiratory system elements was observed at a relatively late gestational age (E56). These findings, showing distinct, optimal gestational time windows for transplantation of embryonic pig liver, pancreas, and lung, might explain, in part, the disappointing results in previous transplantation trials and could help enhance the chances for successful implementation of embryonic pig tissue in the treatment of a wide spectrum of human diseases.

  11. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease.

    PubMed

    Rossi, Giovanni Maria; Rocco, Rossana; Accorsi Buttini, Eugenia; Marvisi, Chiara; Vaglio, Augusto

    2017-04-01

    Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.

  12. Spectrum of retroperitoneal and genitourinary paraganglioma: Experience at a North Indian tertiary care center

    PubMed Central

    Choudhary, Gautam Ram; Singh, Shivanshu; Prasad, Seema; Singh, Shrawan Kumar; Bhansali, Anil; Bhadada, Sanjay; Dutta, Pinaki

    2015-01-01

    Introduction Genitourinary and retroperitoneal paragangliomas are infrequent tumors with bizarre presentation. A high index of suspicion is required to make a diagnosis in young hypertensive individuals. Our aim is to study the varied clinical presentations and management of these paragangliomas. Herein, we share our experience of clinical presentation, diagnosis, and management of these paragangliomas. Material and methods Seventeen consecutive patients who underwent surgery for paraganglioma at our institute from August 2009 to July 2014 were included. Demographic, peri-operative, surgical, and follow up data were collected and analyzed. Results Mean age of presentation was 34.8 years with female predominance. The majority of the tumors were located in the retroperitoneum and urinary bladder. Most of them presented with classical symptoms of catecholamine excess and hypertension. Complete surgical resection could be performed in 13 cases. At a median follow up of two years, cases with R0 resection (no microscopic malignant cells) did not show recurrence. Among patients on chemotherapy, one died, another had partial response, and yet another had progressive disease. Conclusions Genitourinary and retroperitoneal paragangliomas are a disease of a young age group with variable clinical features at presentation. Appropriate pre-operative optimization and complete surgical resection provide the best chance of cure. PMID:26855794

  13. Does concomitant aortic bypass and renal artery revascularization using the retroperitoneal approach increase perioperative risk?

    PubMed

    Darling, R C; Shah, D M; Chang, B B; Leather, R P

    1995-08-01

    While elective repair of abdominal aortic aneurysms and aortoiliac occlusive disease is associated with an acceptable (3%) mortality rate, combined aortic and renal revascularization has usually been reported to have a higher perioperative mortality. Over the past 5 years, 785 elective aortic procedures have been performed at the authors' medical center. During the same period, 77 renal artery reconstructions have been performed in 73 patients in conjunction with aortic procedures. All were done using the retroperitoneal approach to the aorta and renal arteries. Indication for concomitant renal artery revascularization included 79% (61 of 77 patients) for either significant stenosis or anatomic involvement, 18% for renovascular hypertension (14 of 73) and 3% (two of 73) for renal impairment. The demographics and risk factors were similar in both groups. Operative mortality rate was 2.9% (23 of 785) in the aortic group and 3% (two of 73) in the combined group. Complications in the combined group were one stroke (1.4%), one re-exploration for bleeding (1.4%), two pulmonary pneumonia (2.7%) and five patients had elevated serum creatinine (> 350 mumol/l) after operation. Of these patients two died, one had an occluded graft and two eventually improved. There was one early graft thrombosis and one late thrombosis. In the authors' experience, concomitant aortic bypass and renal artery revascularization can be performed with an acceptable mortality and morbidity using the retroperitoneal approach.

  14. The two-port laparoscopic retroperitoneal approach for minimal access pancreatic necrosectomy

    PubMed Central

    Cresswell, AB; Nageswaran, H; Belgaumkar, A; Kumar, R; Menezes, N; Riga, A; Worthington, TR

    2015-01-01

    Introduction Despite advances in surgery and critical care, severe pancreatitis continues to be associated with a high rate of mortality, which is increased significantly in the presence of infected pancreatic necrosis. Controversy persists around the optimal treatment for such cases, with specialist units variously advocating open necrosectomy, simple percutaneous drainage or one of several minimal access approaches. We describe our technique and outcomes with a two-port laparoscopic retroperitoneal necrosectomy (2P-LRN). Methods Thirteen consecutive patients with proven infected pancreatic necrosis were treated by 2P-LRN over a three-year period in the setting of a specialist hepatopancreatobiliary unit. The median patient age was 46 years (range: 28–87 years) and 10 of the patients were male. Results The median number of procedures required to clear the necrosis was 2 (range: 1–5), with a median time to discharge following the procedure of 44 days (range: 10–135 days). There was no 90-day mortality and the morbidity rate was 38%, consisting of pancreatic fistula (31%) and bleeding (23%). Conclusions Two-port laparoscopic retroperitoneal necrosectomy has been demonstrated to confer similar or better outcomes to other techniques for necrosectomy. It carries the additional advantages of better visualisation, leading to fewer procedures and the opportunity to deploy simple laparoscopic instruments such as diathermy or haemostatic clips. PMID:26264086

  15. Lateral retroperitoneal transpsoas interbody fusion in a patient with achondroplastic dwarfism.

    PubMed

    Staub, Blake N; Holman, Paul J

    2015-02-01

    The authors present the first reported use of the lateral retroperitoneal transpsoas approach for interbody arthrodesis in a patient with achondroplastic dwarfism. The inherent anatomical abnormalities of the spine present in achondroplastic dwarfism predispose these patients to an increased incidence of spinal deformity as well as neurogenic claudication and potential radicular symptoms. The risks associated with prolonged general anesthesia and intolerance of significant blood loss in these patients makes them ideal candidates for minimally invasive spinal surgery. The patient in this case was a 51-year-old man with achondroplastic dwarfism who had a history of progressive claudication and radicular pain despite previous extensive lumbar laminectomies. The lateral retroperitoneal transpsoas approach was used for placement of interbody cages at L1/2, L2/3, L3/4, and L4/5, followed by posterior decompression and pedicle screw instrumentation. The patient tolerated the procedure well with no complications. Postoperatively his claudicatory and radicular symptoms resolved and a CT scan revealed solid arthrodesis with no periimplant lucencies.

  16. Immunoglobulin G4-Related Retroperitoneal Fibrosis Treated with Hochuekkito, a Kampo Medicine, following Steroid Treatment

    PubMed Central

    Fukuchi, Minoru; Sakurai, Shinji; Kogure, Toshiaki; Naitoh, Hiroshi; Kuwano, Hiroyuki

    2014-01-01

    We report a case of immunoglobulin G4 (IgG4)-related retroperitoneal fibrosis (RF) with complete remission and no relapses after therapy with steroids and Hochuekkito, a Kampo (i.e. traditional Japanese herbal) medicine. A 62-year-old Japanese man was admitted to our hospital for treatment of a retroperitoneal mass detected by computed tomography. The mass had a maximum diameter of 11.0 cm; it involved the left ureter and was associated with left hydronephrosis. After inserting a ureteral stent, we performed a biopsy by laparotomy. Histopathology revealed IgG4-related RF. The lesion disappeared after 7 months of steroid therapy. We subsequently used Hochuekkito as an alternative maintenance treatment because of steroid-related complications. The patient has not relapsed in the 3 years since starting the medication. To the best of our knowledge, this is the first case of IgG4-related RF treated with Hochuekkito as a maintenance treatment. PMID:24987323

  17. Laparoscopic splenopexy of a pediatric wandering spleen by creation of a retroperitoneal pocket.

    PubMed

    Hedeshian, Mohir H; Hirsh, Michael P; Danielson, Paul D

    2005-12-01

    Wandering spleen is a rare condition in which the spleen is attached by a long, vascular pedicle and lacks its usual peritoneal attachments and supporting ligaments. This condition predisposes the spleen to torsion and infarction. We report the case of a 2-year-old boy with a history of intermittent abdominal pain and early satiety who presented with abdominal pain and severe gastric distention. A work-up including computed tomographic and ultrasonographic imaging suggested a wandering spleen. The diagnosis was confirmed during laparoscopy, and splenopexy was performed by anchoring the spleen in a retroperitoneal pocket in the left upper quadrant at the level of the tenth rib. To our knowledge, this is the first reported case of minimally invasive splenopexy for wandering spleen that involves the creation of a retroperitoneal pocket without the use of mesh. The authors believe that this is a safe and effective method that takes advantage of laparoscopy and avoids the risk of infection and complications associated with the use of synthetic material.

  18. A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome.

    PubMed

    Fukui, Toshiro; Okazaki, Kazuichi; Yoshizawa, Hazuki; Ohashi, Shinya; Tamaki, Hiroyuki; Kawasaki, Kimio; Matsuura, Minoru; Asada, Masanori; Nakase, Hiroshi; Nakashima, Yasuaki; Nishio, Akiyoshi; Chiba, Tsutomu

    2005-01-01

    We report a very rare case of autoimmune pancreatitis (AIP) associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome. The patient had an enlarged pancreas, and autoantibodies were detected in the serum. Serum IgG and IgG4 concentrations were also elevated. Endoscopic retrograde cholangiopancreatography revealed an irregular narrowing of the main pancreatic duct from the head to the body and sclerotic change in the intrapancreatic common bile duct, which later extended to the intrahepatic bile ducts. In addition, histological examination of the liver revealed lymphocytic sclerosis around the bile ducts, similar to the histology in the pancreas of AIP. Retroperitoneal tumors were diagnosed as retroperitoneal fibrosis by histological examination. Serological and functional abnormalities suggestive of Sjögren's syndrome were detected, and histological findings of the lip were compatible with Sjögren's syndrome. Immunohistochemistry of each lesion disclosed that most of the infiltrating lymphocytes were T cells with similar levels of both CD4+ and CD8+ cells. Moreover, some of the infiltrating plasma cells were positive for anti-IgG4 monoclonal antibody. These diseases were dramatically improved by steroid therapy. Although the pathophysiology of AIP is still unclear, the present case suggests a common pathophysiological mechanism for AIP, sclerosing cholangitis, retroperitoneal fibrosis and Sjögren's syndrome.

  19. Iliocaval Stenosis and Iliac Venous Thrombosis in Retroperitoneal Fibrosis: Percutaneous Treatment by Use of Hydrodynamic Thrombectomy and Stenting

    SciTech Connect

    Vorwerk, Dierk; Guenther, Rolf W.; Wendt, Georg; Neuerburg, Joerg; Schuermann, Karl

    1996-11-15

    A case of bilateral iliac stenosis and caval stenosis due to retroperitoneal fibrosis was treated by caval stenting and iliac balloon angioplasty, but was complicated by subsequent iliac thrombosis. Venous thrombectomy was successfully achieved by hydrodynamic thrombectomy, and iliac patency was stabilized by bilateral stent insertion.

  20. Association of idiopathic retroperitoneal fibrosis, rapidly progressive glomerulonephritis and antiproteinase 3 antineutrophil cytoplasmic antibodies (anti PR3-ANCA).

    PubMed

    Martínez-Odriozola, P; Gutiérrez-Macías, A; Moina Eguren, I; Arrieta Lezama, J

    2008-09-01

    We report a case of idiopathic retroperitoneal fibrosis and rapidly progressive glomerulonephritis with serum antiproteinase 3 antineutrophil cytoplasmic antibodies (anti-PR3-ANCA), without clinical or histological signs of Wegener's granulomatosis, in a 46-year-old man. Our case and previously reported cases showing the same association support the hypothesis that the association is not fortuitous, but reflects a common immunological mechanism.

  1. Diagnostic imaging findings of pelvic retroperitoneal ganglioneuroma in a child: a case report with the emphasis on initial ultrasound findings.

    PubMed

    Mut, Deniz Turkyılmaz; Orhan Soylemez, Umut Percem; Demir, Mesut; Tanık, Canan; Ozer, Alper

    2016-03-01

    Ganglioneuromas are rare benign tumors of neural crest origin developed along the sympathetic chain. The pelvic retroperitoneum is the rarest location of these tumors. Clinically these tumors are commonly asypmtomatic even if they reach large sizes. Here we report the radiological features of a 16 year old boy with pathologically proven retroperitoneal ganglioneuroma that was detected initially by ultrasound. Relevant literature is also discussed.

  2. Thoracic Stent Graft Implantation for Aortic Coarctation with Patent Ductus Arteriosus via Retroperitoneal Iliac Approach in the Presence of Small Sized Femoral Artery

    PubMed Central

    Korkmaz, Ozge; Beton, Osman; Goksel, Sabahattin; Kaya, Hakkı; Berkan, Ocal

    2016-01-01

    Endovascular stent graft implantation is a favorable method for complex aortic coarctation accompanied by patent ductus arteriosus. Herein, an 18-year-old woman with complex aortic coarctation and patent ductus arteriosus was successfully treated by endovascular thoracic stent graft via retroperitoneal approach. The reason for retroperitoneal iliac approach was small sized common femoral arteries which were not suitable for stent graft passage. This case is the first aortic coarctation plus patent ductus arteriosus case described in the literature which is treated by endovascular thoracic stent graft via retroperitoneal approach. PMID:27242935

  3. [Primary renal angiosarcoma].

    PubMed

    Costero-Barrios, Cesáreo B; Oros-Ovalle, Cuauhtémoc

    2004-01-01

    The twenty-fourth case of primary renal angiosarcoma is described, according to the available international literature, this present in a 71-year-old male, a mechanic by trade, without carcinogenic antecedents. Hematuria, pain in flank, and left-side tumoral mass of approximately 20 cm in diameter located in kidney by computerized axial tomography (CT) constituted manifestations. A left nefrectomy was performed. No metastasis was found. The tumor replaced 4/5 of the organ and weighed 1145 g. It showed angiomatous structure with atypical proliferation of endothelial cells in a sinusoldal trauma and anastomosatic vascular channels that invaded neighboring parenchymal and capsule. Tymorous cells were positive for CD31 and CD34 and negative for cytokeratins, S100 and HMB 45 proteins. The patient was subjected to treatment with chemotherapy and radiotherapy (lineal accelerator), but 12 months after surgery he presented retroperitonal tumoral relapse and hepatic metastasis. Diagnostic differentiation with benign vascular tumors is pointed out, as well as carcinomas and sarcomas that showed an outstanding angiomatous component, both primary and/or secondary. Primary renal angiosarcoma exposes the multiplicity of localizations that it is capable of with a tumor of this type, as well as renal parenquimatous capacity to be the seat of a great variety of neoplasias.

  4. Retroperitoneal fibrosis

    MedlinePlus

    ... upper urinary tract obstruction. In: Wein AJ, ed. Campbell-Walsh Urology . 10th ed. Philadelphia, PA: Elsevier Saunders; ... of urinary tract obstruction. In: Wein AJ, ed. Campbell-Walsh Urology . 10th ed. Philadelphia, PA: Elsevier Saunders; ...

  5. [Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma: Description of a case and anesthetic implications].

    PubMed

    Arteche Andrés, M A; Zugasti Echarte, O; de Carlos Errea, J; Pérez Rodríguez, M; Leyún Pérez de Zabalza, R; Azcona Calahorra, M A

    2015-10-01

    N-methyl-D-aspartate receptor encephalitis is an autoimmune encephalitis relationated or not with a neoplasm. Although its incidence is unknown, probably remains underdiagnosed. Epidemiological studies place it as the second cause of immune-mediated encephalitis and the first in patients aged less of 30 years. It shows neuropsychiatric symptoms and autonomic instability. After diagnosis, based on the detection of antibodies in serum or cerebrospinal fluid, an occult malignancy must be investigated. While increasing number of cases have been diagnosed and the important role of this receptor in general anesthesia mechanisms, the interaction of the disease with anesthetic agents and perioperative stress is unknown. We describe the case of a patient with encephalitis associated to ovarian teratoma that underwent gynaecological laparoscopy.

  6. A huge malignant peripheral nerve sheath tumor with hepatic metastasis arising from retroperitoneal ganglioneuroma.

    PubMed

    Meng, Z H; Yang, Y S; Cheng, K L; Chen, G Q; Wang, L P; Li, W

    2013-01-01

    Ganglioneuromas (GNs) are the rarest and most benign of the neuroblastic tumors. We experienced a case of huge retroperitoneal GN which differentiated into malignant peripheral nerve sheath tumors (MPNST) with hepatic metastasis. The tumor was located in the upper right quarter of the abdomen and pressed the right lobe of the liver, which was initially misdiagnosed as a liver carcinoma. The tumor shared blood supply with the right liver lob and had rich blood supplies from the abdominal aorta, renal artery and hepatic artery. It was also associated with skin pigment and recurrence shortly following resection. Our finding demonstrated that MPNST is a potent invasive malignant tumor and metastasis earlier with very poor prognosis.

  7. Pneumomediastinum and retroperitoneal air after removal of papillomas with the microdebrider and jet ventilation.

    PubMed Central

    Sims, H. Steven; Lertsburapa, Keith

    2007-01-01

    OBJECTIVE: To discuss the complication of pneumothorax from alveolar rupture after transtracheal high-frequency jet ventilation and to present a case of pneumothorax, pneumomediastinum and pneumoperitoneum after jet ventilation coupled with use of the microdebrider. METHOD: Detailed case report. RESULTS: Unilateral pnuemothorax, subcutaneous emphysema, pneumomediastinum and retroperitoneal air discovered after jet ventilation for removal of airway papillomas resolved with conservative management. DISCUSSION: We discuss the difference between the respective patterns of air seepage in a peripheral alveolar injury versus a probable microperforation in the trachea. We also review the epidemiology of this rare disorder and its incidence in the African-American community. CONCLUSION: The recurrent nature of this disorder mandates multiple surgical procedures. Great care must be taken to eradicate disease and avoid complications. Pneumomediastinum in this setting can be managed conservatively. Images Figure 1 Figure 2 PMID:17913120

  8. Retroperitoneal Approach for Dissection of Inflamed Pelvic Viscera in Acute Pelvic Inflammatory Disease- Case Report

    PubMed Central

    Ganesh, Deepa; Srinivasan, Jayashree; S, Jayakumar; Mathew, Renu

    2014-01-01

    Tubo-ovarian abscesses can rupture spontaneously after a manual examination or an accidental trauma. A critically ill patient with septic peritonitis will only deteriorate if timely surgical removal of pus is not done. The operation of choice is removal of free pus, together with the abscess, the uterus, the tubes and usually, the ovaries. Anatomy is distorted, dependable landmarks are obscured and tissues are thick and oedematous. Loops of densely adhered intestine are difficult to separate. If an intra peritoneal approach is used, it is likely that the fragments of ovary will be left behind. This can subsequently cause signs and symptoms of ovarian remnant syndrome. Injury to the serosa of distended bowel occurs inadvertently, thus increasing the morbidity which results from the procedure. We are hereby presenting a unique case of a ruptured tubo-ovarian abscess where a retroperitoneal approach was used. PMID:24995219

  9. Treatment of a retroperitoneal abscess by omentalization in an orangutan (Pongo pygmaeus pygmaeus).

    PubMed

    Chai, Norin; Hazan, Thierry; Wedlarski, Rudy; Rigoulet, Jacques

    2009-06-01

    A 38-yr-old orangutan (Pongo pygmaeus pygmaeus) presented with chronic lethargy and difficulty in locomotion that progressed to weakness, anorexia, and permanent dorsal and/or lateral recumbency. The orangutan was immobilized with ketamine. Abdominal ultrasonography revealed a mass in the caudal portion of the abdomen. Exploratory surgery was performed, but the mass could not be resected. Instead, the mass was drained and omentalized in an attempt to establish continuous drainage after surgery. The only complication was a wound infection that was treated locally with a disinfectant and installation of a drain that was changed every 2 days under anesthesia. Omentalization was successful in providing continuous fluid drainage for this retroperitoneal abscess and required minimal postoperative handling of the animal.

  10. Retroperitoneal mucinous cystadenoma of the appendix mimicking hydatid cyst: A case report

    PubMed Central

    Sikar, Hasan Ediz; Çetin, Kenan; Gündoğan, Ersin; Gündoğan, Gökçen Alinak; Kaptanoğlu, Levent

    2016-01-01

    Appendiceal mucocele is a cystic dilatation of the appendix due to abnormal appendiceal mucinous secretion. Cystadenoma of the appendix is one of the most common causes and is encountered in 0.6% of all appendectomy specimens. The diagnosis may be difficult due to the asymptomatic nature of the disease; pain in the right lower quadrant may be the only symptom. Complex ovarian cyst, urolithiasis or cystic hydatid disease of the liver have been reported as mimicking appendiceal mucocele in the literature. In this study, we present a case of mucinous cystadenoma of the appendix mimicking retroperitoneal hydatid cyst in a 59-year-old woman. The patient was treated with laparoscopic appendectomy with partial resection of the caecum following laparoscopic exploration. PMID:27446577

  11. Intraneural pseudocyst (so-called ganglion) in an unusual retroperitoneal periadnexal location?

    PubMed Central

    2014-01-01

    A case of an unusual unilocular cystic lesion of diameter 7 cm located retroperitoneally in the pelvis in close connection to the right adnexa of a 61 year-old woman is presented. Macroscopically, the lesion had a smooth outer and inner surface and was filled with translucent fluid. Histological examination revealed a fibrous and hyalinized wall which lacked a specific lining. Numerous nerve bundles in the cyst wall constituted the most conspicuous element of its histology possibly with some contribution of perineurial and/or mesothelial components. The morphology and immunohistochemistry speak for an intraneural pseudocyst sometimes called intraneural ganglion cyst which is rare in this location. Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1357862917132314 PMID:25044274

  12. Clinical significance of systematic retroperitoneal lymphadenectomy during interval debulking surgery in advanced ovarian cancer patients

    PubMed Central

    Takada, Toshio; Iitsuka, Chiaki; Nomura, Hidetaka; Abe, Akiko; Taniguchi, Tomoko; Takizawa, Ken

    2015-01-01

    Objective To investigate the clinical significance of systematic retroperitoneal lymphadenectomy during interval debulking surgery (IDS) in advanced epithelial ovarian cancer (EOC) patients. Methods We retrospectively reviewed the medical records of 124 advanced EOC patients and analyzed the details of neoadjuvant chemotherapy (NACT), IDS, postoperative treatment, and prognoses. Results Following IDS, 98 patients had no gross residual disease (NGRD), 15 had residual disease sized <1 cm (optimal), and 11 had residual disease sized ≥1 cm (suboptimal). Two-year overall survival (OS) and progression-free survival (PFS) rates were 88.8% and 39.8% in the NGRD group, 40.0% and 13.3% in the optimal group (p<0.001 vs. NGRD for both), and 36.3% and 0% in the suboptimal group, respectively. Five-year OS and 2-year PFS rates were 62% and 56.1% in the lymph node-negative (LN-) group and 26.2% and 24.5% in the lymph node-positive (LN+) group (p=0.0033 and p=0.0024 vs. LN-, respectively). Furthermore, survival in the LN+ group, despite surgical removal of positive nodes, was the same as that in the unknown LN status group, in which lymphadenectomy was not performed (p=0.616 and p=0.895, respectively). Multivariate analysis identified gross residual tumor during IDS (hazard ratio, 3.68; 95% confidence interval, 1.31 to 10.33 vs. NGRD) as the only independent predictor of poor OS. Conclusion NGRD after IDS improved prognosis in advanced EOC patients treated with NACT-IDS. However, while systematic retroperitoneal lymphadenectomy during IDS may predict outcome, it does not confer therapeutic benefits. PMID:26197771

  13. CT-Guided Radioactive {sup 125}I Seed Implantation Therapy of Symptomatic Retroperitoneal Lymph Node Metastases

    SciTech Connect

    Wang, Zhongmin; Lu, Jian; Gong, Ju; Zhang, Liyun; Xu, Yingjia; Song, Shaoli; Chen, Kemin; Liu, Fenju; Gang, Huang

    2013-04-12

    PurposeThis study explored the clinical efficacy of CT-guided radioactive {sup 125}I seed implantation in treating patients with symptomatic retroperitoneal lymph node metastases.MethodsTwenty-five patients with pathologically confirmed malignant tumors received CT-guided radioactive {sup 125}I seed implantation to treat metastatic lymph nodes. The diameter of the metastatic lymph nodes ranged from 1.5 to 4.5 cm. Treatment planning system (TPS) was used to reconstruct the three-dimensional image of the tumor and then calculate the corresponding quantity and distribution of {sup 125}I seeds.ResultsFollow-up period for this group of patients was 2–30 months, and median time was 16 months. Symptoms of refractory pain were significantly resolved postimplantation (P < 0.05), and Karnofsky score rose dramatically (P < 0.05). Most patients reported pain relief 2–5 days after treatment. Follow-up imaging studies were performed 2 months later, which revealed CR in 7 patients, PR in 13 patients, SD in 3 patients, and PD in 2 patients. The overall effective rate (CR + PR) was 80 %. Median survival time was 25.5 months. Seven patients died of recurrent tumor; 16 patients died of multiorgan failure or other metastases. Two patients survived after 30 months follow-up. Two patients reported localized skin erythema 1 week postimplantation, which disappeared after topical treatment.ConclusionsCT-guided radioactive {sup 125}I seed implantation, which showed good palliative pain relief with acceptable short-term effects, has proved in our study to be a new, safe, effective, and relatively uncomplicated treatment option for symptomatic retroperitoneal metastatic lymph nodes.

  14. [DESCRIPTION OF A RETROPERITONEAL ACCESS ROUTE TO THE VESSELS OF THE SPLEEN FOR SPLENORENAL ARTERIAL AND VENOUS ANASTOMOSIS].

    PubMed

    Gil-Vernet Vila, José María

    2014-01-01

    To perform arterial or venous spleno-renal anastomoses, surgeons have so far systematically used the transperitoneal way whic is burdened by a high mortality an morbility percentage. On the basis of anatomo-surgical considerations, a retroperitoneal approach has been found reaching the hilus of the spleen via the lumbar region; the first arterial spleno-renal anastomosis by this way was performed in 1972 and the first venous spleno-renal anastomosis due to portal hipertension also by this way was performed in 1974, the alter proving to be the least aggresive by avoiding damaging the páncreas, the most surgical and direct for reaching the splenic vessels thereby enabling a better exposure and an easier performing of the anastomoses. By being retroperitoneal, the loss or infección of the ascitic liquid in the cirrhotic patient is prevented.

  15. Antenatal Diagnosis of a Large Immature Abdominal Wall Teratoma by 2D-3D Ultrasound Using HDlive and Magnetic Resonance Imaging.

    PubMed

    Werner, Heron; Mocarzel, Carolina; Sá, Renato Augusto; Tonni, Gabriele; Novoa Y Novoa, Victoria Arruga; Avvad-Portari, Elyzabeth; Bonasoni, Paola; Araujo Júnior, Edward

    2016-01-01

    We describe the first case of prenatally detected teratoma of the fetal abdomen wall using ultrasound and fetal magnetic resonance imaging (MRI). A heterogeneous mass, partly solid and cystic, originating from the anterior abdominal wall of the fetus close to an omphalocele sac was detected by means of 2D/3D ultrasound and MRI. Amniodrainage was performed and due to sign of impending fetal risk, an emergency Cesarean section was performed. A bulky, crumbly and bleeding tumoral mass was confirmed at delivery. Ligation of the supplying artery to the tumor was complicated by uncontrollable hemorrhage and early neonatal death. Pathology identified the tumor as an immature teratoma of the anterior fetal abdominal wall. 2D/3D ultrasound, especially using HDlive application and MRI demonstrated accurate detection and characterization of this congenital tumor.

  16. An unusual case of a mature teratoma on the left perineal region of a young cat: surgical treatment and pathological description

    PubMed Central

    2013-01-01

    A 10-month-old intact male cat with a clinical history of a large mass in the left perineal region was submitted to the surgery department. The mass had reportedly been present as a small swelling after birth. Cytological evaluation using a fine-needle aspirate showed eosinophilic keratinaceous debris, and was not convincing for the definitive diagnosis. Complete surgical excision was performed. Postoperative function and aesthetics were excellent. Based on gross and histological features the definitive diagnosis of the tumor was mature teratoma with ectodermal and endodermal components. After a follow-up period of 4 months, no signs of recurrence were evident. Surgical excision of the teratoma in our case was considered curative. A perineal location has not been previously reported in the cat and should be considered a rare condition in this species. PMID:23844766

  17. Luteinizing hormone, sex steroids and extracorporeal circulation - a promising link to treat retroperitoneal sarcomas. A reconsideration of cancer treatment.

    PubMed

    Lazar, Angela Madalina

    2012-10-01

    Retroperitoneal sarcomas are rare and aggressive tumors with a negative prognosis as there is currently no satisfactory treatment for them. The only proven factor that can significantly increase the otherwise poor survival of sarcoma patients is the radically of resection. However, the completeness of resection is hindered by the hypervascularized nature of sarcomas and the frequent involvement of major blood vessels. In this context, we propose to operate on retroperitoneal sarcomas only with the use of extracorporeal circulation, applying vascular clamps above and below the tumor, even with short periods of hypothermic circulatory arrest in complex cases. This technique would allow the surgeon to achieve complete tumor resections, approach large blood vessels easier and perform sofisticated vascular reconstructions with no fear of hemorrhage which is fundamental to achieve a bloodless surgical field. Also, we speculate on the etiology of retroperitoneal sarcomas that appear mostly during the period of menopause/andropause. Although both estrogens and androgens have been incriminated in inducing various cancer types, including sarcomas, an endogenous estradiol cathabolyte has been shown to have anti-tumor effects. Considering that during menopause/andropause sex steroid levels actually decrease, our second working hypothesis is that the increasing follicle-stimulating hormone (FSH) and especially luteinizing hormone (LH) levels, together with the relative estrogen/androgen imbalance, may be the triggering cause. Also, a certain level of estrogens (Methoxyestradiol) may be essential in limiting tumor development and dedifferentiation. Given that extragonadal sarcomas appear to behave as endocrine tumors, a targeted hormonal therapy, together with controlled radical resections in complex cases of tumor vascular involvement, would certainly provide a strong link to both prevention and treatment of retroperitoneal sarcomas and even of cancer in general.

  18. Retroperitoneal Endometriosis: A Possible Cause of False Positive Finding at 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography

    PubMed Central

    Maffione, Anna Margherita; Panzavolta, Riccardo; Lisato, Laura Camilla; Ballotta, Maria; D'Isanto, Mariangela Zanforlini; Rubello, Domenico

    2015-01-01

    Endometriosis is a frequent and clinically relevant problem in young women. Laparoscopy is still the gold standard for the diagnosis of endometriosis, but frequently both morphologic and functional imaging techniques are involved in the diagnostic course before achieving a conclusive diagnosis. We present a case of a patient affected by infiltrating retroperitoneal endometriosis falsely interpreted as a malignant mass by contrast-enhanced magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography/computed tomography. PMID:26097425

  19. Utilization of a submental island flap and 3D printed model for skull base reconstruction: Infantile giant cranio-cervicofacial teratoma.

    PubMed

    Wiedermann, Joshua P; Joshi, Arjun S; Jamshidi, Aria; Conchenour, Carolyn; Preciado, Diego

    2017-01-01

    Transcranial masses in the infant population can leave considerable skull base defects following resection. Traditional reconstructive techniques can result in significant morbidity or poor cosmetic results. We present a case of a large skull base defect in a six week-old infant, after removal of a giant cranio-cervicofacial teratoma, which was reconstructed using a versatile submental island flap. Additionally, preoperative planning and intraoperative navigation was advanced with the use of a 3D constructed model of the patient's mass.

  20. Simultaneous Retroperitoneal Robotic Partial Nephrectomy and Hepatectomy for Synchronous Renal-Cell Carcinoma and Hepatocellular Carcinoma in a Cirrhotic Patient

    PubMed Central

    Ong, Khaa Hoo; Huang, Steven Kuan-Hua; Yen, Chia-Sheng; Tian, Yu-Feng

    2016-01-01

    Abstract Background: The development of laparoscopic and robotic surgeries represents the modern era with the objective of improving patient outcomes; this surgical method is widespread in urology and general surgery. Retroperitoneal laparoscopic/robotic surgery is common in urologic surgery, but not in liver surgery. Tumors located in the posterosuperior aspect of the liver are difficult to access using a transperitoneal approach, and control of bleeding can also be difficult, especially in patients with cirrhosis. Case Presentation: Herein, we present a 66-year-old man who had a cirrhotic liver with concurrent renal and hepatic tumors. The renal tumor was located at the upper pole of the right kidney and the liver tumor was located at the liver dome (segment VII); the patient underwent simultaneous robotic hepatectomy and partial nephrectomy with a retroperitoneal approach. Conclusion: To our knowledge, this is the first case involving a retroperitoneal approach for a simultaneous robotic hepatectomy and partial nephrectomy; this method was feasible and safe. We hope this approach serves as an alternative surgical method for patients with synchronous renal and posterior segment liver tumors. PMID:27868101

  1. Spontaneous healing of retroperitoneal chylous leakage following anterior lumbar spinal surgery: a case report and literature review

    PubMed Central

    Su, I-Chang

    2007-01-01

    Cisterna chyli is prone to injury in any retroperitoneal surgery. However, retroperitoneal chylous leakage is a rare complication after anterior spinal surgery. To the best of our knowledge, only ten cases have been reported in the English literature. We present a case of a 49-year-old man who had lumbar metastasis and associated radiculopathy. He had transient retroperitoneal chylous leakage after anterior tumor decompression, interbody bony fusion, and instrumental fixation from L2 to L4. The leakage stopped spontaneously after we temporarily clamped the drain tube. Intraperitoneal ascites accumulation developed thereafter due to nutritional loss and impaired hepatic reserves. We gathered ten reported cases of chylous leak after anterior thoracolumbar or lumbar spinal surgery, and categorized all these cases into two groups, depending on the integrity of diaphragm. Six patients received anterior spinal surgery without diaphragm splitting. Postoperative chylous leak stopped after conservative treatment. Another five cases received diaphragm splitting in the interim of anterior spinal surgery. Chylous leakage stopped spontaneously in four patients. The remaining one had a chylothorax secondary to postop chyloretroperitoneum. It was resolved only after surgical intervention. In view of these cases, all the chylous leakage could be spontaneously closed without complications, except for one who had a secondary chylothorax and required thoracic duct ligation and chemopleurodesis. We conclude that intraoperative diaphragm splitting or incision does not increase the risk of secondary chylothorax if it was closed tightly at the end of the surgery and the chest tube drainage properly done. PMID:17273839

  2. Necrotizing cellulitis of the abdominal wall, caused by Pediococcus sp., due to rupture of a retroperitoneal stromal cell tumor

    PubMed Central

    Michalopoulos, Nick; Arampatzi, Stergiani; Papavramidis, Theodossis S.; Kotidis, Efstathios; Laskou, Styliani; Papavramidis, Spiros T.

    2013-01-01

    INTRODUCTION Soft tissue necrotizing infections are a significant cause of morbidity and mortality. The aim of this study is to present a patient with necrotizing infection of abdominal wall resulting from the rupture of a retroperitoneal stromal tumor. PRESENTATION OF CASE We present a 60-year-old Caucasian male patient with necrotizing infection of abdominal wall secondary to the rupture of a retroperitoneal stromal tumor. The patient was initially treated with debridement and fasciotomy of the anterior abdominal wall. Laparotomy revealed purulent peritonitis caused by infiltration and rupture of the splenic flexure by the tumor. Despite prompt intervention the patient died 19 days later. The isolated microorganism causing the infection was the rarely identified as cause of infections in humans Pediococcus sp., a gram-positive, catalase-negative coccus. DISCUSSION Necrotizing infections of abdominal wall are usually secondary either to perineal or to intra-abdominal infections. Gastrointestinal stromal cell tumors could be rarely complicated with perforation and abscess formation. In our case, the infiltrated by the extra-gastrointestinal stromal cell tumor ruptured colon was the source of the infection. The pediococci are rarely isolated as the cause of severe septicemia. CONCLUSION Ruptured retroperitoneal stromal cell tumors are extremely rare cause of necrotizing fasciitis, and before this case, Pediococcus sp. has never been isolated as the responsible agent. PMID:23357010

  3. Injectable hydrogel promotes early survival of induced pluripotent stem cell-derived oligodendrocytes and attenuates longterm teratoma formation in a spinal cord injury model.

    PubMed

    Führmann, T; Tam, R Y; Ballarin, B; Coles, B; Elliott Donaghue, I; van der Kooy, D; Nagy, A; Tator, C H; Morshead, C M; Shoichet, M S

    2016-03-01

    Transplantation of pluripotent stem cells and their differentiated progeny has the potential to preserve or regenerate functional pathways and improve function after central nervous system injury. However, their utility has been hampered by poor survival and the potential to form tumors. Peptide-modified biomaterials influence cell adhesion, survival and differentiation in vitro, but their effectiveness in vivo remains uncertain. We synthesized a peptide-modified, minimally invasive, injectable hydrogel comprised of hyaluronan and methylcellulose to enhance the survival and differentiation of human induced pluripotent stem cell-derived oligodendrocyte progenitor cells. Cells were transplanted subacutely after a moderate clip compression rat spinal cord injury. The hydrogel, modified with the RGD peptide and platelet-derived growth factor (PDGF-A), promoted early survival and integration of grafted cells. However, prolific teratoma formation was evident when cells were transplanted in media at longer survival times, indicating that either this cell line or the way in which it was cultured is unsuitable for human use. Interestingly, teratoma formation was attenuated when cells were transplanted in the hydrogel, where most cells differentiated to a glial phenotype. Thus, this hydrogel promoted cell survival and integration, and attenuated teratoma formation by promoting cell differentiation.

  4. 'Primary extrarenal Wilms' tumour': rare presentation of a common paediatric tumour.

    PubMed

    Goel, Vandana; Verma, Amit Kumar; Batra, Vineeta; Puri, Sunil Kumar

    2014-06-06

    Wilms' tumour (nephroblastoma), the most common abdominal malignancy of childhood, occurs primarily as a malignant renal tumour. Extrarenal Wilms' tumour is rare with occasional reports from the Indian subcontinent. The various locations of extrarenal Wilms' tumour include retroperitoneum, uterus, skin and thorax. In this report we will discuss the imaging features highlighting the imaging differential diagnosis in a case of retroperitoneal (extrarenal) primary Wilms' tumour.

  5. Rapid Decline of Follicular Lymphoma-Associated Chylothorax after Low Dose Radiotherapy to Retroperitoneal Lymphoma Localization

    PubMed Central

    Borger, Jacques; Troost, Esther G. C.; Werner, Philo

    2014-01-01

    Chylothorax is caused by disruption or obstruction of the thoracic duct or its tributaries that results in the leakage of chyle into the pleural space. A number of interventions have been used to treat chylothorax including the treatment of the underlying disease. Lymphoma is found in 70% of cases with nontraumatic malignant aetiology. Although patients usually have advanced lymphoma, supradiaphragmatic disease is not always present. We discuss the case of a 63-year-old woman presenting with progressive respiratory symptoms due to chylothorax. She was diagnosed with a stage IIE retroperitoneal grade 1 follicular lymphoma extending from the coeliac trunk towards the pelvic inlet. Despite thoracocentesis and medium-chain triglycerides (MCT), diet chylothorax reoccurred. After low dose radiotherapy (2 × 2 Gy) to the abdominal lymphoma there was a marked decrease in lymphadenopathy at the coeliac trunk and a complete regression of the pleural fluid. In this case, radiotherapy was shown to be an effective nontoxic treatment option for lymphoma-associated chylothorax with long-term remission of pleural effusion. PMID:24891961

  6. Tissue expander placement and adjuvant radiotherapy after surgical resection of retroperitoneal liposarcoma offers improved local control

    PubMed Central

    Park, Hyojun; Lee, Sanghoon; Kim, BoKyong; Lim, Do Hoon; Choi, Yoon-La; Choi, Gyu Seong; Kim, Jong Man; Park, Jae Berm; Kwon, Choon Hyuck David; Joh, Jae-Won; Kim, Sung Joo

    2016-01-01

    Abstract Given that retroperitoneal liposarcoma (LPS) is extremely difficult to completely resect, and has a relatively high rate of recurrence, radiotherapy (RT) is the treatment of choice after surgical resection. However, it is difficult to obtain a sufficient radiation field because of the close proximity of surrounding organs. We introduce the use of tissue expanders (TEs) after LPS resection in an attempt to secure a sufficient radiation field and to improve recurrence-free survival. This study is a retrospective review of 53 patients who underwent surgical resection of LPS at Samsung Medical Center between January 1, 2005, and December 31, 2012, and had no residual tumor detected 2 months postoperatively. The median follow-up period was 38.9 months. Patients were divided into 3 groups. Those in group 1 (n = 17) had TE inserted and received postoperative RT. The patients in group 2 (n = 9) did not have TE inserted and received postoperative RT. Finally, those in group 3 (n = 27) did not receive postoperative RT. Multivariate analysis was performed to identify the risk factors associated with recurrence-free survival within 3 years. Younger age, history of LPS treatment, and RT after TE insertion (group 1 vs group 2 or 3) were significantly favorable factors influencing 3-year recurrence-free survival. TE insertion after LPS resection is associated with increased 3-year recurrence-free survival, most likely because it allows effective delivery of postoperative RT. PMID:27512857

  7. [Severe Acute Myocardial Infarction during Induction Chemotherapy for Retroperitoneal Germ Cell Tumor : A Case Report].

    PubMed

    Sakka, Shotaro; Kawai, Koji; Tsujimoto, Ippei; Kurobe, Masahiro; Ichioka, Daishi; Kantori, Shuya; Kojima, Takahiro; Suetomi, Takahiro; Jouraku, Akira; Miyazaki, Jun; Hoshi, Tomoya; Nishiyama, Hiroyuki

    2016-09-01

    A 37-year-old man presented at our hospital. Pathological examination of a right orchiectomy specimen, radiographic examination, and tumor marker profile resulted in a diagnosis of retroperitoneal nonseminomatous germ cell tumor (intermediate risk according to IGCC classification). Laboratory testing revealed mild elevation of low density lipoprotein cholesterol. Induction chemotherapy with bleomycin, etoposide and cisplatin (BEP) was started, but he complained of chest pain on day 10 of the second cycle of BEP. We immediately started cardiac monitoring. One hour later, he suffered cardiac arrest due to ventricular fibrillation. Fortunately, sinus rhythm was restored after defibrillation. A diagnosis of acute myocardial infarction (AMI) with total occlusion at the mid-portion of the left anterior descending coronary artery was established by coronary angiography. After percutaneous transluminal coronary angioplasty was successfully performed, he recovered uneventfully. The induction chemotherapy was re-started 19 days after AMI. To avoid endothelial damage by bleomycin, we elected to treat with etoposide, ifosfamide, and cisplatin (VIP). After two further courses of VIP, the patient underwent resection of retoperitoneal tumor and achieved complete remission. The patient has remained disease-free during 3 years follow up without recurrence of AMI.

  8. [A case of appendceal cancer with abdominal, intraabdominal, and retroperitoneal abscesses].

    PubMed

    Fujiwara, Yasuhiro; Nakajima, Masao; Tomochika, Shinobu; Matoba, Katsuhiro

    2014-11-01

    A 65-year-old woman was admitted to our hospital owing to difficulty walking and an abdominal tumor in the right lower abdomen. An irregular mass with calcification was detected in her cecum, and abdominal, intraabdominal, and retroperitoneal abscess was detected by computed tomography(CT). An irregular mass was diagnosed as Group 5 adenocarcinoma by biopsy. After the inflammation improved by abscess drainage, we conducted ileocecal resection and fenestrated the abscess. Pathological analysis showed well-differentiated tubular adenocarcinoma: dimensions 20 × 20 mm, pSS, stroma: med, INF a, ly1, v0, pPM0 (70 mm), pDM0 (70 mm), pRM0 (8mm), LN(-): #201: (0/9), fStageII. Thepatie nts can now walk, and she remains in good health and has been recurrence-free 8 months after surgery. To our knowledge, colon cancer with an abdominal abscess is comparatively rare, and we discuss this in light of the literature.

  9. Anhidrosis after anterior retroperitoneal approach for L4-L5 artificial disc replacement.

    PubMed

    Kasliwal, Manish K; Deutsch, Harel

    2011-07-01

    Description of injury to the sympathetic nervous system after the anterior approach to the lumbosacral spine remains restricted to reports of retrograde ejaculation in males occurring in 2% to 20% of patients. The authors describe a 46-year-old female who presented with low back pain for several years due to degenerative disk disease at L4-L5 that had been treated with an L4-L5 artificial disc replacement though a left anterior retroperitoneal approach. She has had an excellent outcome with complete resolution of her back pain, but noticed the development of dryness of her left lower limb with anhidrosis and skin breakdown, which has caused persistent discomfort. The complication had persisted at her 1-year follow up. Damage to the sympathetic system may often go unrecognized but present with subtle symptoms, as described in the present report. Sympathetic injury in women is often not addressed and remains unrecognized except for a few reports of vaginal dryness or anaesthesia. A short description of this unusual and underappreciated complication, which may be a cause of discomfort despite successful surgery, is presented.

  10. [Opsoclonus-myoclonus syndrome in a 2 year old boy with prenatally diagnosed retroperitoneal tumour].

    PubMed

    Jamroz, Ewa; Głuszkiewicz, Ewa; Madziara, Wojciech; Kiełtyka, Aleksandra

    2011-01-01

    Opsoclonus-myoclonus syndrome, also named Myoclonic Encephalopathy of Infants, Opsoclonus- Myoclonus Ataxia, Dancing Eyes - Dancing Feet Syndrome, Dancing Eyes Syndrome, Kinsbourne syndrome, is a rare, paraneoplastic or possibly post-viral chronic neurological disorder. The age of presentation ranges from 6 months to 3 years. In 50% of affected children the syndrome is associated with an underlying occult or clinically apparent neuroblastoma. In most patients the tumour is localized, small and well differentiated, with no NMYC gene copy number amplification. The syndrome may also occur after tumour resection or at relapse. The opsoclonus-myoclonus syndrome can occur in children without neuroblastoma, in such idiopathiccases, the onset of neurological symptoms is related to infection. It is assumed, that in idiopathic cases the syndrome could have developed in the course of neuroblastoma which had undergone a complete spontaneous regression. The most characteristic clinical features of opsoclonus-myoclonus syndrome are: opsoclonus, myoclonus, ataxia, irritability, mutism and sleep disturbances. The disease course is usually long-term with episodes of remission and relapses. Approximately 80% of children with opsoclonus-myoclonus syndrome suffer from mild to severe neurological handicaps, mainly cognitive impairment. The authors present a 2-year old boy with opsoclonus-myoclonus syndrome preceded by involution of prenatally documented retroperitoneal area tumour.

  11. Rare complication after a transrectal ultrasound guided prostate biopsy: a giant retroperitoneal hematoma.

    PubMed

    Chiancone, Francesco; Mirone, Vincenzo; Fedelini, Maurizio; Meccariello, Clemente; Pucci, Luigi; Carrino, Maurizio; Fedelini, Paolo

    2016-05-24

    Common complications related to transrectal ultrasound (TRUS) guided prostatic needle biopsy are hematuria, hematospermia, and hematochezia. To the best of our knowledge, we report the second case of a very large hematoma extending from the pelvis into the retroperitoneal space in literature.A 66-year-old man with a serum prostate-specific antigen (PSA) of 5.4 ng/ml was admitted to our department for a TRUS-guided prostatic needle biopsy. Laboratory values on the day before biopsy, including coagulation studies, were all normal. The patients did not take any anticoagulant drugs. No immediate complications were encountered. Nevertheless, 7 hours after the biopsy, the patient reached our emergency department with severe diffuse abdominal pain, hypotension, tachycardia, and confusional state. He underwent an ultrasonography and then a computed tomography (CT) scan that showed "a blood collection in the pelvis that extending to the lower pole of left kidney associated with a focus of active contrast extravasation, indicating active ongoing prostate bleeding." Consequently, he underwent a diagnostic angiography that showed no more contrast extravasation, without the need of embolization. Management of hematoma has been conservative and hematoma was completely reabsorbed 4 months later.

  12. Percutaneous Retroperitoneal Splenorenal Shunt for Symptomatic Portal Vein Thrombosis After Liver Transplantation.

    PubMed

    Pulitano, C; Rogan, C; Sandroussi, C; Verran, D; McCaughan, G W; Waugh, R; Crawford, M

    2015-08-01

    Acute or recurrent bleeding from ectopic varices is a potentially life-threatening condition in rare patients with extrahepatic complete portal vein thrombosis (PVT) after liver transplantation (LT). In this setting, the role of interventional radiology is very limited and surgical shunts, in particular splenorenal shunts are usually used, despite the high associated mortality. We present the first reports of the clinical use of a new minimally invasive technique, percutaneous retroperitoneal splenorenal shunt (PRESS), in two LT recipients with life-threatening variceal hemorrhage secondary to PVT. Both patients had a successful PRESS using a transplenic approach with resolution of bleeding, avoiding the need for a potentially complicated laparotomy. The PRESS procedure is a useful addition to the interventional armamentarium that can be used in cases unsuitable for surgical shunt, and refractory to endoscopic management. In the future, this technique may be an alternative to surgical shunts as the standard procedure in patients with extra-hepatic PVT, just as the transjugular intrahepatic portosystemic shunt (TIPS) procedure has become for the management of portal hypertension in the absence of PVT. Longer-term follow-up will be needed to establish the long-term success of this procedure.

  13. Retroperitoneal Sarcoma Involving Unilateral Double Ureter: Management, Treatment and Psychological Implications

    PubMed Central

    Leanza, Vito; Garraffo, Claudia; Leanza, Gianluca; Leanza, Antonio

    2014-01-01

    The case of a 45-year-old woman who was admitted to our university hospital for polymenorrhea, weight gain and pain in the left iliac region is reported. An abdominal ultrasound revealed a 9.5 × 5.2-cm, hypoechoic and inhomogeneous mass located on the left side of the pelvis and behind the ovary. The patient underwent surgery. The pelvic mass was firmly anchored to the small intestine, colon, sigma and uterine fundus. After removing the adhesions, double ureters, which had been incorporated in the mass, were observed on the left side. Resection of the unilateral double ureters was necessary in order to remove the entire mass, and thereafter, a left salpingoophorectomy was performed. A histological examination showed a malignant retroperitoneal mass. Termino-terminal ureteral anastomosis with two double-J stents was carried out. Total hysterectomy with preservation of the right adenexum and regional lymphadenectomy was performed. The purpose of this case report is to discuss the physical and psychological implications related to the combination of two rare entities: leiomyosarcoma and a double ureter located within the mass. A literature review on the clinical management and psychological aspects from a female cancer patient's perspective undergoing surgery with the aforementioned disorders will be discussed. PMID:24932171

  14. Tissue expander placement and adjuvant radiotherapy after surgical resection of retroperitoneal liposarcoma offers improved local control.

    PubMed

    Park, Hyojun; Lee, Sanghoon; Kim, BoKyong; Lim, Do Hoon; Choi, Yoon-La; Choi, Gyu Seong; Kim, Jong Man; Park, Jae Berm; Kwon, Choon Hyuck David; Joh, Jae-Won; Kim, Sung Joo

    2016-08-01

    Given that retroperitoneal liposarcoma (LPS) is extremely difficult to completely resect, and has a relatively high rate of recurrence, radiotherapy (RT) is the treatment of choice after surgical resection. However, it is difficult to obtain a sufficient radiation field because of the close proximity of surrounding organs. We introduce the use of tissue expanders (TEs) after LPS resection in an attempt to secure a sufficient radiation field and to improve recurrence-free survival.This study is a retrospective review of 53 patients who underwent surgical resection of LPS at Samsung Medical Center between January 1, 2005, and December 31, 2012, and had no residual tumor detected 2 months postoperatively. The median follow-up period was 38.9 months.Patients were divided into 3 groups. Those in group 1 (n = 17) had TE inserted and received postoperative RT. The patients in group 2 (n = 9) did not have TE inserted and received postoperative RT. Finally, those in group 3 (n = 27) did not receive postoperative RT. Multivariate analysis was performed to identify the risk factors associated with recurrence-free survival within 3 years. Younger age, history of LPS treatment, and RT after TE insertion (group 1 vs group 2 or 3) were significantly favorable factors influencing 3-year recurrence-free survival.TE insertion after LPS resection is associated with increased 3-year recurrence-free survival, most likely because it allows effective delivery of postoperative RT.

  15. Multiple nevoid basal cell carcinoma syndrome associated with congenital orbital teratoma, caused by a PTCH1 frameshift mutation.

    PubMed

    Rodrigues, A L; Carvalho, A; Cabral, R; Carneiro, V; Gilardi, P; Duarte, C P; Puente-Prieto, J; Santos, P; Mota-Vieira, L

    2014-07-25

    Gorlin-Goltz syndrome, or nevoid basal cell carcinoma syndrome (NBCCS), is a rare autosomal dominant disorder caused by mutations in the PTCH1 gene and shows a high level of penetrance and variable expressivity. The syndrome is characterized by developmental abnormalities or neoplasms and is diagnosed with 2 major criteria, or with 1 major and 2 minor criteria. Here, we report a new clinical manifestation associated with this syndrome in a boy affected by NBCCS who had congenital orbital teratoma at birth. Later, at the age of 15 years, he presented with 4 major and 4 minor criteria of NBCCS, including multiple basal cell carcinoma and 2 odontogenic keratocysts of the jaw, both confirmed by histology, more than 5 palmar pits, calcification of the cerebral falx, extensive meningeal calcifications, macrocephaly, hypertelorism, frontal bosses, and kyphoscoliosis. PTCH1 mutation analysis revealed the heterozygous germline mutation c.290dupA. This mutation generated a frameshift within exon 2 and an early premature stop codon (p.Asn97LysfsX43), predicting a truncated protein with complete loss of function. Identification of this mutation is useful for genetic counseling. Although the clinical symptoms are well-known, our case contributes to the understanding of phenotypic variability in NBCCS, highlighting that PTCH1 mutations cannot be used for predicting disease burden and reinforces the need of a multidisciplinary team in the diagnosis, treatment, and follow-up of NBCCS patients.

  16. A Rare Case of Primary Anterior Mediastinal Yolk Sac Tumor in an Elderly Adult Male

    PubMed Central

    Nakhla, Sammy G.; Sundararajan, Srinath

    2016-01-01

    Mediastinal germ cell tumors are extragonadal germ cell tumors (EGGCTs) commonly seen in children and young adults. They are more common in men. Clinically they are classified as teratomas, seminomas, and nonseminomatous germ cell tumors. Primary mediastinal yolk sac neoplasm is an extremely rare tumor. We present here a very rare case of primary yolk sac tumor of the anterior mediastinum in a 73-year-old male. Mediastinal germ cell tumors have a worse prognosis than gonadal germ cell tumors. Chemotherapy followed by adjuvant surgery improves overall response in EGGCTs. However, comorbidities can render treatment with chemotherapy and surgery challenging in elderly patients. PMID:27144043

  17. Late effects of intraoperative radiation therapy on retroperitoneal tissues, intestine, and bile duct in a large animal model

    SciTech Connect

    Sindelar, W.F.; Tepper, J.E.; Kinslla, T.J.; Barnes, M.; DeLuca, A.M.; Terrill, R.; Matthews, D.; Johnstone, P.A.S.; Anderson, W.J.; Bollinger, B.K.

    1994-07-01

    The late histopathological effects of intraoperative radiotherapy (IORT) on retroperitoneal tissues, intestine, and bile duct were investigated in dogs. Fourteen adult foxhounds were subjected to laparotomy and varying doses (0-45 Gy) of IORT (11 MeV electrons) delivered to retroperitoneal tissues including the great vessels and ureters, to a loop of defunctionalized small bowel, or to the extrahepatic bile duct. One control animal received an aortic transection and reanastomosis at the time of laparotomy; another control received laparotomy alone. This paper describes the late effects of single-fraction IORT occurring 3-5 years following treatment. Dogs receiving IORT to the retroperitoneum through a 4 X 15 cm portal showed few gross or histologic abnormalities at 20 Gy. At doses ranging from 30-45 Gy, radiation changes in normal tissues were consistently observed. Retroperitoneal fibrosis with encasement of the ureters and great vessels developed at doses {ge}30 Gy. Radiation changes were present in the aorta and vena cava at doses {ge}40 Gy. A 30 Gy dog developed an in-field malignant osteosarcoma at 3 years which invaded the vertebral column and compressed the spinal cord. A 40 Gy animal developed obstruction of the right ureter with fatal septic hydronephrosis at 4 years. Animals receiving IORT through a 5 cm IORT portal to an upper abdominal field which included a defunctionalized loop of small bowel, showed few gross or histologic abnormalities at a dose of 20 Gy. At 30 Gy, hyaline degeneration of the intestinal muscularis layer of the bowel occurred. At a dose of 45 Gy, internal intestinal fistulae developed. One 30 Gy animal developed right ureteral obstruction and hydronephrosis at 5 years. A dog receiving 30 Gy IORT through a 5 cm portal to the extrahepatic bile duct showed diffuse fibrosis through the gastroduodenal ligament. These canine studies contribute to the area of late tissue tolerance to IORT. 7 refs., 3 figs., 5 tabs.

  18. Intensity modulated radiation-therapy for preoperative posterior abdominal wall irradiation of retroperitoneal liposarcomas

    SciTech Connect

    Bossi, Alberto . E-mail: alberto.bossi@uz.kuleuven.ac.be; De Wever, Ivo; Van Limbergen, Erik; Vanstraelen, Bianca

    2007-01-01

    Purpose: Preoperative external-beam radiation therapy (preop RT) in the management of Retroperitoneal Liposarcomas (RPLS) typically involves the delivery of radiation to the entire tumor mass: yet this may not be necessary. The purpose of this study is to evaluate a new strategy of preop RT for RPLS in which the target volume is limited to the contact area between the tumoral mass and the posterior abdominal wall. Methods and Materials: Between June 2000 and Jan 2005, 18 patients with the diagnosis of RPLS have been treated following a pilot protocol of pre-op RT, 50 Gy in 25 fractions of 2 Gy/day. The Clinical Target Volume (CTV) has been limited to the posterior abdominal wall, region at higher risk for local relapse. A Three-Dimensional conformal (3D-CRT) and an Intensity Modulated (IMRT) plan were generated and compared; toxicity was reported following the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events v3.0. Results: All patients completed the planned treatment and the acute toxicity was tolerable: 2 patients experienced Grade 3 and 1 Grade 2 anorexia while 2 patients developed Grade 2 nausea. IMRT allows a better sparing of the ipsilateral and the contralateral kidney. All tumors were successfully resected without major complications. At a median follow-up of 27 months 2 patients developed a local relapse and 1 lung metastasis. Conclusions: Our strategy of preop RT is feasible and well tolerated: the rate of resectability is not compromised by limiting the preop CTV to the posterior abdominal wall and a better critical-structures sparing is obtained with IMRT.

  19. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    SciTech Connect

    Baldini, Elizabeth H.; Abrams, Ross A.; Bosch, Walter; Roberge, David; Haas, Rick L.M.; Catton, Charles N.; Indelicato, Daniel J.; Olsen, Jeffrey R.; Deville, Curtiland; Chen, Yen-Lin; Finkelstein, Steven E.; DeLaney, Thomas F.; Wang, Dian

    2015-08-01

    Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.

  20. Relationship between impaired adipogenesis of retroperitoneal adipose tissue and hypertrophic obesity: role of endogenous glucocorticoid excess

    PubMed Central

    Zubiría, María G; Vidal-Bravo, Juana; Spinedi, Eduardo; Giovambattista, Andrés

    2014-01-01

    Although the pro-adipogenic effect of glucocorticoid (GC) on adipose tissue (AT) precursor cell differentiation is openly accepted, the effect of chronically high peripheral levels of GC on AT mass expansion is not fully understood. In the present study, we aim to assess the in vitro adipogenic capacity of AT precursor cells isolated from retroperitoneal (RP) AT pads of the hypercorticosteronaemic, adult neonatally treated monosodium L-glutamate (MSG) male rat. To ascertain this issue, we explored the in vitro adipogenic process of stromal-vascular fraction (SVF) cells isolated from RPAT pads of 60-day-old MSG rats. The data recorded indicated that RPAT-SVF cells from hypercorticosteronaemic MSG rats, although displaying an enhanced proliferation capacity, differentiated slower than normal cells. This dysfunction was associated with a reduction in key parameters indicative of precursor cell commitment, differentiation capacity and the percentage of fully differentiated adipocytes, with a retarded maturation process. The distorted adipogenic capacity was highly conditioned by RPAT-SVF cells displaying a low committed population and both excessive and reduced expression of anti- (Pref-1 and Wnt-10b) and pro-adipogenic (mineralocorticoid receptor) signals respectively. Notably, the normalization of peripheral corticosterone levels in MSG rats, as a result of bilateral adrenalectomy combined with GC replacement therapy, fully prevented reduced RPAT precursor cell commitment and overall impaired adipogenesis. Our study strongly supports that the impaired adipogenic process observed in the adult hypertrophic obese MSG male rat is a GC-dependent mechanism, thus explaining the unhealthy RPAT expansion observed in human hypertrophic obese phenotypes, such as in the Cushing's syndrome. PMID:24913911

  1. Treatment Guidelines for Preoperative Radiation Therapy for Retroperitoneal Sarcoma: Preliminary Consensus of an International Expert Panel

    SciTech Connect

    Baldini, Elizabeth H.; Wang, Dian; Haas, Rick L.M.; Catton, Charles N.; Indelicato, Daniel J.; Kirsch, David G.; Roberge, David; Salerno, Kilian; Deville, Curtiland; Guadagnolo, B. Ashleigh; O'Sullivan, Brian; Petersen, Ivy A.; Le Pechoux, Cecile; Abrams, Ross A.; DeLaney, Thomas F.

    2015-07-01

    Purpose: Evidence for external beam radiation therapy (RT) as part of treatment for retroperitoneal sarcoma (RPS) is limited. Preoperative RT is the subject of a current randomized trial, but the results will not be available for many years. In the meantime, many practitioners use preoperative RT for RPS, and although this approach is used in practice, there are no radiation treatment guidelines. An international expert panel was convened to develop consensus treatment guidelines for preoperative RT for RPS. Methods and Materials: An expert panel of 15 academic radiation oncologists who specialize in the treatment of sarcoma was assembled. A systematic review of reports related to RT for RPS, RT for extremity sarcoma, and RT-related toxicities for organs at risk was performed. Due to the paucity of high-quality published data on the subject of RT for RPS, consensus recommendations were based largely on expert opinion derived from clinical experience and extrapolation of relevant published reports. It is intended that these clinical practice guidelines be updated as pertinent data become available. Results: Treatment guidelines for preoperative RT for RPS are presented. Conclusions: An international panel of radiation oncologists who specialize in sarcoma reached consensus guidelines for preoperative RT for RPS. Many of the recommendations are based on expert opinion because of the absence of higher level evidence and, thus, are best regarded as preliminary. We emphasize that the role of preoperative RT for RPS has not been proven, and we await data from the European Organization for Research and Treatment of Cancer (EORTC) study of preoperative radiotherapy plus surgery versus surgery alone for patients with RPS. Further data are also anticipated pertaining to normal tissue dose constraints, particularly for bowel tolerance. Nonetheless, as we await these data, the guidelines herein can be used to establish treatment uniformity to aid future assessments of efficacy

  2. Open surgery versus retroperitoneal laparoscopic nephrectomy for renal tuberculosis: a retrospective study of 120 patients

    PubMed Central

    Wang, Cheng; Xiong, Hu; Fu, Sheng-Jun

    2016-01-01

    Background Laparoscopic renal surgery has been widely used in the treatment of renal diseases. However, there is still little research about its application in addressing renal tuberculosis. The purpose of this study is to retrospectively investigate the surgical results of laparoscopic and open surgery for nonfunctional tuberculous kidneys. Methods Between May 2011 and June 2016, 120 nephrectomies were performed in patients with a nonfunctional tuberculous kidney. Of these, 69 patients underwent retroperitoneal laparoscopic nephrectomy, and 51 patients underwent open nephrectomy. Data about the patients’ characteristics and surgical outcomes were collected from their electronic medical records. Outcomes were compared between these two groups. Results Our results showed that a number of renal tuberculosis patients presented no significant symptoms during their disease. Lower urinary tract symptoms (LUTS) were the most common at a rate of 73/120, followed by flank pain or accidently discovery (66/120), urine abnormality (30/120) and fever (27/120). Patients who underwent open surgery were similar to laparoscopic patients with regard to sex, BMI, location, previous tuberculous history, grade, anemia, adhesion, hypertension, diabetes and preoperative serum creatinine level, but were generally older than laparoscopic patients. There were no significant differences between open and laparoscopic surgery in estimated blood loss, transfusion, postoperative hospital days and perioperative complication rate. However, the median operation time of laparoscopic operation was much longer than open surgery (180 [150–225] vs 135 [120–165] minutes, P < 0.01). Seven of the 69 laparoscopic operations were converted to open surgery because of severe adhesions. Conclusion Laparoscopic nephrectomy is as an effective treatment as open surgery for a nonfunctional tuberculous kidney, although it requires more time during the surgical procedure. No significant differences in other

  3. Combined Modality Management of Retroperitoneal Sarcomas: A Single-Institution Series of 121 Patients

    SciTech Connect

    Bishop, Andrew J.; Zagars, Gunar K.; Torres, Keila E.; Hunt, Kelly K.; Cormier, Janice N.; Feig, Barry W.; Guadagnolo, B. Ashleigh

    2015-09-01

    Purpose: The purpose of this study was to investigate local control, survival outcomes, and complication rates of patients treated with aggressive surgery and radiation therapy (RT) for retroperitoneal sarcomas (RPS). Methods and Materials: We reviewed the medical records of 121 consecutive patients treated for RPS with surgery and RT between 1965 and 2012. The most common histology was liposarcoma (n=42; 35%). The median follow-up was 100 months (range: 20-467 months). Eighty-six patients (71%) were treated for initial presentation of RPS, and 35 patients (29%) presented with and were treated for RPS recurrence. RT was preoperative in 88 patients (73%; median dose: 50.4 Gy) and postoperative in 33 patients (27%; median dose: 55 Gy). Results: Five-year local control and overall survival rates were 56% and 57%, respectively. Two factors were associated with higher risk of any intra-abdominal recurrence at 5 years: positive or uncertain margins (58% vs 30% for negative margins, P<.001; hazard ratio [HR]: 2.7; 95% confidence interval [CI]: 1.6-4.8) and disease recurrence after previous resection (76% vs 31% for de novo RPS, P<.001; HR: 4.4; 95% CI: 2.5-7.5). The 10-year complication rate was 5%, and RT-related complications were associated with postoperative RT (P<.001) and RT dose of ≥60 Gy (P<.001). Conclusions: Intra-abdominal RPS recurrence continues to be a significant challenge despite the use of aggressive surgery and radiation therapy. Given the complications associated with postoperative radiation therapy, we recommend that preoperative radiation therapy is the preferred strategy when combined modality therapy is recommended.

  4. Minimally Invasive Necrosectomy Techniques in Severe Acute Pancreatitis: Role of Percutaneous Necrosectomy and Video-Assisted Retroperitoneal Debridement

    PubMed Central

    Logue, Jennifer A.; Carter, C. Ross

    2015-01-01

    Consensus advocating a principle of early organ support, nutritional optimisation, followed ideally by delayed minimally invasive intervention within a “step-up” framework where possible has radically changed the surgical approach to complications of acute pancreatitis in the last 20 years. The 2012 revision of the Atlanta Classification incorporates these changes, and provides a background which underpins the complexities of individual patient management decisions. This paper discusses the place for delayed minimally invasive surgical intervention (percutaneous necrosectomy, video-assisted retroperitoneal debridement (VARD)), and the rationale for opting to adopt a percutaneous approach over endoscopic or laparoscopic approaches in different clinical situations. PMID:26587018

  5. [The surgery after. . . retroperitoneal lymph node dissection and surgery of the residual masses after chemotherapy for advanced testicular cancer].

    PubMed

    Guillemin, François; Belinga, Jean-François Betala; Marchal, Frédéric; Rauch, Philippe; Verhaeghe, Jean-Luc

    2011-01-01

    Thirty-six cases of retroperitoneal lymph node dissections for residual mass after chemotherapy for testicular cancer are reported. In a reference center, the recruitment is modified by the severity of the situations related to very big masses, tumors of poor prognosis and resistant tumors. Lymph node dissection is often atypical and surgery of metastatic residual masses is frequent (13 operations). The 8-year global survival remains stable, over 90%. The 5-year cumulated risk of recurrence is 20%, but these situations can be overtaken.

  6. Retroperitoneal cold abscess with tuberculosis of sacro-iliac joint and pubic bone: a case report of unusual presentation of osteo-articular tuberculosis.

    PubMed

    Ramakrishnaiah, V P; Jain, V; Choon, A T; Rao, B H

    2000-03-01

    Tuberculosis involving sacro-iliac joint and pubic bone presenting with massive retroperitoneal abscess is a rare entity. A 29-year-old female presented with history of discharging sinus in the sacrococcygeal region of 2 months duration. Plain x-ray revealed osteolytic lesion in right pubic bone and left sacro-iliac joint. Computed tomography scan revealed massive pus collection in the retroperitoneal region. Pus was drained extraperitoneally. Biopsy of the scraping of the abscess wall showed granulation tissue with foreign body type of giant cell. On follow-up the patient was doing well.

  7. Non-genomic estrogen/estrogen receptor α promotes cellular malignancy of immature ovarian teratoma in vitro.

    PubMed

    Hung, Yao-Ching; Chang, Wei-Chun; Chen, Lu-Min; Chang, Ying-Yi; Wu, Ling-Yu; Chung, Wei-Min; Lin, Tze-Yi; Chen, Liang-Chi; Ma, Wen-Lung

    2014-06-01

    Malignant immature ovarian teratomas (IOTs) most often occur in women of reproductive age. It is unclear, however, what roles estrogenic signaling plays in the development of IOT. In this study, we examined whether estrogen receptors (ERα and β) promote the cellular malignancy of IOT. Estradiol (E2), PPT (propylpyrazole), and DPN (diarylpropionitrile) (ERα- and β-specific agonists, respectively), as well as ERα- or ERβ-specific short hairpin (sh)RNA were applied to PA-1 cells, a well-characterized IOT cell line. Cellular tumorigenic characteristics, for example, cell migration/invasion, expression of the cancer stem/progenitor cell marker CD133, and evidence for epithelial-mesenchymal transition (EMT) were examined. In PA-1 cells that expressed ERα and ERβ, we found that ERα promoted cell migration and invasion. We also found that E2/ERα signaling altered cell behavior through non-classical transactivation function. Our data show non-genomic E2/ERα activations of focal adhesion kinase-Ras homolog gene family member A (FAK-RhoA) and ERK governed cell mobility capacity. Moreover, E2/ERα signaling induces EMT and overexpression of CD133 through upregulation micro-RNA 21 (miR21; IOT stem/progenitor promoter), and ERK phosphorylations. Furthermore, E2/ERα signaling triggers a positive feedback regulatory loop within miR21 and ERK. At last, expression levels of ERα, CD133, and EMT markers in IOT tissue samples were examined by immunohistochemistry. We found that cytosolic ERα was co-expressed with CD133 and mesenchymal cell markers but not epithelial cell markers. In conclusion, estrogenic signals exert malignant transformation capacity of cancer cells, exclusively through non-genomic regulation in female germ cell tumors.

  8. Ginkgo biloba Extract Improves Insulin Signaling and Attenuates Inflammation in Retroperitoneal Adipose Tissue Depot of Obese Rats

    PubMed Central

    Hirata, Bruna Kelly Sousa; Banin, Renata Mancini; Dornellas, Ana Paula Segantine; de Andrade, Iracema Senna; Zemdegs, Juliane Costa Silva; Caperuto, Luciana Chagas; Oyama, Lila Missae; Ribeiro, Eliane Beraldi; Telles, Monica Marques

    2015-01-01

    Due to the high incidence and severity of obesity and its related disorders, it is highly desirable to develop new strategies to treat or even to prevent its development. We have previously described that Ginkgo biloba extract (GbE) improved insulin resistance and reduced body weight gain of obese rats. In the present study we aimed to evaluate the effect of GbE on both inflammatory cascade and insulin signaling in retroperitoneal fat depot of diet-induced obese rats. Rats were fed with high fat diet for 2 months and thereafter treated for 14 days with 500 mg/kg of GbE. Rats were then euthanized and samples from retroperitoneal fat depot were used for western blotting, RT-PCR, and ELISA experiments. The GbE treatment promoted a significant reduction on both food/energy intake and body weight gain in comparison to the nontreated obese rats. In addition, a significant increase of both Adipo R1 and IL-10 gene expressions and IR and Akt phosphorylation was also observed, while NF-κB p65 phosphorylation and TNF-α levels were significantly reduced. Our data suggest that GbE might have potential as a therapy to treat obesity-related metabolic diseases, with special interest to treat obese subjects resistant to adhere to a nutritional education program. PMID:25960614

  9. Three-dimensional segmentation of retroperitoneal masses using continuous convex relaxation and accumulated gradient distance for radiotherapy planning.

    PubMed

    Suárez-Mejías, Cristina; Pérez-Carrasco, Jose Antonio; Serrano, Carmen; López-Guerra, Jose Luis; Parra-Calderón, Carlos; Gómez-Cía, Tomás; Acha, Begoña

    2017-01-01

    An innovative algorithm has been developed for the segmentation of retroperitoneal tumors in 3D radiological images. This algorithm makes it possible for radiation oncologists and surgeons semiautomatically to select tumors for possible future radiation treatment and surgery. It is based on continuous convex relaxation methodology, the main novelty being the introduction of accumulated gradient distance, with intensity and gradient information being incorporated into the segmentation process. The algorithm was used to segment 26 CT image volumes. The results were compared with manual contouring of the same tumors. The proposed algorithm achieved 90 % sensitivity, 100 % specificity and 84 % positive predictive value, obtaining a mean distance to the closest point of 3.20 pixels. The algorithm's dependence on the initial manual contour was also analyzed, with results showing that the algorithm substantially reduced the variability of the manual segmentation carried out by different specialists. The algorithm was also compared with four benchmark algorithms (thresholding, edge-based level-set, region-based level-set and continuous max-flow with two labels). To the best of our knowledge, this is the first time the segmentation of retroperitoneal tumors for radiotherapy planning has been addressed.

  10. The presence of centrioles and centrosomes in ovarian mature cystic teratoma cells suggests human parthenotes developed in vitro can differentiate into mature cells without a sperm centriole

    SciTech Connect

    Lee, Bo Yon; Shim, Sang Woo; Kim, Young Sun; Kim, Seung Bo

    2011-11-18

    Highlights: Black-Right-Pointing-Pointer The sperm centriole is the progenitor of centrosomes in all somatic cells. Black-Right-Pointing-Pointer Centrioles and centrosomes exist in parthenogenetic ovarian teratoma cells. Black-Right-Pointing-Pointer Without a sperm centriole, parthenogenetic oocytes produce centrioles and centrosomes. Black-Right-Pointing-Pointer Parthenogenetic human oocytes can develop and differentiate into mature cells. -- Abstract: In most animals, somatic cell centrosomes are inherited from the centriole of the fertilizing spermatozoa. The oocyte centriole degenerates during oogenesis, and completely disappears in metaphase II. Therefore, the embryos generated by in vitro parthenogenesis are supposed to develop without any centrioles. Exceptional acentriolar and/or acentrosomal developments are possible in mice and in some experimental cells; however, in most animals, the full developmental potential of parthenogenetic cells in vitro and the fate of their centrioles/centrosomes are not clearly understood. To predict the future of in vitro human parthenogenesis, we explored the centrioles/centrosomes in ovarian mature cystic teratoma cells by immunofluorescent staining and transmission electron microscopy. We confirmed the presence of centrioles and centrosomes in these well-known parthenogenetic ovarian tumor cells. Our findings clearly demonstrate that, even without a sperm centriole, parthenotes that develop from activated oocytes can produce their own centrioles/centrosomes, and can even develop into the well-differentiated mature tissue.

  11. Transformation of follicular lymphoma in the retroperitoneal muscles demonstrated by CT-guided needle biopsy of FDG-avid lesions; case series.

    PubMed

    Uni, Masahiro; Nakamura, Fumihiko; Yoshimi, Akihide; Shinozaki-Ushiku, Aya; Hosoi, Atsuko; Nakazaki, Kumi; Nannya, Yasuhito; Fukayama, Masashi; Kurokawa, Mineo

    2014-01-01

    We herein report two cases of relapsed follicular lymphoma (FL) with transformation in the retroperitoneal muscles. Fluorodeoxyglucose (FDG)-positron emission tomography (PET) combined with computed tomography (CT) showed high uptakes in the retroperitoneal muscles. We considered excisional biopsy at first, since it is definitely the most reliable means to obtain histological diagnosis. However, excisional biopsy of the retroperitoneal muscles is challenging for anatomical reasons. Moreover, our patients were kept under poor performance status. Thus, CT-guided percutaneous needle biopsy of FDG-avid retroperitoneal muscles was performed. Histopathological examination of the biopsied specimens demonstrated proliferation of transformed large B cells in both cases. Sheets of large B cells were also recorded in one case. CT-guided needle biopsy is less prioritized than excisional biopsy because of limited information on tissue architecture and increasingly complicated WHO classification. Our series indicate that image-guided needle biopsy of FDG-avid lesions is sufficient for the diagnosis of transformation. Higher priority should be given to this method in the setting of transformed aggressive lymphoma.

  12. Solitary Bone Plasmacytoma Progressing into Retroperitoneal Plasma Cell Myeloma with No Related End Organ or Tissue Impairment: A Case Report and Review of the Literature

    PubMed Central

    Tikku, Gargi; Jain, Monica; Mridha, Asit; Grover, Rajesh

    2014-01-01

    Solitary bone plasmacytomas and plasma cell myeloma are clonal proliferations of plasma cells. Many patients with solitary bone plasmacytomas develop plasma cell myeloma on follow-up. We present a case of a 70-year-old man who presented with fracture and a lytic lesion in the subtrochanteric region of the left femur and was assigned a diagnosis of solitary bone plasmacytoma. He received local curative radiotherapy. However, 4 months later his serum M protein and β2-microglobulin levels increased to 2.31 g/dL and 5.965 mg/L, respectively. He complained of abdominal fullness and constipation. Ultrasound and non-contrast CT imaging revealed multiple retroperitoneal masses. Colonoscopic examination was normal. Biopsy of the a retroperitoneal mass confirmed it to be a plasmacytoma. Repeat hemogram, blood urea, serum creatinine, skeletal survey, and bone marrow examination revealed no abnormalities. This is an unusual presentation of plasma cell myeloma, which manifested as multiple huge extramedullary retroperitoneal masses and arose from a solitary bone plasmacytoma, without related end organ or tissue impairment and bone marrow plasmacytosis. The patient succumbed to his disease 8 months after the appearance of the retroperitoneal masses. This case highlights the importance of close monitoring of patients diagnosed with solitary bone plasmacytoma with increased serum M protein and serum β2-microglobulin levels, so that early therapy can be instituted to prevent conversion to plasma cell myeloma. PMID:25330522

  13. Solitary bone plasmacytoma progressing into retroperitoneal plasma cell myeloma with no related end organ or tissue impairment: a case report and review of the literature.

    PubMed

    Tikku, Gargi; Jain, Monica; Mridha, Asit; Grover, Rajesh

    2014-09-05

    Solitary bone plasmacytomas and plasma cell myeloma are clonal proliferations of plasma cells. Many patients with solitary bone plasmacytomas develop plasma cell myeloma on follow-up. We present a case of a 70-year-old man who presented with fracture and a lytic lesion in the subtrochanteric region of the left femur and was assigned a diagnosis of solitary bone plasmacytoma. He received local curative radiotherapy. However, 4 months later his serum M protein and β2-microglobulin levels increased to 2.31 g/dL and 5.965 mg/L, respectively. He complained of abdominal fullness and constipation. Ultrasound and non-contrast CT imaging revealed multiple retroperitoneal masses. Colonoscopic examination was normal. Biopsy of the a retroperitoneal mass confirmed it to be a plasmacytoma. Repeat hemogram, blood urea, serum creatinine, skeletal survey, and bone marrow examination revealed no abnormalities. This is an unusual presentation of plasma cell myeloma, which manifested as multiple huge extramedullary retroperitoneal masses and arose from a solitary bone plasmacytoma, without related end organ or tissue impairment and bone marrow plasmacytosis. The patient succumbed to his disease 8 months after the appearance of the retroperitoneal masses. This case highlights the importance of close monitoring of patients diagnosed with solitary bone plasmacytoma with increased serum M protein and serum β2-microglobulin levels, so that early therapy can be instituted to prevent conversion to plasma cell myeloma.

  14. Successful treatment of a rare extended retroperitoneal necrotizing soft tissue infection caused by extended-spectrum beta-lactamase-producing Escherichia coli

    PubMed Central

    He, Rui; Qi, Xin; Wen, Bing; Li, XiangYan; Guo, Li

    2016-01-01

    Abstract Rationale: Retroperitoneal necrotizing soft tissue infection (NSTI) is a rare but life-threatening disease. Here, we present a case of extended retroperitoneal NSTI caused by extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli (E coli). Patient concerns: The patient complained of progressive redness, swelling, and right flank pain for 10 days, extending to the scrotum for 1 day. Diagnoses: He was admitted with an initial diagnosis of cellulitis. Interventions: Debridement was performed after the scrotum developed necrosis on day 2 of hospitalization. The source of infection was found to be an idiopathic retroperitoneal abscess, which was confirmed by computed tomography. Two consecutive microbiological cultures (aerobic plus anaerobic) of the tissue revealed the presence of ESBL-producing E coli. With the application of negative pressure wound therapy (NPWT), we sutured the wound after consecutive debridement. Outcomes: During the 32 months of follow-up, the patient recovered very well and felt extremely satisfied. Lessons: This case reminds us that ESBL-producing E coli can cause retroperitoneal abscesses, which may induce NSTI. Aggressive debridement and broad-spectrum antibiotics should be administrated immediately when NSTI is suspected, and NPWT is an effective adjuvant therapy for wound closure. PMID:27930570

  15. PET imaging of primary mediastinal tumours.

    PubMed Central

    Kubota, K.; Yamada, S.; Kondo, T.; Yamada, K.; Fukuda, H.; Fujiwara, T.; Ito, M.; Ido, T.

    1996-01-01

    Mediastinal masses include a wide variety of tumours and remain an interesting diagnostic challenge for radiologist. We performed positron emission tomography (PET) studies of primary mediastinal tumours in order to predict the malignancy of these tumours preoperatively. Twenty-two patients with primary mediastinal tumours were studied with PET using 2-deoxy-2-[18F]fluoro-D-glucose (FDG). The histological findings of surgical pathology or biopsy, or mediastinoscopy were compared with those of computerised tomography (CT) and PET. PET images were evaluated semiquantitatively using the differential uptake ratio (DUR). Increased FDG uptake was observed in nine of ten patients with malignant tumours, including thymic carcinomas, lymphomas, invasive thymomas and a case of sarcoidosis. A moderate level of FDG uptake was found in a myeloma, non-invasive thymomas, and a schwannoma, whereas a low uptake was observed in a teratoma and various benign cysts. The mean FDG uptake of malignant tumours was significantly higher than that of benign tumours. Both thymic cancer and invasive thymoma showed a high FDG uptake. CT examination resulted in three false-negative and two false-positive cases when used in predicting tumour invasion, while PET was associated with a false-positive and a false-negative case. In conclusion, the use of FDG with PET is clinically helpful in evaluating the malignant nature of primary mediastinal tumours. Our results also suggest that a high FDG uptake reflects the invasiveness of malignant nature of thymic tumours. Images Figure 1 Figure 2 PMID:8611400

  16. Uncommon cause of life-threatening retroperitoneal hemorrhage in a healthy young hispanic patient: Splenic artery aneurysm rupture

    PubMed Central

    Figueroa-Jiménez, Luis A.; González-Márquez, Amy Lee; Negrón-García, Luis; Rodríguez-Cruz, Edwin; Cáceres-Perkins, William; Santiago-Casiano, Mónica; Rosas, Francisco

    2017-01-01

    Splenic artery aneurysms (SAA) are a rare life threatening clinical diagnosis. They may either be due to congenital defects or acquired conditions such as secondary to atherosclerosis formation. SAA are important to recognize and to consider in acute settings. Patients who present with ruptured or symptomatic SAA, are exposed to a life-threatening condition with hemodynamic collapse and high mortality. We present a case of a 19-year-old woman without history of any systemic illness, who was brought to the urgency room complaining of diffuse abdominal pain of 24 hours of evolution. Abdominopelvic CT-scan revealed the presence of a large left retroperitoneal space hematoma causing severe mass effect upon the adjacent celiac trunk, splenic artery and vein, pancreas, spleen and left kidney. An abdominal arteriogram was performed revealing an aneurysm of the middle branch of the splenic artery with active leakage. The defect was embolized with complete resolution of the retroperitoneal bleeding. We would like to draw attention to the importance of early recognition of SAA rupture in women presenting with hypovolemic shock and abdominal pain. Rupture is the most fatal clinical presentation of a SAA. Patients are exposed to a high mortality rate of 70% to 90%. The favored method of treatment at present of this rare entity is embolization. Future directions must continue to focus on risk factors for rapid growth and further delineation of SAA natural history, which could ultimately identify patients who benefit most from early prophylactic intervention. Physicians should be aware of this rare entity, especially when patients present to the emergency department complaining of severe epigastric pain with associated hypovolemic shock more concerning in the female population. PMID:26035985

  17. Mucinous adenocarcinoma of the intestinal type arising from mature cystic teratoma of the ovary: a rare case report and review of the literature

    PubMed Central

    2012-01-01

    Background Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary. Malignant tranformation occurs in 1-2% of these neoplasms. Although most of the malignancies arising from MCTs are squamous cell carcinomas, adenocarcinoma of the gastrointestinal type is extremery rare. We herein present a case of adenocarcinoma of the intestinal type arising from a MCT. Case A 49-year-old female underwent surgery for a left ovarian tumor. The histology of the cyst walls revealed a MCT with a few hair shafts and a squamous layer, while another part of the tumor showed adenocarcinoma of the intestinal type. Five years after surgery, she is alive without disease. PMID:23216975

  18. Oligodendroglial cell proliferation arising in an ovarian mature cystic teratoma. Clinicopathological, inmunohistochemical, and ultrastructural study of a case that may represent an oligodendroglioma.

    PubMed

    Serrano-Arévalo, Mónica Lizzette; Lino-Silva, Leonardo Saúl; Domínguez Malagón, Hugo Ricardo

    2017-01-01

    Ovarian mature cystic teratoma (OMCT) is an ovarian benign neoplasm with excellent prognosis presenting components of the three germinal layers. However, transformation into a malignant neoplasm is a rare event (so-called somatic transformation). In most of the cases, the malignant component expresses as epidermoid carcinoma, but occasionally central nervous system tumors occur. Some of the previously reported tumors are astrocytoma, glioblastoma, and ependymoma. Somatic transformation of OMCT into an oligodendroglioma is exceptional. We report a 19-year-old female with a left OMCT with an area of oligonedroglial cells proliferation characterized by immunohistochemical studies with positivity for GFAP and S100, with a low Ki67 index (5%). Additionally, electron microscopy revealed oligodendrocytes with parallel bundles of cytoplasmic intermediate filaments, confirming the oligodendroglial nature of the proliferation. The patient was treated only with left oophorectomy, and three and half years after surgery, there is no evidence of disease.

  19. Primary Mucinous Adenocarcinoma of the Urinary Bladder with Signet-Ring Cells: Description of an Uncommon Case and Critical Points in Its Management

    PubMed Central

    Amorim Aita, Giuliano; Amorim Aita, Daniele

    2016-01-01

    We present an uncommon case of mucinous adenocarcinoma of the bladder (MAB) with signet-ring cells extensively infiltrating prostate gland and pelvic/retroperitoneal lymph node stations and not responsive to usual systemic chemotherapy regimens. This case highlights the important features of MAB including the pattern of tumor spread, the tendency for initial misdiagnosis, and the importance of immunohistochemical study in order to define its primary origin from the bladder and choose the most appropriate treatment since the beginning. PMID:28078160

  20. Femoral neuropathy due to retroperitoneal bleeding. A red herring in medicine complicates anticoagulant therapy and influences the Russian Communist Revolution (Crown Prince Alexis, Rasputin).

    PubMed

    Willbanks, O L; Willbanks, S E

    1983-02-01

    Femoral neuropathy occurs when occult retroperitoneal bleeding impinges on the appropriate nerve roots. The syndrome involves the acute onset of groin and thigh pain with characteristic flexion and external rotation of the hip. It may mimic other conditions such as acute arterial occlusion. Thorough knowledge of the anatomy of the femoral nerve explains the clinical features and leads the clinician to suspect the occurrence of this syndrome. Three cases have been reviewed that exhibited this condition as a result of retroperitoneal bleeding, a complication of systemic heparin therapy. The hemophilia that afflicted Alexis, the Crown Prince of Russia and son of Tsar Nicholas and Tsarina Alexandra, resulted in this clinical syndrome. The consequences enabled the sinister starets, Gregory Rasputin, to become intimately involved with the royal family, influencing the response of the Tsar to the political events in Russia, thereby playing an important role in setting the stage for the 1917 Russian communist revolution.

  1. Biliary Duct-to-Duct Reconstruction with a Tunneled Retroperitoneal T-Tube During Liver Transplantation: a Novel Approach to Decrease Biliary Leaks After T-Tube Removal.

    PubMed

    Navez, Julie; Mohkam, Kayvan; Darnis, Benjamin; Cazauran, Jean-Baptiste; Ducerf, Christian; Mabrut, Jean-Yves

    2017-04-01

    The benefit of placing a T-tube for duct-to-duct biliary reconstruction during orthotopic liver transplantation (OLT) remains controversial because it could be associated with specific complications, especially at the time of T-tube removal. While the utility of T-tube during OLT represents an eternal debate, only a few technical refinements of T-tube placement have been described since the report of the original technique by Starzl and colleagues. Herein, we present a novel technique of T-tube placement for duct-to-duct biliary reconstruction during OLT, using a tunneled retroperitoneal route. On the basis of our experience of 305 patients who benefitted from the reported technique, the placement of a tunneled retroperitoneal biliary T-tube appears to be safe and results in a low rate of biliary complications, especially at the time of T-tube removal.

  2. Novel KAT6B-KANSL1 Fusion Gene Identified by RNA Sequencing in Retroperitoneal Leiomyoma with t(10;17)(q22;q21)

    PubMed Central

    Panagopoulos, Ioannis; Gorunova, Ludmila; Bjerkehagen, Bodil; Heim, Sverre

    2015-01-01

    Retroperitoneal leiomyoma is a rare type of benign smooth muscle tumor almost exclusively found in women and with histopathological features similar to uterine leiomyomas. The pathogenesis of retroperitoneal leiomyoma is unclear and next to nothing is known about the cytogenetics and molecular genetics of the tumor. Here we present the first cytogenetically analyzed retroperitoneal leiomyoma. It had a t(10;17)(q22;q21) as the sole chromosomal abnormality. Using RNA-Sequencing and the ‘grep’ command to search the fastq files of the sequence data we found that the translocation resulted in fusion of the genes KAT6B (10q22) with KANSL1 (17q21). RT-PCR together with direct (Sanger) sequencing verified the presence of a KAT6B-KANSL1 fusion transcript. No reciprocal KANSL1-KAT6B transcript was amplified suggesting that it was either absent or unexpressed. The KAT6B-KANSL1 fusion transcript consists of exons 1 to 3 of KAT6B and exons 11 to 15 of KANSL1, is 3667 bp long, has a 1398 bp long open reading frame, and codes for a 466 amino acid residue protein. The corresponding KAT6B-KANSL1 protein contains the NEMM domain (including the linker histone H1/H5, domain H15) of KAT6B and the PEHE domain of KANSL1. The function of the fusion protein might be regulation of transcription with an affinity for chromatin (linker histone H1/H5) and interaction with the HAT domain of KAT8 (PEHE domain). The tumor expressed HMGA2 and HMGA1 even though 12q14-15 and 6p looked normal by G-banding analysis. The tumor also expressed MED12 in the absence of exon 2 mutations. Overall, the data show that the examined retroperitoneal leiomyoma resembles a subset of uterine leiomyomas in terms of histology and genetics. PMID:25621995

  3. Diagnostic Yield of Computed Tomography-Guided Coaxial Core Biopsy of Undetermined Masses in the Free Retroperitoneal Space: Single-Center Experience

    SciTech Connect

    Stattaus, Joerg Kalkmann, Janine Kuehl, Hilmar; Metz, Klaus A.; Nowrousian, Mohammad R.; Forsting, Michael Ladd, Susanne C.

    2008-09-15

    The purpose of this study was to evaluate the diagnostic yield of core biopsy in coaxial technique under guidance of computed tomography (CT) for retroperitoneal masses. We performed a retrospective analysis of CT-guided coaxial core biopsies of undetermined masses in the non-organ-bound retroperitoneal space in 49 patients. In 37 cases a 15-G guidance needle with a 16-G semiautomated core biopsy system, and in 12 cases a 16-G guidance needle with an 18-G biopsy system, was used. All biopsies were technically successful. A small hematoma was seen in one case, but no relevant complication occurred. With the coaxial technique, up to 4 specimens were obtained from each lesion (mean, 2.8). Diagnostic accuracy in differentiation between malignant and benign diseases was 95.9%. A specific histological diagnosis could be established in 39 of 42 malignant lesions (92.9%). Correct subtyping of malignant lymphoma according to the WHO classification was possible in 87.0%. Benign lesions were correctly identified in seven cases, although a specific diagnosis could only be made in conjunction with clinical and radiological information. In conclusion, CT-guided coaxial core biopsy provides safe and accurate diagnosis of retroperitoneal masses. A specific histological diagnosis, which is essential for choosing the appropriate therapy, could be established in most cases of malignancy.

  4. Case of H syndrome with massive skin involvement, retroperitoneal fibrosis and Raynaud's phenomenon with a novel mutation in the SLC29A3 gene.

    PubMed

    Fujita, Etsuko; Komine, Mayumi; Tsuda, Hidetoshi; Adachi, Akimasa; Murata, Satoru; Kamata, Yasuyuki; Minota, Seiji; Ohtsuki, Mamitaro

    2015-12-01

    We describe a case of H syndrome with massive skin involvement, retroperitoneal fibrosis and Raynaud's phenomenon. A 48-year-old man with parents of a consanguineous marriage, first appeared with decreased urine output, skin sclerosis on his inner thighs and short stature (142 cm, 47 kg). The patient had suffered from hearing loss since the age of 1 year, and his secondary sexual characteristics had not developed. Computed tomography showed periaortic fibrosis, bilateral ureteral stenosis, hydronephrosis and sclerosis of the germinal cords. A biopsy from the retroperitoneal mass revealed remarkable fibrosis with chronic inflammatory cells. Biopsies from the skin lesion showed thick collagen bundles through the dermis and lymphohistiocytic infiltration with numerous plasma cells. Serum inflammatory markers, such as C-reactive protein, vascular endothelial factor, transforming growth factor-β and soluble interleukin-2 receptor, were elevated. Prednisolone was effective in treating skin lesions and in lowering serum inflammatory markers. After a long period of follow up, genomic DNA of the patient was obtained, and we identified a homozygous mutation in exon 5, c.625G>A, which caused transition of glycine to arginine, p.Gly208Arg, in the patient, but not in DNA samples from another 50 healthy individuals. This is the first case of H syndrome with Raynaud's phenomenon and retroperitoneal fibrosis, and the first Japanese case of H syndrome reported in the English published work with a novel mutation in the SLC29A3 gene.

  5. [A case of possible immunoglobulin G4-related disease (IgG4-RD) with retroperitoneal fibrosis and central diabetes insipidus due to infundibulohypophysitis].

    PubMed

    Tanaka, Jun; Arai, Atsushi; Hayashi, Shigeto; Sakagami, Yoshio; Araki, Kota; Kakiuchi, Seiji; Nomura, Tetsuhiko; Kuwamura, Keiichi; Kohmura, Eiji

    2014-06-01

    We report a case of possible immunoglobulin G4-related disease(IgG4-RD)that resulted in complications such as retroperitoneal fibrosis and infundibulohypophysitis. The patient was a 72-year-old male who presented with polyuria and polydipsia. Magnetic resonance imaging(MRI)revealed a thickened pituitary stalk and contrast enhancement with gadolinium. T1-weighted imaging revealed that the posterior pituitary high-signal zone had disappeared. Central diabetes insipidus was diagnosed on the basis of results of the hypertonic saline test. In addition, pressure due to retroperitoneal fibrosis resulted in hydronephrosis and elevated serum IgG4 levels. Because it was determined that the patient could have IgG4-RD, he was administered prednisolone, following which a decrease in the size of the pituitary stalk and retroperitoneal fibrosis was observed. IgG4-RD is characterized by elevated serum IgG4 levels and the infiltration of IgG4-positive plasma cells into various organs, including the central nervous system. Recently, IgG4-RD research teams organized by the Ministry of Health, Labour and Welfare established guidelines for the diagnosis of IgG4-RD. According to these guidelines, this case would fall under the category of "possible IgG4-RD." This case suggested that when infundibulohypophysitis is detected by neuroradiology, further investigation into the possibility of IgG4-RD should be recommended.

  6. [Dosimetry verification of radioactive seed implantation with 3D printing template and CT guidance for paravertebral/retroperitoneal malignant tumor].

    PubMed

    Ji, Z; Jiang, Y L; Guo, F X; Peng, R; Sun, H T; Fan, J H; Wang, J J

    2017-04-04

    Objective: To compare the dose distributions of postoperative plans with preoperative plans for seeds implantations of paravertebral/retroperitoneal tumors assisted by 3D printing guide template and CT guidance, explore the effects of the technology for seeds implantations in dosimetry level and provide data support for the optimization and standardization in seeds implantation. Methods: Between December 2015 and July 2016, a total of 10 patients with paravertebral/retroperitoneal tumors (12 lesions) received 3D printing template assist radioactive seeds implantations in department of radiation oncology of Peking University Third Hospital, and included in the study. The diseases included cervical cancer, kidney cancer, abdominal stromal tumor, leiomyosarcoma of kidney, esophageal cancer and carcinoma of ureter. The prescribed doses was 110-150 Gy. All patients received preoperative planning design, individual template design and production, and the dose distribution of postoperative plan was compared with preoperative plan. Dose parameters including D(90), MPD, V(100), V(150,)conformal index(CI), EI of target volume and D(2cc) of organs at risk (spinal cord, aorta, kidney). Statistical software was SPSS 19.0 and statistical method was non-parameters Wilcoxon symbols test. Results: A total of 10 3D printing templates were designed and produced which were including 12 treatment areas.The mean D(90) of postoperative target area (GTV) was 131.1 (97.8-167.4 Gy) Gy. The actual seeds number of post operation increased by 3 to 12 in 5 cases (42.0%). The needle was well distributed. For postoperative plans, the mean D(90,)MPD, V(100,)V(150) was 131.1 Gy, 69.3 Gy, 90.2% and 65.2%, respectively, and which was 140.2 Gy, 65.6 Gy, 91.7% and 26.8%, respectively, in preoperative plans. This meant that the actual dose of target volume was slightly lower than preplanned dose, and the high dose area of target volume was larger than preplanned range, but there was no statistical

  7. Amenorrhea - primary

    MedlinePlus

    ... of periods - primary Images Primary amenorrhea Normal uterine anatomy (cut section) Absence of menstruation (amenorrhea) References Bulun SE. The physiology and pathology of the female reproductive axis. In: ...

  8. Novel Technique for Retroperitoneal Implantation of Telemetry Transmitters for Physiologic Monitoring in Göttingen Minipigs (Sus scrofa domesticus)

    PubMed Central

    Willens, Scott; Cox, David M; Braue, Ernest H; Myers, Todd M; Wegner, Matthew D

    2014-01-01

    Telemetric monitoring of physiologic parameters in animal models is a critical component of chemical and biologic agent studies. The long-term collection of neurobehavioral and other physiologic data can require larger telemetry devices. Furthermore, such devices must be implanted in a location that is safe, well-tolerated, and functional. Göttingen minipigs (Sus scrofa domesticus) present an ideal large animal model for chemical agent studies due to their relatively small size, characterized health status, and ease of training and handling. We report an effective approach to implanting a novel device to measure transthoracic impedance to approximate respiratory tidal volume and rate in Suidae. We tested the approach using 24 male Göttingen minipigs. A ventral midline abdominal incision extending from the umbilicus to the prepuce was followed by a paramedian incision of the parietal peritoneum and dorsal blunt dissection to create a retroperitoneal pocket. The device was anchored inside the pocket to the internal abdominal musculature with 3-0 nonabsorbable suture, biopotential leads were routed through the abdominal musculature, and the pocket was closed with 3-0 absorbable suture. Paired biopotential leads were anchored intermuscularly at the level of the seventh rib midway between spine and sternum bilaterally to provide surrogate data for respiratory function. Postoperative recovery and gross pathology findings at necropsy were used to assess safety and refine the surgical procedure. Results demonstrated that this procedure permitted effective monitoring of complex physiologic data, including transthoracic impedance, without negatively affecting the health and behavior of the animals. PMID:25527027

  9. Thyroid-like follicular carcinoma of the kidney with metastases to the lungs and retroperitoneal lymph nodes

    PubMed Central

    Dhillon, Jasreman; Tannir, Nizar M.; Matin, Surena F.; Tamboli, Pheroze; Czerniak, Bogdan A.; Guo, Charles C.

    2014-01-01

    Summary Thyroid-like follicular carcinoma of the kidney is an extremely rare variant of renal cell carcinoma. Most previously reported cases presented as incidental small tumors confined to the kidney. Here we report a unique case in which the patient presented with flank pain and hematuria. Imaging studies demonstrated a large tumor in the right kidney and metastases to the lungs and retroperitoneal lymph nodes. Both the renal tumor and the sampled lung metastasis were composed almost entirely of follicles with dense, colloid-like material resembling thyroid follicular carcinoma. However, no lesion was found in the thyroid gland, and the patient’s thyroid function tests were normal. The tumor cells were immunoreactive for PAX2 and PAX8 but lacked reactivity for thyroglobulin and thyroid transcription factor-1. To our knowledge, this is the first case of thyroid-like follicular carcinoma of the kidney to be initially associated with marked symptoms and widespread metastases, providing evidence that this rare variant of renal cell carcinoma can be clinically aggressive. PMID:20971497

  10. Systematic Review of Surgical Approaches for Adrenal Tumors: Lateral Transperitoneal versus Posterior Retroperitoneal and Laparoscopic versus Robotic Adrenalectomy

    PubMed Central

    Chai, Young Jun; Kwon, Hyungju; Yu, Hyeong Won; Kim, Su-jin; Choi, June Young; Lee, Kyu Eun; Youn, Yeo-Kyu

    2014-01-01

    Background. Laparoscopic lateral transperitoneal adrenalectomy (LTA) has been the standard method for resecting benign adrenal gland tumors. Recently, however, laparoscopic posterior retroperitoneal adrenalectomy (PRA) has been more popular as an alternative method. This systematic review evaluates current evidence on adrenalectomy techniques, comparing laparoscopic LTA with PRA and laparoscopic adrenalectomy with robotic adrenalectomy. Methods. PubMed, Embase, and ISI Web of Knowledge databases were searched systematically for studies comparing surgical outcomes of laparoscopic LTA versus PRA and laparoscopic versus robotic adrenalectomy. The studies were evaluated according to the PRISMA statement. Results. Eight studies comparing laparoscopic PRA and LTA showed that laparoscopic PRA was superior or at least comparable to laparoscopic LTA in operation time, blood loss, pain score, hospital stay, and return to normal activity. Conversion rates and complication rates were similar. Six studies comparing robotic and laparoscopic adrenalectomy found that outcomes and complications were similar. Conclusion. Laparoscopic PRA was more effective than LTA, especially in reducing operation time and hospital stay, but there was no evidence showing that robotic adrenalectomy was superior to laparoscopic adrenalectomy. Cost reductions and further technical advances are needed for wider application of robotic adrenalectomy. PMID:25587275

  11. Successful treatment by selective arterial embolization of severe retroperitoneal hemorrhage secondary to bone marrow biopsy in post-polycythemic myelofibrosis.

    PubMed

    Arellano-Rodrigo, E; Real, M I; Muntañola, A; Burrel, M; Rozman, M; Fraire, G V; Cervantes, F

    2004-01-01

    Severe retroperitoneal hemorrhage represents an infrequent and serious complication of bone marrow biopsy. A 53-year-old man, diagnosed with polycythemia vera 12 years earlier, was submitted to a bone marrow biopsy due to the appearance of anemia with clinical and hematological features suggesting myelofibrotic transformation, a diagnosis that was confirmed by the marrow study. At 2 h of a right anterior iliac bone marrow trephine biopsy, the patient suddenly developed severe pain in the area of the biopsy, with antialgic flexion of the right leg. Computed tomographic (CT) scan of the abdomen showed a 5 x 9.5 cm hematoma in the right iliac and psoas muscles. The patient was initially managed with analgesics and transfusional support, but the pain persisted and a continuous fall in the hematocrit was observed in the following days. Angiographic examination of the right external iliac artery showed contrast extravasation arising from the circumflex iliac branch, which was embolized using polivinyl alcohol particles and one coil. Following such procedure, the patient recovered uneventfully and was discharged in good condition a few days later. This case illustrates the effectiveness of an endovascular approach in providing a fast and minimally invasive treatment for this life-threatening complication of bone marrow trephine biopsy.

  12. Merkel cell carcinoma of the lower leg with retroperitoneal GIST: a very rare association.

    PubMed

    Wollina, Uwe; Hansel, Gesina; Zimmermann, Felicitas; Schönlebe, Jacqueline; Nowak, Andreas

    2015-05-01

    Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. Although its association with other malignancies is well known, an association with gastrointestinal stromal tumor (GIST) has yet not been described.We report about a 65-year-old female patient who presented with a hypervascularized subcutaneous tumor mass of her left calf. Resection of the primary tumor and histopathological investigations confirmed the diagnosis of MCC. The patient was treated by delayed Mohs surgery, and tumor-free margins were obtained. Sentinel lymph node biopsy was negative for metastatic spread. Primary tumor and lymph node basin were treated by adjuvant radiotherapy. During staging of the patient, a second malignancy-a GIST-was detected. Neoadjuvant treatment with multikinase inhibitor imatinib induced a partial response of GIST that was eventually removed by surgery. However, 8 months later, the patient developed subcutaneous regional metastases of MCC, which were surgically removed. Adjuvant therapy was planned by oncologists.To the best of our knowledge, the occurrence of MCC and GIST in the same patient has yet not been reported. In contrast to GIST, MCC did not respond to imatinib, although c-kit mutations are common in MCC.

  13. Diffuse large B-cell lymphoma occurring in an ovarian cystic teratoma: expanding the spectrum of large B-cell lymphoma associated with chronic inflammation.

    PubMed

    Valli, Riccardo; Froio, Elisabetta; Alvarez de Celis, Maria Isabel; Mandato, Vincenzo Dario; Piana, Simonetta

    2014-12-01

    Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) is a well-recognized entity, originally recorded as pyothorax-associated lymphoma because of the association with artificial pneumothorax. Clinically, it is characterized by a mass arising in a long-standing inflammation and by a poor prognosis. Recently, DLBCL-CI has been described growing along the wall of a preexisting cyst, without forming a mass. Here we describe a case of DLBCL-CI arising in the wall of a mature cystic teratoma of the ovary. On histology, the cystic surface of the cyst was infiltrated by large lymphocytes, immunoreacting with CD20, Multiple Myeloma Oncogene-1/Interferon Regulating Factor-4 (MUM1/IRF4), and PAX5 and positive for Epstein-Barr virus. "Cystic" DLBCL-CIs usually hold an indolent behavior despite heterogeneous therapeutic approaches. Some authors understandably wonder whether patients affected by "cystic" DLBCL-CIs are at risk for overtreatment, and, consequently, DLBCL-CIs associated with cystic lesions should be classified as an entity separated from classic pyothorax-associated lymphomas.

  14. Risk factor analysis for massive lymphatic ascites after laparoscopic retroperitonal lymphadenectomy in gynecologic cancers and treatment using intranodal lymphangiography with glue embolization

    PubMed Central

    2016-01-01

    Objective To evaluate risk factors for massive lymphatic ascites after laparoscopic retroperitoneal lymphadenectomy in gynecologic cancer and the feasibility of treatments using intranodal lymphangiography (INLAG) with glue embolization. Methods A retrospective analysis of 234 patients with gynecologic cancer who received laparoscopic retroperitonal lymphadenectomy between April 2006 and November 2015 was done. In June 2014, INLAG with glue embolization was initiated to manage massive lymphatic ascites. All possible clinicopathologic factors related to massive lymphatic ascites were determined in the pre-INLAG group (n=163). Clinical courses between pre-INLAG group and post-INLAG group (n=71) were compared. Results In the pre-INLAG group (n=163), four patients (2.5%) developed massive lymphatic ascites postoperatively. Postoperative lymphatic ascites was associated with liver cirrhosis (three cirrhotic patients, p<0.001). In the post-INLAG group, one patient with massive lymphatic ascites had a congestive heart failure and first received INLAG with glue embolization. She had pelvic drain removed within 7 days after INLAG. The mean duration of pelvic drain and hospital stay decreased after the introduction of INLAG (13.2 days vs. 10.9 days, p=0.001; 15.2 days vs. 12.6 days, p=0.001). There was no evidence of recurrence after this procedure. Conclusion Underlying medical conditions related to the reduced effective circulating volume, such as liver cirrhosis and heart failure, may be associated with massive lymphatic ascites after retroperitoneal lymphadenectomy. INLAG with glue embolization can be an alternative treatment options to treat leaking lymphatic channels in patients with massive lymphatic leakage. PMID:27171674

  15. CT-guided 125I seed implantation for inoperable retroperitoneal sarcoma: A technique for delivery of local tumor brachytherapy

    PubMed Central

    Yang, Biao; Guo, Wen-Hao; Lan, Ting; Yuan, Fang; Liu, Guan-Jian; Zan, Rui-Yu; You, Xin; Tan, Qiao-Yue; Liao, Zheng-Yin

    2016-01-01

    Radical surgery is currently the first treatment of choice for retroperitoneal soft tissue sarcoma (RSTS). However, the prognosis of RSTS remains poor due to ineffective local control and a high incidence of metastasis after surgical resection. Brachytherapy has been shown to safely provide local radiotherapy for numerous types of cancer when used alone or in combination with surgical resection, but has not been well characterized in the management of RSTS. The aim of this study was to evaluate CT-guided 125I seed implantation for local control and pain relief in the treatment of inoperable RSTS. A total of 23 patients with RSTS were treated with 125I implantation. Pain was assessed using a visual analog scale. Other endpoints were evaluated via computed tomography scan or phone call/e-mail records. The occurrence of complications was assessed preoperatively (baseline) and during postoperatively follow-up or until patient succumbed. All patients were successfully treated with 125I implantation. A mean number of 70.87 radioactive seeds were applied in each patient. During the follow-up, two patients were unaccounted for, local recurrence occurred in three patients, five succumbed and complications were observed in sixteen. The patient's VAS score changed from 7.4 preoperatively to 7.6, 2.3, 2.0, 1.2, 1.5, 1.4 and 2.5 at 24 h, 1, 3, 6, 12, 24 and 36 months after the procedure, respectively. Good local control and significant pain relief after 125I seed implantation was observed in patients with inoperable RSTS. Thus, the present results suggest that this method could be an effective treatment option for patients with inoperable RSTS. PMID:28101168

  16. Percutaneous computed tomography-guided cryoablation for recurrent retroperitoneal soft tissue sarcoma: a study of safety and efficacy

    PubMed Central

    Fan, Wenzhe; Niu, Lizhi; Wang, Yu; Zhang, Yingqiang; Yao, Xuehua; Tan, Guosheng; Yang, Jianyong; Li, Jiaping

    2016-01-01

    Aims To evaluate the use of computed tomography image-guided percutaneous cryoablation for recurrent retroperitoneal soft tissue sarcomas (RPSs). Results Adverse events were limited to grades 1 and 2, included fever (n = 19), local pain (n = 11), emesis (n = 10), frostbite (n = 6), and nerve injury (n = 1). Fever was more frequent in the large tumor group (15.8%) than in small tumor group (1.9%) (P = 0.008). Median PFS and OS were 37.0 ± 7.7 months (range, 4–39 months) and 43.0 ± 5.9 months (range, 6–54 months), respectively. PFS and OS were significantly longer in the small tumor group than in the large tumor group (P = 0.011 and P = 0.015, respectively), but the response rate (82.7% vs. 72.8%, P = 0.240) did not differ significantly. On univariate analysis, tumor size, tumor invasion grade, and distant metastasis were significant prognostic factors for PFS and OS. On multivariate analysis, a tumor size ≥10 cm was an independent negative prognostic factor for PFS and OS after cryoablation (HR: 3.98, 95% CI: 1.27–12.50, P = 0.018 and HR: 4.33, 95% CI: 1.41–13.26, P = 0.010, respectively). Materials and Methods Data from 72 patients with recurrent RPSs who underwent percutaneous cryoablation were reviewed retrospectively. The prognostic factors for progression-free survival (PFS), overall survival (OS), and efficacy based on mRECIST criteria were analysis. Adverse events were compared according to tumor size (<10 and ≥10 cm). Conclusion Minimally invasive percutaneous cryoablation was safe and efficacious for recurrent RPSs. PMID:27223071

  17. Primary Hyperparathyroidism

    MedlinePlus

    ... What is PRIMARY HYPERPARATH YROIDIS M? The body’s parathyroid glands—four pea-sized glands in the neck—produce parathyroid hormone (PTH). Primary hyperparathyroidism (PHPT) is a condition ...

  18. Primary thrombocythemia

    MedlinePlus

    ... as myeloproliferative disorders. Others include: Chronic myelogenous leukemia Polycythemia vera Primary myelofibrosis This disorder is most common ... PA: Elsevier Saunders; 2013:chap 68. Tefferi A. Polycythemia vera, essential thrombocytoemia, and primary myelofibrosis. In: Goldman ...

  19. Primary Aldosteronism

    MedlinePlus

    ... Endocrinology Find an Endocrinologist Value of an Endocrinologist Learn About Clinical Trials Keep Your Body in Balance › Primary Aldosteronism Fact Sheet Primary Aldosteronism March 2012 Download PDFs English Espanol Editors Paul Stewart, MD, FRCP William Young, ...

  20. Reversal of Anticoagulation With Dabigatran in an 82-Year-Old Patient With Traumatic Retroperitoneal Arterial Bleeding Using the New Antidote Idarucizumab: A Case Report.

    PubMed

    Hofer, Stefan; Philipsenburg, Christoph; Weigand, Markus A; Brenner, Thorsten

    2016-12-01

    Dabigatran etexilate is a direct oral anticoagulant used for the prevention of stroke in atrial fibrillation. Idarucizumab is a recently approved specific antidote that reverses the effect of dabigatran within minutes. We report the case of an 82-year-old patient with traumatic retroperitoneal arterial bleeding under anticoagulation with dabigatran etexilate. By administration of idarucizumab, we successfully normalized coagulation and saved the patient from an operation. In the course of the disease, a slight reincrease in dabigatran etexilate plasma levels was observed 2 days after the reversal, which could lead to a new onset of bleeding.

  1. Laparoscopic Retroperitoneal Nephron-Sparing Surgery Without Renal Artery Clamping with Preoperative Selective Arterial Embolization for Management of Right Renal Angiomyolipoma of Diameter 10 cm: A Case Report

    PubMed Central

    Hoshii, Tatsuhiko; Morita, Shinichi; Ikeda, Yohei; Hasegawa, Go

    2017-01-01

    Abstract A 38-year-old female without the tuberous sclerosis complex was diagnosed with right renal angiomyolipoma of 10 cm in diameter. She underwent laparoscopic retroperitoneal nephron-sparing surgery without renal artery clamping with preoperative selective arterial embolization to avoid a significant risk of hemorrhage and the damage of the renal function during nephron-sparing surgery. The tumor was resected completely. The time taken to complete the procedure was 4 hours 11 minutes and blood loss was 780 mL. She was transfused 400 mL of autologous blood. PMID:28265590

  2. Impact of geometric mean imaging in the accurate determination of partial function in MAG3 renal scanning in a patient with retroperitoneal mass.

    PubMed

    Takesh, Mustafa; Zechmann, Christian M; Haufe, Sabine; Giesel, Frederik L; Kratochwil, Clemens

    2011-01-01

    Liposarcoma frequently occurs in the retroperitoneum and lower extremities, accounting for 20% of all mesenchymal malignancies. Liposarcomas vary by histology and can be classified into four types. Those four types are well differentiated, myxoid/round cell, pleomorphic and dedifferentiated. Due to retroperitoneal location of this tumor, it is expected to affect the kidney position. Renography has provided a unique tool for noninvasive evaluation of various functional parameters e.g. relative renal function. Most renography studies are carried out using the posterior view, under the assumption that the depths of both kidneys are similar so that the radiotracer counts in the region of interest will be attenuated to the same extent. Errors in estimation of the relative renal function may arise if the kidneys are at different depths e.g. secondary to a pushing tumor. Geometric mean imaging from combined anterior and posterior views helps to overcome this issue. This case shows the impact of geometric mean imaging in the truthful determination of partial function in patients with retroperitoneal liposarcoma.

  3. RFHVMn ORF73 is structurally related to the KSHV ORF73 latency-associated nuclear antigen (LANA) and is expressed in retroperitoneal fibromatosis (RF) tumor cells

    SciTech Connect

    Burnside, Kellie L.; Ryan, Jonathan T.; Bielefeldt-Ohmann, Helle; Gregory Bruce, A.; Thouless, Margaret E.; Tsai, Che-Chung; Rose, Timothy M. . E-mail: trose@u.washington.edu

    2006-10-10

    Retroperitoneal fibromatosis herpesvirus (RFHV), the macaque homolog of the human rhadinovirus, Kaposi's sarcoma-associated herpesvirus (KSHV), was first identified in retroperitoneal fibromatosis (RF) tumor lesions of macaques with simian AIDS. We cloned and sequenced the ORF73 latency-associated nuclear antigen (LANA) of RFHVMn from the pig-tailed macaque. RFHVMn LANA is structurally analogous to KSHV ORF73 LANA and contains an N-terminal serine-proline-rich region, a large internal glutamic acidic-rich repeat region and a conserved C-terminal domain. RFHVMn LANA reacts with monoclonal antibodies specific for a glutamic acid-proline dipeptide motif and a glutamic acid-glutamine-rich motif in the KSHV LANA repeat region. Immunohistochemical and immunofluorescence analysis revealed that RFHVMn LANA is a nuclear antigen which is highly expressed in RF spindloid tumor cells. These data suggest that RFHV LANA is an ortholog of KSHV LANA and will function similarly to maintain viral latency and play a role in tumorigenicity in macaques.

  4. [A case of possible retroperitoneal metastasis of breast cancer successfully treated with oral S-1 and cyclophosphamide therapy after TC therapy].

    PubMed

    Yoneyama, Kimiyasu; Takeshita, Toshio; Suzuki, Hiroshi; Morise, Masaki; Suzuki, Tetsutarou; Kishi, Shinya; Tsutsui, Atsuko; Matsumoto, Akiko

    2011-03-01

    We report a case of possible retroperitoneal metastasis of breast cancer successfully treated with oral S-1 and cyclophosphamide therapy after docetaxel and cyclophosphamide (TC) therapy. A 57-year-old woman with a history of bilateral breast cancer showed an increase in tumor markers during treatment with oral anastrozole as postoperative adjuvant therapy 4 years after her second cancer surgery. After careful examination, the patient was diagnosed as having multiple bone metastases and her medication was changed to oral letrozole. After 3 months, the patient developed left back pain and was referred to our hospital. CT scanning showed an enhanced mass in the region from the left perirenal and posterior pararenal spaces to the left psoas major muscle and the anterior aspect of the left iliacus muscle, suggesting retroperitoneal metastasis. TC therapy was performed and, as a result, tumor markers decreased and the mass disappeared on CT imaging. After discontinuation of TC therapy, the tumor markers increased again, following which oral S-1 and cyclophosphamide therapy were administered, and the tumor markers decreased. At the time of this writing, the patient is still undergoing therapy, and no recurrence has been observed. We concluded that oral S-1 and cyclophosphamide therapy were useful in the present case and were associated with few adverse effects.

  5. Thymic hyperplasia associated with primary Sjogren’s syndrome cured by thymectomy

    PubMed Central

    Xin, Yanzhong; Cai, Hongfei; Li, Yang

    2017-01-01

    Thymus hyperplasia associated with Sjogren’s syndrome is a rare morbid state. The present study described a 55-year-old woman who presented with a dryness of the oral cavity, and itchy eyes. Chest computed tomography identified a mass, measuring 4×2.5×2.5 cm, located at the anterior mediastinum. The mass was suspected as thymoma, thymic cyst, or teratoma, and resected by thymectomy. The postoperative pathological diagnosis was thymic lymphoid hyperplasia. After 1-year follow-up period, her sicca syndrome has been resolved. The present study records a successful case for thymectomy to treat the patients with thymic hyperplasia associated with primary Sjogren’s syndrome (pSS). PMID:28275496

  6. Primary Hyperparathyroidism

    MedlinePlus

    ... D blood test. This test is recommended because vitamin D deficiency is common in people with primary hyperparathyroidism. How ... bone density measurements every 1 to 2 years. Vitamin D deficiency should be corrected if present. Patients who are ...

  7. 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.

  8. [Retroperitoneal Tumor: Neurofibroma.

    PubMed

    Lada, Paul Eduardo; Marriot, Daniela; Sanchez Tasonne, Carlos; Sanchez, Martin; Caballero, Fabian; Massa, Martin

    2016-01-01

    The neurofibroma is a benign tumor that grows from the sheath of the peripheral nerves, which is often localized on superficial tissues, especially in isolated forms. The neurofibromas can be of two types, localized or diffuse, the last one closely related to Von Recklinghausen disease or NF-1. We described a 37 years old male e patient, 37 years without symptoms, and the computed tomography scan (CT), and magnetic resonance imaging showed a tumor in proximity to the right kidney, the psoas muscle, the spine and compressing the cava vein, but cannot be accurately determinate the invasion of these structures. We review the differential diagnosis, therapeutic and the management of this disease in this clinical case.

  9. Retroperitoneal hematoma after using the extreme lateral interbody fusion (XLIF) approach: Presentation of a case and a review of the literature.

    PubMed

    Peiró-García, A; Domínguez-Esteban, I; Alía-Benítez, J

    2016-01-01

    The transpsoas approach, also known as extreme lateral interbody fusion (XLIF), to the lumbar spine is a novel minimally invasive technique with positive clinical outcomes and a low complication rate. There is a low risk of bleeding, due to this approach causing less soft tissue disruption than traditional spine surgery, but segmental arteries and great vessels can be damaged. Retroperitoneal haematoma is a major complication, with few cases reported. This is the first case reported in a Stand-alone XLIF and also the first case reported with haemorrhagic shock. Non-specific symptoms such tachycardia, hypotension, and anaemia are the most prevalent in this complication. With this case, our aim is to describe serious complications related to XLIF.

  10. [Indications and results of preventive endoprosthezing of the abdominal wall during operations on organs of the abdominal cavity and retroperitoneal space].

    PubMed

    Sukovatykh, B S; Valuĭskaia, N M; Netiaga, A A; Zhukovskiĭ, V A; Pravednikova, N V; Kas'ianova, M A

    2011-01-01

    Complex examination and treatment of 120 patients with anatomo-functional insufficiency of the abdominal wall was made after operations on organs of the abdominal cavity and retroperitoneal space. In the 1st group (60 patients) the abdominal wall was sutured by traditional methods. In the 2nd group (60 patients) laparotomy was followed by implantation of polypropylene endoprosthesis by over-aponeurotic method, and suturing of the lateral wall--by sub-aponeurotic method. At the long-term postoperative period the postoperative ventral hernias were formed in 21.6% of patients of the 1st group, in the 2nd group of patients hernias were not detected. Physical component of quality of life of the 2nd group was 1.5 times, and mental component 1.7 times higher than in the 1st group.

  11. Severe retroperitoneal and intra-abdominal bleeding after stapling procedure for prolapsed haemorrhoids (PPH); diagnosis, treatment and 6-year follow-up of the case.

    PubMed

    Safadi, Wajdi; Altshuler, Alexander; Kiv, Sakal; Waksman, Igor

    2014-10-30

    Procedure for prolapsed haemorrhoids (PPH) is a popular treatment of haemorrhoids. PPH has the advantages of a shorter operation time, minor degree of postoperative pain, shorter hospital stay and quicker recovery but may be followed by several postoperative complications. Rectal bleeding, acute pain, chronic pain, rectovaginal fistula, complete rectal obliteration, rectal stenosis, rectal pocket, tenesmus, faecal urgency, faecal incontinence, rectal perforation, pelvic sepsis and rectal haematoma have all been reported as postoperative complications of PPH. Additionally, one rare complication of the procedure is intra-abdominal bleeding. There are a few case reports describing intra-abdominal bleeding after the procedure. We report a case of a 26-year-old man who developed severe intra-abdominal and retroperitoneal haemorrhage after PPH. The diagnosis was made on the second postoperative day by CT of the abdomen and pelvis. The patient was treated conservatively and had an uneventful recovery.

  12. Primary hyperparathyroidism

    PubMed Central

    Madkhali, Tarıq; Alhefdhi, Amal; Chen, Herbert; Elfenbein, Dawn

    2016-01-01

    Primary hyperparathyroidism is a common endocrine disorder caused by overactivation of parathyroid glands resulting in excessive release of parathyroid hormone. The resultant hypercalcemia leads to a myriad of symptoms. Primary hyperparathyroidism may increase a patient’s morbidity and even mortality if left untreated. During the last few decades, disease presentation has shifted from the classic presentation of severe bone and kidney manifestations to most patients now being diagnosed on routine labs. Although surgery is the only curative therapy, many advances have been made over the past decades in the diagnosis and the surgical management of primary hyperparathyroidism. The aim of this review is to summarize the characteristics of the disease, the work up, and the treatment options. PMID:26985167

  13. [Primary hyperoxaluria].

    PubMed

    Cochat, Pierre; Fargue, Sonia; Bacchetta, Justine; Bertholet-Thomas, Aurélia; Sabot, Jean-François; Harambat, Jérôme

    2011-07-01

    Primary hyperoxalurias are rare recessive inherited inborn errors of glyoxylate metabolism. They are responsible for progressive renal involvement, which further lead to systemic oxalate deposition, which can even occur in infants. Primary hyperoxaluria type 1 is the most common form in Europe and is due to alanine-glyoxylate aminostransferase deficiency, a hepatic peroxisomal pyridoxin-dependent enzyme. Therefore primary hyperoxaluria type 1 is responsible for hyperoxaluria leading to aggressive stone formation and nephrocalcinosis. As glomerular filtration rate decreases, systemic oxalate storage occurs throughout all the body, and mainly in the skeleton. The diagnosis is first based on urine oxalate measurement, then on genotyping, which may also allow prenatal diagnosis to be proposed. Conservative measures - including hydration, crystallization inhibitors and pyridoxine - are safe and may allow long lasting renal survival, provided it is given as soon as the diagnosis has been even suspected. No dialysis procedure can remove enough oxalate to compensate oxalate overproduction from the sick liver, therefore a combined liver and kidney transplantation should be planned before advanced renal disease has occurred, in order to limit/avoid systemic oxalate deposition. In the future, primary hyperoxaluria type 1 may benefit from hepatocyte transplantation, chaperone molecules, etc.

  14. Malignant teratoma of the mediastinum

    MedlinePlus

    ... following tests help diagnose the tumor: Chest x-ray CT scans of the chest, abdomen, and pelvis Blood tests to check beta-HCG, alpha fetoprotein (AFP), and lactate dehydrogenase (LDH) levels Mediastinoscopy ...

  15. Primary hyperparathyroidism.

    PubMed

    Govett, G; White, J

    1989-07-01

    Primary hyperparathyroidism is a pathological entity due to excessive secretion of parathormone from a single or multiple parathyroid glands. The biochemical hallmark of this disorder is an elevated serum calcium. The relationship of the parathyroid glands with the thymus gland in fetal development accounts for the occasional aberrant location of the parathyroids. By utilizing computed tomography or nuclear scanning or both preoperatively, the surgeon can isolate the hyperfunctioning adenoma and resect it, thus minimizing potential complications.

  16. Primary osteosarcoma of the ovary. A case report.

    PubMed

    Sakata, H; Hirahara, T; Ryu, A; Sawada, T; Yamamoto, M; Sakurai, I

    1991-04-01

    A case of primary osteosarcoma arising in the left ovary of a 75-year-old female is described. The chief complaint was a sensation of lower abdominal mass. An abdominal plain film showed a large calcified mass in pelvic region, and a preoperative diagnosis of "ovarian fibroma" was made. The excised tumor was divided into 4 pieces, resembling an oyster shell. Microscopically, the tumor fragments were composed of compact bone or woven bone with surrounding atypical osteoblasts and osteoclasts. The tumor was partly composed of numerous spindle cells with malignant osteoid or atypical chondroid formation, and diagnosed as "osteosarcoma". The cystic part of the lesion was lined with a single layer of columnar cells, but the tumor contained no other germ elements or stem cells, or malignant epithelium. Therefore, it is doubtful that this tumor originated from teratoma or malignant mixed mesodermal tumor, and we conclude that this ovarian osteosarcoma arose through a neoplastic change in ovarian stromal cells. The patient died 4 months after surgery due to intra-abdominal and intrathoracic dissemination of the tumor.

  17. Comparison of Three-Dimensional (3D) Conformal Proton Radiotherapy (RT), 3D Conformal Photon RT, and Intensity-Modulated RT for Retroperitoneal and Intra-Abdominal Sarcomas

    SciTech Connect

    Swanson, Erika L.; Indelicato, Daniel J.; Louis, Debbie; Flampouri, Stella; Li, Zuofeng; Morris, Christopher G.; Paryani, Nitesh; Slopsema, Roelf

    2012-08-01

    Purpose: To compare three-dimensional conformal proton radiotherapy (3DCPT), intensity-modulated photon radiotherapy (IMRT), and 3D conformal photon radiotherapy (3DCRT) to predict the optimal RT technique for retroperitoneal sarcomas. Methods and Materials: 3DCRT, IMRT, and 3DCPT plans were created for treating eight patients with retroperitoneal or intra-abdominal sarcomas. The clinical target volume (CTV) included the gross tumor plus a 2-cm margin, limited by bone and intact fascial planes. For photon plans, the planning target volume (PTV) included a uniform expansion of 5 mm. For the proton plans, the PTV was nonuniform and beam-specific. The prescription dose was 50.4 Gy/Cobalt gray equivalent CGE. Plans were normalized so that >95% of the CTV received 100% of the dose. Results: The CTV was covered adequately by all techniques. The median conformity index was 0.69 for 3DCPT, 0.75 for IMRT, and 0.51 for 3DCRT. The median inhomogeneity coefficient was 0.062 for 3DCPT, 0.066 for IMRT, and 0.073 for 3DCRT. The bowel median volume receiving 15 Gy (V15) was 16.4% for 3DCPT, 52.2% for IMRT, and 66.1% for 3DCRT. The bowel median V45 was 6.3% for 3DCPT, 4.7% for IMRT, and 15.6% for 3DCRT. The median ipsilateral mean kidney dose was 22.5 CGE for 3DCPT, 34.1 Gy for IMRT, and 37.8 Gy for 3DCRT. The median contralateral mean kidney dose was 0 CGE for 3DCPT, 6.4 Gy for IMRT, and 11 Gy for 3DCRT. The median contralateral kidney V5 was 0% for 3DCPT, 49.9% for IMRT, and 99.7% for 3DCRT. Regardless of technique, the median mean liver dose was <30 Gy, and the median cord V50 was 0%. The median integral dose was 126 J for 3DCPT, 400 J for IMRT, and 432 J for 3DCRT. Conclusions: IMRT and 3DCPT result in plans that are more conformal and homogenous than 3DCRT. Based on Quantitative Analysis of Normal Tissue Effects in Clinic benchmarks, the dosimetric advantage of proton therapy may be less gastrointestinal and genitourinary toxicity.

  18. A Rare Type of Primary Internal Hernia Causing Small Intestinal Obstruction

    PubMed Central

    Mohapatra, Vandana; Rath, Pratap Kumar

    2016-01-01

    Primary internal hernias are extremely rare in adults. They are an important cause of small intestinal obstruction and lead to high morbidity and mortality if left untreated. Clinical presentation of internal hernia is nonspecific. Imaging has been of limited utility in cases of acute intestinal obstruction; moreover, interpretation of imaging features is operator dependant. Thus, internal hernias are usually detected at laparotomy and preoperative diagnosis in an emergency setting is either difficult or most of the time not suspected. We report herein a case of a 45-year-old male who presented with acute intestinal obstruction which was attributed later to a very rare type of internal hernia on exploratory laparotomy. A loop of ileum was found to enter the retroperitoneum through a hernia gate which was located lateral to the sigmoid colon in the left paracolic gutter. The segment of intestine was reduced and the hernial defect was closed. Our finding represents an extremely rare variant of retroperitoneal hernias. PMID:27999703

  19. [Primary aldosteronism].

    PubMed

    Amar, Laurence

    2015-06-01

    Primary aldosteronism affects 6% of hypertensive patients. The diagnosis should be suspected in any patient with severe or resistant hypertension or hypertension associated with hypokalemia. The screening test consists on the assessment of the aldosterone to renin ratio. In case of an elevated ratio, the diagnosis of primary aldosteronism is confirmed by either elevated concentrations of basal plasma and/or urinary aldosterone or absence of suppression of aldosterone during dynamic test (including the saline infusion test). CT aims to ensure the absence of adrenal carcinoma and to study the morphology of the adrenals. The unilateral or bilateral type of aldosterone secretion is based on the realization of an adrenal venous sampling. When the hypersecretion is unilateral, the treatment consists of adrenalectomy leading to cure of hypertension in 42% of cases, improvement in 40% of cases. For patient with bilateral disease or who don't want to undergo surgery, treatment is based on spironolactone usually at doses of 25 or 50 mg in combination with other antihypertensives drugs such as diuretics or calcium channel blockers.

  20. Combination of a fillet flap, free tissue transfer, and autologous tissue grafts in pelvic reconstruction following retroperitoneal sarcoma: a case report.

    PubMed

    Saksena, Anshuman; Aho, Johnathon; Winocour, Sebastian; Maricevich, Renata; Senchenkov, Alex; Rose, Peter; Leibovich, Bradley; Zietlow, Scott; Saint-Cyr, Michel

    2015-05-01

    The resection of large pelvic tumors is challenging due to their infiltrative nature into multiple structures and organ systems. In this report, we describe the use of multiple vascularized and nonvascularized spare parts to reconstruct a pelvic defect in a patient with a uniquely large pelvic sarcoma invading the spinal canal. A 39-year-old Caucasian female who presented with a large retroperitoneal sarcoma where the tumor encased the left ureter, kidney, colon, and external iliac vessels and invaded the L3-S1 vertebral bodies. An extensive hemipelvectomy and reconstruction was performed over two days. A free thigh and leg fillet flap together with ipsilateral fibula flap, based on the superficial femoral artery and venae comitantes, was used for spinal reinforcement as well as abdominal and pelvic wall reconstruction. The postoperative course was uneventful without complications, no flap compromise or wound healing problems. After a follow-up period of 4 months, the patient had no complications and returned to activities of daily living with mild limitations. The success of this flap procedure shows the practicality and usefulness of using the full spectrum of tissue transfer for the purposes of a large pelvic reconstruction.

  1. Extensive retroperitoneal and right thigh abscess in a patient with ruptured retrocecal appendicitis: an extremely fulminant form of a common disease.

    PubMed

    Hsieh, Chi-Hsun; Wang, Yu-Chun; Yang, Horng-Ren; Chung, Ping-Kuei; Jeng, Long-Bin; Chen, Ray-Jade

    2006-01-21

    As a disease commonly encountered in daily practice, acute appendicitis is usually diagnosed and managed easily with a low mortality and morbidity rate. However, acute appendicitis may occasionally become extraordinarily complicated and life threatening. A 56-year-old man, healthy prior to this admission, was brought to the hospital due to spiking high fever, poor appetite, dysuria, progressive right flank and painful swelling of the thigh for 3 d. Significant inflammatory change of soft tissue was noted, involving the entire right trunk from the subcostal margin to the knee joint. Painful disability of the right lower extremity and apparent signs of peritonitis at the right lower abdomen were disclosed. Laboratory results revealed leukocytosis and an elevated C-reactive protein level. Abdominal CT revealed several communicated gas-containing abscesses at the right retroperitoneal region with mass effect, pushing the duodenum and the pancreatic head upward, compressing and encasing inferior vena cava, destroying psoas muscle and dissecting downward into the right thigh. Laparotomy and right thigh exploration were performed immediately and about 500 mL of frank pus was drained. A ruptured retrocecal appendix was the cause of the abscess. The patient fully recovered at the end of the third post-operation week. This case reminds us that acute appendicitis should be treated carefully on an emergency basis to avoid serious complications. CT scan is the diagnostic tool of choice, with rapid evaluation followed by adequate drainage as the key to the survival of the patient.

  2. Retroperitoneal Sarcoma (RPS) High Risk Gross Tumor Volume Boost (HR GTV Boost) Contour Delineation Agreement Among NRG Sarcoma Radiation and Surgical Oncologists

    PubMed Central

    Baldini, Elizabeth H.; Bosch, Walter; Kane, John M.; Abrams, Ross A.; Salerno, Kilian E.; Deville, Curtiland; Raut, Chandrajit P.; Petersen, Ivy A.; Chen, Yen-Lin; Mullen, John T.; Millikan, Keith W.; Karakousis, Giorgos; Kendrick, Michael L.; DeLaney, Thomas F.; Wang, Dian

    2015-01-01

    Purpose Curative intent management of retroperitoneal sarcoma (RPS) requires gross total resection. Preoperative radiotherapy (RT) often is used as an adjuvant to surgery, but recurrence rates remain high. To enhance RT efficacy with acceptable tolerance, there is interest in delivering “boost doses” of RT to high-risk areas of gross tumor volume (HR GTV) judged to be at risk for positive resection margins. We sought to evaluate variability in HR GTV boost target volume delineation among collaborating sarcoma radiation and surgical oncologist teams. Methods Radiation planning CT scans for three cases of RPS were distributed to seven paired radiation and surgical oncologist teams at six institutions. Teams contoured HR GTV boost volumes for each case. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results HRGTV boost volume contour agreement between the seven teams was “substantial” or “moderate” for all cases. Agreement was best on the torso wall posteriorly (abutting posterior chest abdominal wall) and medially (abutting ipsilateral para-vertebral space and great vessels). Contours varied more significantly abutting visceral organs due to differing surgical opinions regarding planned partial organ resection. Conclusions Agreement of RPS HRGTV boost volumes between sarcoma radiation and surgical oncologist teams was substantial to moderate. Differences were most striking in regions abutting visceral organs, highlighting the importance of collaboration between the radiation and surgical oncologist for “individualized” target delineation on the basis of areas deemed at risk and planned resection. PMID:26018727

  3. Paternal high-fat diet consumption induces common changes in the transcriptomes of retroperitoneal adipose and pancreatic islet tissues in female rat offspring.

    PubMed

    Ng, Sheau-Fang; Lin, Ruby C Y; Maloney, Christopher A; Youngson, Neil A; Owens, Julie A; Morris, Margaret J

    2014-04-01

    We previously showed that paternal high-fat diet (HFD) consumption programs β-cell dysfunction in female rat offspring, together with transcriptome alterations in islets. Here we investigated the retroperitoneal white adipose tissue (RpWAT) transcriptome using gene and pathway enrichment and pathway analysis to determine whether commonly affected network topologies exist between these two metabolically related tissues. In RpWAT, 5108 genes were differentially expressed due to a paternal HFD; the top 5 significantly enriched networks identified by pathway analysis in offspring of HFD fathers compared with those of fathers fed control diet were: mitochondrial and cellular response to stress, telomerase signaling, cell death and survival, cell cycle, cellular growth and proliferation, and cancer. A total of 187 adipose olfactory receptor genes were down-regulated. Interrogation against the islet transcriptome identified specific gene networks and pathways, including olfactory receptor genes that were similarly affected in both tissues (411 common genes, P<0.05). In particular, we highlight a common molecular network, cell cycle and cancer, with the same hub gene, Myc, suggesting early onset developmental changes that persist, shared responses to programmed systemic factors, or crosstalk between tissues. Thus, paternal HFD consumption triggers unique gene signatures, consistent with premature aging and chronic degenerative disorders, in both RpWAT and pancreatic islets of daughters.

  4. Intramedullary and retroperitoneal melanocytic tumor associated with congenital blue nevus and nevus flammeus: an uncommon combination of neurocutaneous melanosis and phacomatosis pigmentovascularis--case report.

    PubMed

    Kurokawa, Ryu; Kim, Phyo; Kawamoto, Toshiki; Matsuda, Hadzki; Hayashi, Shujiro; Yamazaki, Soji; Hatamochi, Atsushi; Mori, Shozo; Shimoda, Mitsugi; Kubota, Keiichi

    2013-01-01

    Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and PPV has not been reported to the best of our knowledge. Melanocytoma, which usually involves the leptomeninges or spinal cord, is extremely rare in the retroperitoneum. We present here a case of a patient with NCM, PPV, and melanocytic tumors in the spinal cord and retroperitoneum, which were treated surgically. A 40-year-old woman had a 2-year history of dysesthesia and weakness in the left leg. History included congenital giant blue nevus-like lesion in the trunk, a port-wine stain in the sacral area, and Caesarean section performed 8 years before, when diffuse pigmentation in the peritoneum was noted. Magnetic resonance (MR) imaging of the spine revealed an intramedullary tumor at T10 level with paramagnetic signal characteristics. The spinal cord tumor was totally removed, and the histological diagnosis was melanocytoma. Three months later, a left retroperitoneal mass with histological features of melanocytic tumor was removed. Neither tumors recurred and the patient stays ambulatory 4 years after the surgery. Multiple subtypes of melanocytic tumors with distinctive features of NCM and PPV can develop simultaneously, mimicking malignant melanoma. Gross total resection of each tumor, when indicated, is beneficial.

  5. Primary hyperparathyroidism

    PubMed Central

    Bilezikian, John P; Cusano, Natalie E.; Khan, Aliya A.; Liu, Jian-Min; Marcocci, Claudio; Bandeira, Francisco

    2017-01-01

    Primary hyperparathyroidism (PHPT) is a common disorder in which parathyroid hormone (PTH) is excessively secreted from one or more of the four parathyroid glands. A single benign parathyroid adenoma is the cause in most people. However, multiglandular disease is not rare and is typically seen in familial PHPT syndromes. The genetics of PHPT is usually monoclonal when a single gland is involved and polyclonal when multiglandular disease is present. The genes that have been implicated in PHPT include proto-oncogenes and tumour-suppressor genes. Hypercalcaemia is the biochemical hallmark of PHPT. Usually, the concentration of PTH is frankly increased but can remain within the normal range, which is abnormal in the setting of hypercalcaemia. Normocalcaemic PHPT, a variant in which the serum calcium level is persistently normal but PTH levels are increased in the absence of an obvious inciting stimulus, is now recognized. The clinical presentation of PHPT varies from asymptomatic disease (seen in countries where biochemical screening is routine) to classic symptomatic disease in which renal and/or skeletal complications are observed. Management guidelines have recently been revised to help the clinician to decide on the merits of a parathyroidectomy or a non-surgical course. This Primer covers these areas with particular attention to the epidemiology, clinical presentations, genetics, evaluation and guidelines for the management of PHPT. PMID:27194212

  6. Primary Hyperparathyroidism

    PubMed Central

    Bandeira, Leonardo; Bilezikian, John

    2016-01-01

    Over the past several generations, primary hyperparathyroidism (PHTP) has undergone a change in its clinical presentation in many countries from a symptomatic disease to an asymptomatic one. The reasons for this change in clinical presentation are related to the widespread use of biochemical screening tests, to the measurement of PTH more routinely in the evaluation of metabolic bone disease and to the status of vitamin D sufficiency in the population. Along with recognition of a broader clinical spectrum of disease, including a more recently recognized normocalcemic variant, has come an appreciation that the evaluation of classic target organs that can be affected in PHPT, such as the skeleton and the kidneys, require more advanced imaging technology for complete evaluation. It is clear that even in asymptomatic patients, evidence for microstructural disease in the skeleton and calcifications in the kidneys can be demonstrated often. Potential non-classical manifestations of PHPT related to neurocognition and the cardiovascular system continue to be of interest. As a result of these advances, revised guidelines for the management of asymptomatic PHPT have been recently published to help the clinician determine whether surgery is appropriate or whether a more conservative approach is acceptable. PMID:27508075

  7. Primary hyperoxaluria.

    PubMed

    Lorenzo, Víctor; Torres, Armando; Salido, Eduardo

    2014-05-21

    Primary hyperoxaluria (PH) occurs due to an autosomal recessive hereditary disorder of the metabolism of glyoxylate, which causes excessive oxalate production. The most frequent and serious disorder is due to enzyme deficit of alanine-glyoxylate aminotransferase (PH type I) specific to hepatic peroxisome. As oxalate is not metabolised in humans and is excreted through the kidneys, the kidney is the first organ affected, causing recurrent lithiasis, nephrocalcinosis and early renal failure. With advance of renal failure, particularly in patients on haemodialysis (HD), calcium oxalate is massively deposited in tissues, which is known as oxalosis. Diagnosis is based on family history, the presence of urolithiasis and/or nephrocalcinosis, hyperoxaluria, oxalate deposits in tissue forming granulomas, molecular analysis of DNA and enzyme analysis if applicable. High diagnostic suspicion is required; therefore, unfortunately, in many cases it is diagnosed after its recurrence following kidney transplantation. Conservative management of this disease (high liquid intake, pyridoxine and crystallisation inhibitors) needs to be adopted early in order to delay kidney damage. Treatment by dialysis is ineffective in treating excess oxalate. After the kidney transplant, we normally observe a rapid appearance of oxalate deposits in the graft and the results of this technique are discouraging, with very few exceptions. Pre-emptive liver transplantation, or simultaneous liver and kidney transplants when there is already irreversible damage to the kidney, is the treatment of choice to treat the underlying disease and suppress oxalate overproduction. Given its condition as a rare disease and its genetic and clinical heterogeneity, it is not possible to gain evidence through randomised clinical trials. As a result, the recommendations are established by groups of experts based on publications of renowned scientific rigour. In this regard, a group of European experts (OxalEurope) has

  8. [Primary lipodystrophies].

    PubMed

    Capeau, J; Magré, J; Lascols, O; Caron, M; Béréziat, V; Vigouroux, C

    2007-02-01

    Primary lipodystrophies represent a heterogeneous group of very rare diseases with a prevalence of less than 1 case for 100.000, inherited or acquired, caracterized by a loss of body fat either generalized or localized (lipoatrophy). In some forms, lipoatrophy is associated with a selective hypertrophy of other fat depots. Clinical signs of insulin resistance are often present: acanthosis nigricans, signs of hyperandrogenism. All lipodystrophies are associated with dysmetabolic alterations with insulin resistance, altered glucose tolerance or diabetes and hypertriglyceridemia leading to a risk of acute pancreatitis. Chronic complications are those resulting from diabetes involving the retina, kidney and nerves, cardiovascular complications and steatotic liver lesions that could result in cirrhosis. Genetic forms of generalized lipodystrophy (or Berardinelli-Seip syndrome) result, in most cases, from recessive mutations in one of two genes: either BSCL2 coding seipin or BSCL1 coding AGPAT2, an acyl-transferase involved in triglyceride synthesis. Acquired generalized lipodystrophy (Lawrence syndrome) is of unknown origin but is sometimes associated with signs of autoimmunity. Partial lipodystrophies can be familial with dominant transmission. Heterozygous mutations have been identified in the LMNA gene encoding nuclear lamin A/C belonging to the nuclear lamina, or in PPARG encoding the adipogenic transcription factor PPARgamma. Some less typical lipodystrophies, associated with signs of premature aging, have been linked to mutations in LMNA or in the ZMPSTE24 gene encoding the protease responsible for the maturation of prelamin A into lamin A. Acquired partial lipodystrophy (Barraquer-Simons syndrome) is characterized by cephalothoracic fat loss. Its aetiology is unknown but mutations in LMNB2, encoding the lamina protein lamin B2, could represent susceptibility factors. Highly active antiretroviral treatments for HIV infection are currently the most frequent cause

  9. Less Invasive Endometrial Cancer Surgery with Extraperitoneal Pelvic and Para-aortic Lymphadenectomy via a Small Midline Abdominal Incision and the Retroperitoneal Approach.

    PubMed

    Komiyama, Shinichi; Takeya, Chiaki; Takahashi, Rena; Nagasaki, Sumito; Kubushiro, Kaneyuki

    2016-01-01

    [Objective] To achieve less invasive lymphadenectomy in endometrial cancer patients, we performed extraperitoneal pelvic and para-aortic lymphadenectomy via a small midline abdominal incision with retroperitoneal approach. The feasibility and safety of this method were investigated. [Methods] Inclusion criteria were 1) endometrioid adenocarcinoma diagnosed by preoperative biopsy, 2) myometrial invasion by magnetic resonance imaging, and 3) no peritoneal dissemination or distant metastasis by computed tomography. Systematic extraperitoneal dissection of pelvic and para-aortic lymph nodes was performed via an approximately 12-cm midline lower abdominal incision, after which hysterectomy and bilateral salpingo-oophorectomy were done (extraperitoneal group). The historical control group was patients who underwent standard transperitoneal lymphadenectomy followed by hysterectomy and bilateral salpingo-oophorectomy. The two groups were compared for demographic characteristics, perioperative factors, and complications. [Results] A total of 62 patients were enrolled. Demographic and clinicopathological factors showed no differences between the extraperitoneal group (n = 34) and the historical control group (n = 28). The median number of pelvic (30 vs. 28) and para-aortic (14 vs. 17) nodes dissected was also similar. However, median intraoperative blood loss was significantly smaller in the extraperitoneal group than the control group (220 vs. 573 g). Median operating time (265 vs. 323.5 min), median laparotomy time (60 vs. 295 min), and median initial flatus time (8 vs. 32 hours) were all significantly shorter in the extraperitoneal group, while complications and severe postoperative pain were significantly less frequent. [Conclusions] Our new technique was feasible, safe, and less invasive than standard laparotomy. It is an alternative to laparoscope-assisted or robotic procedures.

  10. Less Invasive Endometrial Cancer Surgery with Extraperitoneal Pelvic and Para-aortic Lymphadenectomy via a Small Midline Abdominal Incision and the Retroperitoneal Approach

    PubMed Central

    Komiyama, Shinichi; Takeya, Chiaki; Takahashi, Rena; Nagasaki, Sumito; Kubushiro, Kaneyuki

    2016-01-01

    [Objective] To achieve less invasive lymphadenectomy in endometrial cancer patients, we performed extraperitoneal pelvic and para-aortic lymphadenectomy via a small midline abdominal incision with retroperitoneal approach. The feasibility and safety of this method were investigated. [Methods] Inclusion criteria were 1) endometrioid adenocarcinoma diagnosed by preoperative biopsy, 2) myometrial invasion by magnetic resonance imaging, and 3) no peritoneal dissemination or distant metastasis by computed tomography. Systematic extraperitoneal dissection of pelvic and para-aortic lymph nodes was performed via an approximately 12-cm midline lower abdominal incision, after which hysterectomy and bilateral salpingo-oophorectomy were done (extraperitoneal group). The historical control group was patients who underwent standard transperitoneal lymphadenectomy followed by hysterectomy and bilateral salpingo-oophorectomy. The two groups were compared for demographic characteristics, perioperative factors, and complications. [Results] A total of 62 patients were enrolled. Demographic and clinicopathological factors showed no differences between the extraperitoneal group (n = 34) and the historical control group (n = 28). The median number of pelvic (30 vs. 28) and para-aortic (14 vs. 17) nodes dissected was also similar. However, median intraoperative blood loss was significantly smaller in the extraperitoneal group than the control group (220 vs. 573 g). Median operating time (265 vs. 323.5 min), median laparotomy time (60 vs. 295 min), and median initial flatus time (8 vs. 32 hours) were all significantly shorter in the extraperitoneal group, while complications and severe postoperative pain were significantly less frequent. [Conclusions] Our new technique was feasible, safe, and less invasive than standard laparotomy. It is an alternative to laparoscope-assisted or robotic procedures. PMID:27313778

  11. Fluorescence-guided surgery of retroperitoneal-implanted human fibrosarcoma in nude mice delays or eliminates tumor recurrence and increases survival compared to bright-light surgery.

    PubMed

    Uehara, Fuminari; Hiroshima, Yukihiko; Miwa, Shinji; Tome, Yasunori; Yano, Shuya; Yamamoto, Mako; Matsumoto, Yasunori; Maehara, Hiroki; Tanaka, Kazuhiro; Bouvet, Michael; Kanaya, Fuminori; Hoffman, Robert M

    2015-01-01

    The aim of this study is to determine if fluorescence-guided surgery (FGS) can eradicate human fibrosarcoma growing in the retroperitoneum of nude mice. One week after retroperitoneal implantation of human HT1080 fibrosarcoma cells, expressing green fluorescent protein (GFP) (HT-1080-GFP), in nude mice, bright-light surgery (BLS) was performed on all tumor-bearing mice (n = 22). After BLS, mice were randomized into 2 treatment groups; BLS-only (n = 11) or the combination of BLS + FGS (n = 11). The residual tumors remaining after BLS were resected with FGS using a hand-held portable imaging system under fluorescence navigation. The average residual tumor area after BLS + FGS was significantly smaller than after BLS-only (0.4 ± 0.4 mm(2) and 10.5 ± 2.4 mm(2), respectively; p = 0.006). Five weeks after surgery, the fluorescent-tumor areas of BLS- and BLS + FGS-treated mice were 379 ± 147 mm(2) and 11.7 ± 6.9 mm(2), respectively, indicating that FGS greatly inhibited tumor recurrence compared to BLS. The combination of BLS + FGS significantly decreased fibrosarcoma recurrence compared to BLS-only treated mice (p < 0.001). Mice treated with BLS+FGS had a significantly higher disease-free survival rate than mice treated with BLS-only at five weeks after surgery. These results suggest that combination of BLS + FGS significantly reduced the residual fibrosarcoma volume after BLS and improved disease-free survival.

  12. Primary Progressive Aphasia

    MedlinePlus

    Primary progressive aphasia Overview By Mayo Clinic Staff Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system (neurological) syndrome ... your ability to communicate. People with primary progressive aphasia can have trouble expressing their thoughts and understanding ...

  13. Diagnosis of Liver Involvement in Primary Sjögren Syndrome

    PubMed Central

    Zeron, Pilar Brito; Retamozo, Soledad; Bové, Albert; Kostov, Belchin Adriyanov; Sisó, Antoni

    2013-01-01

    Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sjögren syndrome (SS). In the 1990s, a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease, liver function and a complete panel of autoantibodies. Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS. The most recent studies have shown that, after eliminating hepatotoxic drugs and fatty liver disease, the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases. The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important, since the two processes require different therapeutic approaches and have different prognoses. With respect to viral infections, chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area, while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries. After eliminating viral hepatitis, primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS. PBC-related SS patients may have a broad spectrum of abnormalities of the liver, including having no clinical or analytical data suggestive of liver disease. Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I), and nearly 10% of these patients have an AIH-PBC overlap. Finally, IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis, especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present. PMID:26355632

  14. 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

  15. Radioanatomy of the retroperitoneal space.

    PubMed

    Coffin, A; Boulay-Coletta, I; Sebbag-Sfez, D; Zins, M

    2015-02-01

    The retroperitoneum is a space situated behind the parietal peritoneum and in front of the transversalis fascia. It contains further spaces that are separated by the fasciae, between which communication is possible with both the peritoneal cavity and the pelvis, according to the theory of interfascial spread. The perirenal space has the shape of an inverted cone and contains the kidneys, adrenal glands, and related vasculature. It is delineated by the anterior and posterior renal fasciae, which surround the ureter and allow communication towards the pelvis. At the upper right pole, the perirenal space connects to the retrohepatic space at the bare area of the liver. There is communication between these two spaces through the Kneeland channel. The anterior pararenal space contains the duodenum, pancreas, and the ascending and descending colon. There is free communication within this space, and towards the mesenteries along the vessels. The posterior pararenal space, which contains fat, communicates with the preperitoneal space at the anterior surface of the abdomen between the peritoneum and the transversalis fascia, and allows communication with the contralateral posterior pararenal space. This space follows the length of the ureter to the pelvis, which explains the communication between these areas and the length of the pelvic fasciae.

  16. [Surgical treatment of retroperitoneal masses].

    PubMed

    Kriger, A G; Berelavichus, S V; Son, A I; Gorin, D S; Akhtanin, E A; Kaldarov, A R; Smirnov, A V; Davydenko, P I; Kalinin, D V

    2017-01-01

    Введение. Неорганные забрюшинные опухоли (НЗО) — собирательная классификационная единица, объединяющая доброкачественные и злокачественные мезенхимальные новообразования, исходящие из забрюшинного пространства и не имеющие определенной органной принадлежности. Частота этих опухолей невелика и составляет 0,03—1,1%. Хирургическое вмешательство является основным в выборе тактики лечения. Цель — улучшить результат лечения больных с НЗО. Материал и методы. Оперированы 83 больных с НЗО. Доброкачественные опухоли были у 57 больных, злокачественные — у 26. Традиционным (Тр) лапаротомным методом оперированы 35 больных, робот-ассистированным (РА) — 34, лапароскопическим (Л) — 14 больных. Медиана размера опухоли в наибольшем измерении при Тр-методе составила 102 мм, при РА — 75 мм, при Л — 81,5 мм. Результаты. Длительность операции (медиана) при Тр-методе составила 112,5 мин, при РА — 140 мин, при Л — 125 мин. Медиана объема кровопотери при Тр-методе составила 125 мл, при РА — 50 мл, при Л — 50 мл. Конверсия при РА-методе потребовалась 1 раз, при Л — 7 раз. Послеоперационные осложнения возникли у 6 больных при Тр и у 5 больных при РА-методе. Летальных исходов не было. Вывод. Для достижения успешного хирургического лечения НЗО необходима оценка возможностей различных методов выполнения операции. Показанием к Тр-методу удаления НЗО является размер опухоли более 10 см, миниинвазивные методы оправданы и позволяют получить хорошие результаты при опухолях менее 10 см. РА-вариант вмешательства существенно облегчает выполнение операции при расположении опухоли в трудных для манипуляций областях и малых анатомических пространствах, в случае прилежания опухоли к магистральным сосудам.

  17. Changes in Brain Function in Patients With Stage I, Stage II, Stage III, or Stage IV Ovarian, Primary Peritoneal, or Fallopian Tube Cancer Who Are Receiving Chemotherapy

    ClinicalTrials.gov

    2016-10-26

    Cognitive Side Effects of Cancer Therapy; Malignant Ovarian Epithelial Tumor; Ovarian Brenner Tumor; Ovarian Carcinosarcoma; Ovarian Choriocarcinoma; Ovarian Clear Cell Cystadenocarcinoma; Ovarian Dysgerminoma; Ovarian Embryonal Carcinoma; Ovarian Endometrioid Adenocarcinoma; Ovarian Mixed Germ Cell Tumor; Ovarian Mucinous Cystadenocarcinoma; Ovarian Polyembryoma; Ovarian Sarcoma; Ovarian Seromucinous Carcinoma; Ovarian Serous Cystadenocarcinoma; Ovarian Teratoma; Ovarian Yolk Sac Tumor; Stage I Ovarian Cancer; Stage IA Fallopian Tube Cancer; Stage IA Ovarian Cancer; Stage IA Ovarian Germ Cell Tumor; Stage IB Fallopian Tube Cancer; Stage IB Ovarian Cancer; Stage IB Ovarian Germ Cell Tumor; Stage IC Fallopian Tube Cancer; Stage IC Ovarian Cancer; Stage IC Ovarian Germ Cell Tumor; Stage II Ovarian Cancer; Stage IIA Fallopian Tube Cancer; Stage IIA Ovarian Cancer; Stage IIA Ovarian Germ Cell Tumor; Stage IIB Fallopian Tube Cancer; Stage IIB Ovarian Cancer; Stage IIB Ovarian Germ Cell Tumor; Stage IIC Fallopian Tube Cancer; Stage IIC Ovarian Cancer; Stage IIC Ovarian Germ Cell Tumor; Stage IIIA Fallopian Tube Cancer; Stage IIIA Ovarian Cancer; Stage IIIA Ovarian Germ Cell Tumor; Stage IIIA Primary Peritoneal Cancer; Stage IIIB Fallopian Tube Cancer; Stage IIIB Ovarian Cancer; Stage IIIB Ovarian Germ Cell Tumor; Stage IIIB Primary Peritoneal Cancer; Stage IIIC Fallopian Tube Cancer; Stage IIIC Ovarian Cancer; Stage IIIC Ovarian Germ Cell Tumor; Stage IIIC Primary Peritoneal Cancer; Stage IV Fallopian Tube Cancer; Stage IV Ovarian Cancer; Stage IV Ovarian Germ Cell Tumor; Stage IV Primary Peritoneal Cancer; Undifferentiated Ovarian Carcinoma

  18. [Multiple primary neoplasms with renal neurilemmoma: a case report and literature review].

    PubMed

    Qiu, Min; Tian, Xiao-jun; Ma, Lu-lin; Lu, Min; Lu, Jian; Yan, Ye

    2015-08-18

    Multiple primary neoplasms are not common, those containing renal neurilemoma are even more rare. Our study involves the diagnosis and treatment of a female patient with multiple primary neoplasms with renal neurilemoma. She was previously diagnosed with left ovarian dermoid cyst, right posterior mediastinal neurilemmoma, left forearm neurilemmoma, and papillary thyroid carcinoma, underwent operation treatment. Physical examination reveals a left renal mass with a left adrenal tumor a month before, and underwent retroperitoneal laparoscopic left adrenal nodule resection and left partial nephrectomy. Operation was successful, without conversions to open. Artery occlusion time was 18 min, and blood loss was 20 mL. Post operation pathologic result shows a left renal cellular leiomyoma neurilemmoma with focal cystic change, and tumor diameter was about 3 cm. Immunohistochemistry reports S-100 (+), Ki-67 (15%+), and short-term follow-up without recurrence. In our case of study, even though the tumors were heterochronism, they were both treated with surgical resection. Renal neurilemoma is benign. Laparoscopic partial nephrectomy is not only minimally invasive, fast in recovery, but most importantly preserves renal tissue to the largest extent. It can prevent preoperative pathological uncertainty, which often undergoes radical nephrectomy.

  19. Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature

    PubMed Central

    Sasaki Yatabe, Midori; Watanabe, Kimio; Hayashi, Yoshimitsu; Yatabe, Junichi; Morimoto, Satoshi; Ichihara, Atsuhiro; Nakayama, Masaaki; Watanabe, Tsuyoshi

    2017-01-01

    The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus. PMID:28049999

  20. Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature.

    PubMed

    Sasaki Yatabe, Midori; Watanabe, Kimio; Hayashi, Yoshimitsu; Yatabe, Junichi; Morimoto, Satoshi; Ichihara, Atsuhiro; Nakayama, Masaaki; Watanabe, Tsuyoshi

    The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus.

  1. Clinicopathological and immunohistochemical features of primary central nervous system germ cell tumors: a 24-years experience.

    PubMed

    Gao, Yuping; Jiang, Jiyao; Liu, Qiang

    2014-01-01

    Primary central nervous system (CNS) germ cell tumors (GCTs) are a rare heterogeneous group of lesions, which the clinicopathological features have a marked degree of heterogeneity comparing with that of gonadal GCTs. Accurately diagnosing CNS GCTs might be extremely difficult and requires immunohistochemical verification. This study was to investigate the biological feature of CNS GCTs and diagnostic value of immunohistochemical markers OCT3/4, C-kit, PLAP, and CD30 in CNS GCTs. A retrospective study was performed on 34 patients with CNS germ cell tumors between 1990 and 2014. 34 CNS GCTs account for 9.2% of all primary CNS neoplasms. The sellar region (35.3%) and pineal gland (17.6%) were the most common sites of intracranial GCTs. Hydrocephalus (82.4%) and diplopia (46.9%) were the two most common clinical presentations. The most common histological subtypes were germinoma (67.6%). PLAP, c-kit, OCT3/4 were highly expressed in gernimomas. CD30 and CK AE1/3 stainings were positive in embryonal carcinoma. Yolk sac tumor component showed positive staining for AFP and CK AE1/3. β-HCG staining was positive in choriocarcinoma and STGC. Patients with mature teratomas and germinomas had a better prognosis (a 5-year survival rate) than those with embryonal carcinoma and choriocarcinoma (a 5-year survival rates were 0). Our finding suggest that the incidences of primary CNS GCTs are higher in South China than in the West, but mixed GCTs are uncommon in our study. The judicious use of a panel of selected markers is helpful in diagnosing and predicting the prognosis for CNS GCTs.

  2. Primary pancreatic lymphoma: two case reports and a literature review

    PubMed Central

    Yu, Lili; Chen, Yajun; Xing, Ligang

    2017-01-01

    Primary pancreatic lymphoma (PPL) is an extremely rare disease, with only a few cases reported in the literature. Clinical manifestations of PPL are often nonspecific and may mimic other pancreatic diseases. Because of the limited experience of PPL, clinicopathological features, differential diagnosis, optimal therapy, and outcomes are not well defined. We described two cases diagnosed as PPL and confirmed by histological examination and immunohistochemical analysis. Case 1 was a young man with obstructive jaundice and upper abdominal malaise mimicking a pancreatic adenocarcinoma. A computed tomography (CT) scan revealed a diffuse heterogeneous mass in the head of the pancreas along with dilated bile ducts, no dilated pancreatic duct, no liver or splenic involvement, or evident retroperitoneal adenopathies. The patient underwent a pancreatico-duodenectomy, and the postoperative histopathology confirmed diffuse large B-cell non-Hodgkin lymphoma. Postoperatively, he received six courses of the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone). Case 2 was an older man with left flank pain. A CT confirmed a mass with irregular margins at the tail of the pancreas and the hilum of the spleen. The mass was heterogeneous, with no clear boundary between lesions, spleen, stomach, and pancreas, with nearby blood vessels wrapped around it, and multiple enlarged lymph nodes in the abdominal cavity. A CT-guided biopsy was performed. The immunohistological findings of the specimen revealed a diffuse large B-cell lymphoma. The size of the tumor was significantly reduced after four cycles of the CHOP chemotherapy regimen. These two cases were different in clinical manifestation, location, and treatment. We reviewed the literature and discussed the clinicopathological features, differential diagnosis, optimal therapy, and outcomes of this neoplasm. PMID:28356755

  3. Brain tumor - primary - adults

    MedlinePlus

    ... Vestibular schwannoma (acoustic neuroma) - adults; Meningioma - adults; Cancer - brain tumor (adults) ... Primary brain tumors include any tumor that starts in the brain. Primary brain tumors can start from brain cells, ...

  4. 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.

  5. Primary enzyme quantitation

    DOEpatents

    Saunders, G.C.

    1982-03-04

    The disclosure relates to the quantitation of a primary enzyme concentration by utilizing a substrate for the primary enzyme labeled with a second enzyme which is an indicator enzyme. Enzyme catalysis of the substrate occurs and results in release of the indicator enzyme in an amount directly proportional to the amount of primary enzyme present. By quantifying the free indicator enzyme one determines the amount of primary enzyme present.

  6. Investigating Primary Source Literacy

    ERIC Educational Resources Information Center

    Archer, Joanne; Hanlon, Ann M.; Levine, Jennie A.

    2009-01-01

    Primary source research requires students to acquire specialized research skills. This paper presents results from a user study testing the effectiveness of a Web guide designed to convey the concepts behind "primary source literacy". The study also evaluated students' strengths and weaknesses when conducting primary source research. (Contains 3…

  7. Primary Care's Dim Prognosis

    ERIC Educational Resources Information Center

    Alper, Philip R.

    2010-01-01

    Given the chorus of approval for primary care emanating from every party to the health reform debate, one might suppose that the future for primary physicians is bright. Yet this is far from certain. And when one looks to history and recognizes that primary care medicine has failed virtually every conceivable market test in recent years, its…

  8. Primary Intraosseous Meningioma.

    PubMed

    Chen, Thomas C

    2016-04-01

    Primary intraosseous meningiomas are a subtype of primary extradural meningiomas. They represent approximately two-thirds of extradural meningiomas and fewer than 2% of meningiomas overall. These tumors originate within the bones of the skull and can have a clinical presentation and radiographic differential diagnosis different from those for intradural meningiomas. Primary intraosseous meningiomas are classified based on location and histopathologic characteristics. Treatment is primarily surgical resection with wide margins if possible. Sparse literature exists regarding the use of adjuvant therapies. The literature regarding primary intraosseous meningiomas consists primarily of clinical case reports and case series. This literature is reviewed and summarized in this article.

  9. EMR Curriculum Guide: Primary.

    ERIC Educational Resources Information Center

    Ruschmeier, Veronica M., Ed.; Rockwell, Linda, Ed.

    Presented is a curriculum guide for educable mentally retarded children in primary and intermediate grades which specifies behavioral and interim objectives in the areas of basic verbal and arithmetic skills, vocational competencies, social competencies, and physical skills. Objectives such as the following are identified at the primary level:…

  10. Using Primary Source Documents.

    ERIC Educational Resources Information Center

    Mintz, Steven

    2003-01-01

    Explores the use of primary sources when teaching about U.S. slavery. Includes primary sources from the Gilder Lehrman Documents Collection (New York Historical Society) to teach about the role of slaves in the Revolutionary War, such as a proclamation from Lord Dunmore offering freedom to slaves who joined his army. (CMK)

  11. Medics in Primary School

    ERIC Educational Resources Information Center

    Press, Colin

    2003-01-01

    Some time ago a flyer on "Medics in Primary School" came the author's way. It described a programme for making placements in primary schools available to medical students. The benefits of the program to medical students and participating schools were highlighted, including opportunities to develop communication skills and demystify…

  12. Transforming Primary Mathematics

    ERIC Educational Resources Information Center

    Askew, Mike

    2011-01-01

    What is good mathematics teaching? What is mathematics teaching good for? Who is mathematics teaching for? These are just some of the questions addressed in "Transforming Primary Mathematics", a highly timely new resource for teachers which accessibly sets out the key theories and latest research in primary maths today. Under-pinned by findings…

  13. Tubal teratoma causing anti-NMDAR encephalitis.

    PubMed

    Power, Laura; James, Joann; Masoud, Ibtisam; Altman, Alon

    2014-12-01

    Contexte : L’encéphalite à anticorps anti-récepteur du N-méthyl d-aspartate (NMDA) est considérée comme un trouble auto-immun rare qui est habituellement causé par un tératome ovarien. La maladie se caractérise, sur le plan clinique, par des symptômes neuropsychiatriques suivis de convulsions réfractaires et d’une hypoventilation; sans traitement, elle est mortelle. Cas : Nous présentons un cas d’encéphalite à anticorps anti-récepteur du NMDA comptant des caractéristiques classiques chez une jeune femme pour laquelle le diagnostic et le traitement ultimes ont été différés en raison de multiples circonstances évitables qui n’ont été mises au jour qu’a posteriori. Conclusion : Nous estimons que ce trouble est en fait plus courant que ce que les signalements à son égard peuvent nous porter à croire. La présence possible d’une encéphalite à anticorps anti-récepteur du NMDA doit être envisagée dans le cadre du diagnostic différentiel de toute jeune femme présentant des symptômes psychiatriques d’apparition nouvelle. Chez les jeunes femmes qui connaissent une baisse aiguë du niveau de conscience, une surveillance immédiate et continue visant à repérer la présence d’une tumeur sous-jacente devrait être mise en œuvre, particulièrement en présence d’une réaction différée au traitement empirique.

  14. [The primary healthcare centres].

    PubMed

    Brambilla, Antonio; Maciocco, Gavino

    2014-04-01

    The central attributes of primary care are: first contact (accessibility), longitudinality (person- focused preventive and curative care overtime), patient-oriented comprehensiveness and coordination (including navigation towards secondary and tertiary care). Besides taking care of the needs of the individuals, primary health care teams are also looking at the community, especially when addressing social determinants of health. The rationale for the benefits for primary care for health has been found in: 1) greater access to needed services; 2) better quality of care; 3) a greater focus on prevention; 4) early management of health problems; 5) organizing and delivering high quality care for chronic non-communicable diseases. This paper describes the role of primary healthcare centres in strengthening community primary services and in reducing health inequalities. Furthemore, the experiences of Regional Health Services from Tuscany and Emilia-Romagna are discussed, with a brief overview of the literature.

  15. Primary Intraocular Lymphoma

    PubMed Central

    Faia, Lisa J.; Chan, Chi-Chao

    2009-01-01

    Primary intraocular lymphoma, recently suggested to be renamed primary retinal lymphoma, is a subset of primary central nervous system lymphoma and is usually an aggressive diffuse large B-cell lymphoma. Between 56% and 85% of patients who initially present with primary intraocular lymphoma alone will develop cerebral lesions. Patients typically complain of decreased vision and floaters, most likely secondary to the chronic vitritis and subretinal lesions. The diagnosis of primary intraocular lymphoma can be difficult to make and requires tissue for diagnosis. The atypical lymphoid cells are large and display a high nuclear to cytoplasmic ratio, prominent nucleoli, and basophilic cytoplasm. Flow cytometry, immunohistochemistry, cytokine analysis, and gene rearrangements also aid in the diagnosis. Local and systemic treatments, such as chemotherapy and radiation, are employed, although the relapse rate remains high. PMID:19653715

  16. 'Biracial'-Looking Twins: A New Twin Type?/Twin Research: Twins with Cystic Teratomas; Sleep Quality and Body Mass Index; Previable Membrane Rupture/Print and Online Reports: Twins Born to a Sister Surrogate; NASA Twin Study; African-Cosmopolitan Twin Fashion Inspirations; Triplet Hockey Stars.

    PubMed

    Segal, Nancy L

    2017-03-28

    Dizygotic (DZ) co-twins born to mothers and fathers from different racial or ethnic backgrounds often resemble one parent much more than the other. As such, these pairs comprise a unique subset of twins for investigating how others' responses to their different looks may affect their personalities and self-esteem. This article describes some of these twin pairs and some challenges of raising them, and suggests ways they may be used in research. Next, recent twin research on cystic teratomas, relations between sleep quality and body mass index, and previable membrane rupture is described. The final section concerns twins, twin studies, and related events in the media, namely: twins born to a sister surrogate, the NASA twin investigation, inspiring African-Cosmopolitan twins in fashion, and triplet Hockey Stars.

  17. Visualizing renal primary cilia.

    PubMed

    Deane, James A; Verghese, Elizabeth; Martelotto, Luciano G; Cain, Jason E; Galtseva, Alya; Rosenblum, Norman D; Watkins, D Neil; Ricardo, Sharon D

    2013-03-01

    Renal primary cilia are microscopic sensory organelles found on the apical surface of epithelial cells of the nephron and collecting duct. They are based upon a microtubular cytoskeleton, bounded by a specialized membrane, and contain an array of proteins that facilitate their assembly, maintenance and function. Cilium-based signalling is important for the control of epithelial differentiation and has been implicated in the pathogenesis of various cystic kidney diseases and in renal repair. As such, visualizing renal primary cilia and understanding their composition has become an essential component of many studies of inherited kidney disease and mechanisms of epithelial regeneration. Primary cilia were initially identified in the kidney using electron microscopy and this remains a useful technique for the high resolution examination of these organelles. New reagents and techniques now also allow the structure and composition of primary cilia to be analysed in detail using fluorescence microscopy. Primary cilia can be imaged in situ in sections of kidney, and many renal-derived cell lines produce primary cilia in culture providing a simplified and accessible system in which to investigate these organelles. Here we outline microscopy-based techniques commonly used for studying renal primary cilia.

  18. Primary vascular access.

    PubMed

    Gibbons, C P

    2006-05-01

    Primary vascular access is usually achievable by a distal autogenous arterio-venous fistula (AVF). This article describes the approach to vascular access planning, the usual surgical options and the factors affecting patency.

  19. Primary infertility (image)

    MedlinePlus

    Primary infertility is a term used to describe a couple that has never been able to conceive a pregnancy ... to do so through unprotected intercourse. Causes of infertility include a wide range of physical as well ...

  20. Parenthood after Primary Infertility.

    ERIC Educational Resources Information Center

    Frances-Fischer, Jana E.; Lightsey, Owen Richard, Jr.

    2003-01-01

    Reviews the literature on the experience of parenting after primary infertility and describes construction and initial testing of an instrument for assessing characteristics of this understudied population. (Contains 52 references and 4 tables.) (GCP)

  1. Primary Nurse - Role Evolution

    ERIC Educational Resources Information Center

    Mundinger, Mary O'Neil

    1973-01-01

    Primary nursing means that each patient has an individual nurse who is responsible for assessing his nursing needs and planning and evaluating his nursing care. The article describes the advantages and problems connected with this approach to patient care. (AG)

  2. Inside the Primary Classroom.

    ERIC Educational Resources Information Center

    Simon, Brian

    1980-01-01

    Presents some of the findings of the ORACLE research program (Observational Research and Classroom Learning Evaluation), a detailed observational study of teacher-student interaction, teaching styles, and management methods within a sample of primary classrooms. (Editor/SJL)

  3. Primary aldosteronism and pregnancy.

    PubMed

    Morton, Adam

    2015-10-01

    Primary aldosteronism is the most common cause of secondary hypertension. Less than 50 cases of pregnancy in women with primary aldosteronism have been reported, suggesting the disorder is significantly underdiagnosed in confinement. Accurate diagnosis is complicated by physiological changes in the renin-angiotensin-aldosterone axis in pregnancy, leading to a risk of false negative results on screening tests. The course of primary aldosteronism during pregnancy is highly variable, although overall it is associated with a very high risk of fetal and maternal morbidity and mortality. The optimal management of primary aldosteronism during pregnancy is unclear, with uncertainty regarding the safety of mineralocorticoid antagonists and amiloride, their relative efficacy compared with the antihypertensive medications commonly used during pregnancy, and as to whether prognosis is improved by laparoscopic adrenalectomy where an adrenal adenoma can be demonstrated.

  4. Primary biliary cirrhosis

    MedlinePlus

    ... medlineplus.gov/ency/article/000282.htm Primary biliary cirrhosis To use the sharing features on this page, ... and leads to scarring of the liver called cirrhosis. This is called biliary cirrhosis. Causes The cause ...

  5. Primary actinomycosis of hand

    PubMed Central

    Padhi, Sanghamitra; Dash, Muktikesh; Turuk, Jyotirmayee; Sahu, Rani; Panda, Pritilata

    2014-01-01

    Actinomycosis is a chronic granulomatous suppurative disease having the propensity for extension to the contagious tissue with the formation of multiple discharging sinus tracts. Primary actinomycosis of extremity is a very uncommon clinical entity and is commonly considered as a soft-tissue infection. We report here, a case of primary actinomycosis of the upper extremity in a 24-year-old male who was treated successfully with surgical excision and extended period of antimicrobial treatment. PMID:25538911

  6. Primary headache disorders.

    PubMed

    Benoliel, Rafael; Eliav, Eli

    2013-07-01

    Primary headache disorders include migraine, tension-type headaches, and the trigeminal autonomic cephalgias (TACs). "Primary" refers to a lack of clear underlying causative pathology, trauma, or systemic disease. The TACs include cluster headache, paroxysmal hemicrania, and short-lasting neuralgiform headache attacks with conjunctival injection and tearing; hemicrania continua, although classified separately by the International Headache Society, shares many features of both migraine and the TACs. This article describes the features and treatment of these disorders.

  7. Primary vitreoretinal lymphoma

    PubMed Central

    Mulay, Kaustubh; Narula, Ritesh; Honavar, Santosh G

    2015-01-01

    Primary vitreoretinal lymphoma (PVRL) is an uncommon, but potentially fatal intraocular malignancy, which may occur with or without primary central nervous system lymphoma (PCNSL). Considered to be a subset of PCNSL, it is mostly of diffuse large B-cell type. The diagnosis of PVRL poses a challenge not only to the clinician, but also to the pathologist. Despite aggressive treatment with chemotherapy and/or radiotherapy, relapses or CNS involvement are common. PMID:25971162

  8. Primary care research ethics.

    PubMed Central

    Jones, R; Murphy, E; Crosland, A

    1995-01-01

    Research activity in primary care is increasing rapidly, and raises a range of specific ethical issues. Many of these relate to the involvement of individuals in the community who are not seeking medical care and to the impact of research participation on relationships between general practitioners and their patients. The ethical issues pertinent to a range of quantitative and qualitative research methodologies in primary care are identified and considered. PMID:8554844

  9. Primary biliary cirrhosis.

    PubMed

    Carey, Elizabeth J; Ali, Ahmad H; Lindor, Keith D

    2015-10-17

    Primary biliary cirrhosis is a chronic cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, leading to fibrosis and potential cirrhosis through resulting complications. The serological hallmark of primary biliary cirrhosis is the antimitochondrial antibody, a highly disease-specific antibody identified in about 95% of patients with primary biliary cirrhosis. These patients usually have fatigue and pruritus, both of which occur independently of disease severity. The typical course of primary biliary cirrhosis has changed substantially with the introduöction of ursodeoxycholic acid (UDCA). Several randomised placebo-controlled studies have shown that UDCA improves transplant-free survival in primary biliary cirrhosis. However, about 40% of patients do not have a biochemical response to UDCA and would benefit from new therapies. Liver transplantation is a life-saving surgery with excellent outcomes for those with decompensated cirrhosis. Meanwhile, research on nuclear receptor hormones has led to the development of exciting new potential treatments. This Seminar will review the current understanding of the epidemiology, pathogenesis, and natural history of primary biliary cirrhosis, discuss management of the disease and its sequelae, and introduce research on new therapeutic options.

  10. Primary cerebral malignant melanoma

    PubMed Central

    Tang, Kai; Kong, Xiangyi; Mao, Gengsheng; Qiu, Ming; Zhu, Haibo; Zhou, Lei; Nie, Qingbin; Xu, Yi; Du, Shiwei

    2017-01-01

    Abstract Primary intracranial melanomas are uncommon and constitute approximately 1% of all melanoma cases and 0.07% of all brain tumors. In nature, these primary melanomas are very aggressive and can spread to other organs. We report an uncommon case of primary cerebral malignant melanoma—a challenging diagnosis guided by clinical presentations, radiological features, and surgical biopsy results, aiming to emphasize the importance of considering primary melanoma when making differential diagnoses of intracranial lesions. We present a rare case of a primary cerebral melanoma in the left temporal lobe. The mass appeared iso-hypodense on brain computed tomography (CT), short signal on T1-weighted magnetic resonance images (T1WI) and long signal on T2WI. It was not easy to make an accurate diagnosis before surgery. We showed the patient's disease course and reviewed related literatures, for readers’ reference. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary. After surgery, the pathological examination confirmed the diagnosis of melanoma. The patient was discharged without any complications and went on to receive adjuvant radiochemotherapy. It is difficult to diagnose primary cerebral melanoma in the absence of any cutaneous melanosis. A high index of clinical suspicion along with good pathology reporting is the key in diagnosing these extremely rare tumors. PMID:28121927

  11. Other primary headaches

    PubMed Central

    Bahra, Anish

    2012-01-01

    The ‘Other Primary Headaches’ include eight recognised benign headache disorders. Primary stabbing headache is a generally benign disorder which often co-exists with other primary headache disorders such as migraine and cluster headache. Primary cough headache is headache precipitated by valsalva; secondary cough has been reported particularly in association with posterior fossa pathology. Primary exertional headache can occur with sudden or gradual onset during, or immediately after, exercise. Similarly headache associated with sexual activity can occur with gradual evolution or sudden onset. Secondary headache is more likely with both exertional and sexual headache of sudden onset. Sudden onset headache, with maximum intensity reached within a minute, is termed thunderclap headache. A benign form of thunderclap headache exists. However, isolated primary and secondary thunderclap headache cannot be clinically differentiated. Therefore all headache of thunderclap onset should be investigated. The primary forms of the aforementioned paroxysmal headaches appear to be Indomethacin sensitive disorders. Hypnic headache is a rare disorder which is termed ‘alarm clock headache’, exclusively waking patients from sleep. The disorder can be Indomethacin responsive, but can also respond to Lithium and caffeine. New daily persistent headache is a rare and often intractable headache which starts one day and persists daily thereafter for at least 3 months. The clinical syndrome more often has migrainous features or is otherwise has a chronic tension-type headache phenotype. Management is that of the clinical syndrome. Hemicrania continua straddles the disorders of migraine and the trigeminal autonomic cephalalgias and is not dealt with in this review. PMID:23024566

  12. Generation of Endoderm derived Human iPS cells from Primary Hepatocytes

    PubMed Central

    Liu, Hua; Ye, Zhaohui; Kim, Yong-Hak; Sharkis, Saul; Jang, Yoon-Young

    2010-01-01

    Recent advances in induced pluripotent stem (iPS) cell research significantly changed our perspective on regenerative medicine. Patient specific iPS cells have been derived not only for disease modeling but also as sources for cell replacement therapy. However, there have been insufficient data to prove that iPS cells are functionally equivalent to hES cells or safer than hES cells. There are several important issues which need to be addressed and foremost are the safety and efficacy of human iPS cells from different origins. Human iPS cells have been derived mostly from cells originated from mesoderm, with a few cases from ectoderm. So far there has been no report of endoderm derived human iPS cells, preventing comprehensive comparative investigations on the quality of human iPS cells from different origins. Here we show for the first time reprogramming of human endoderm derived cells (i.e. primary hepatocytes) to pluripotency. Hepatocyte-derived iPS cells appear indistinguishable from human embryonic stem cells in colony morphology, growth properties, expression of pluripotency-associated transcription factors and surface markers, and differentiation potential in embryoid body formation and teratoma assays. In addition, these cells were able to directly differentiate into definitive endoderm, hepatic progenitors, and mature hepatocytes. The technology to develop endoderm derived human iPS cell lines, together with other established cell lines, will provide a foundation to elucidate the mechanisms of cellular reprogramming and to study the safety and efficacy of differentially originated human iPS cells for cell therapy. For studying liver disease pathogenesis, this technology also provides a potentially more amenable system to generate liver disease specific iPS cells. PMID:20432258

  13. Primary care development zones.

    PubMed

    Beardshaw, V; Gordon, P; Plamping, D

    1993-01-30

    Most commentators on the Tomlinson report have agreed with its emphasis on improving primary and community care. The three elements of such a strategy are a remedial programme to bring primary care up to national standards, a programme to provide such services to people with non-standard needs such as mobile Londoners, ethnic minorities, and homeless people, and the development of an expanded model of primary care. No one model will be appropriate across all of London. The process should start with an audit of existing resources and services within each community, together with an analysis of needs. From this would develop a local programme with specific plans for investment in premises, staffing, training, and management. New contractual mechanisms may be needed to attract practitioners, improve their premises, secure out of hours services, and provide medical cover for community beds. There should also be incentives for closer working between primary and secondary services. No developments on the scale needed for London have been carried out in primary care within the lifetime of the NHS--but their success will be critical to the calibre of health services for Londoners into the next century.

  14. Primary lymphoma of the brain

    MedlinePlus

    Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain ... The cause of primary brain lymphoma is not known. People with a weakened immune system are at high risk for primary lymphoma of the brain. ...

  15. 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.

  16. Plume primary smoke

    NASA Astrophysics Data System (ADS)

    Chastenet, J. C.

    1993-06-01

    The exhaust from a solid propellant rocket motor usually contains condensed species. These particles, also called 'Primary Smoke', are often prejudicial to missile detectability and to the guidance system. To avoid operational problems it is necessary to know and quantify the effects of particles on all aspects of missile deployment. A brief description of the origin of the primary smoke is given. It continues with details of the interaction between particles and light as function of both particles and light properties (nature, size, wavelength, etc). The effects of particles on plume visibility, attenuation of an optical beam propagated through the plume and the contribution of particles on optical signatures of the plume are also described. Finally, various methods used in NATO countries to quantify the primary smoke effects are discussed.

  17. [Primary care in Ireland].

    PubMed

    Sánchez-Sagrado, T

    2017-03-27

    Spanish doctors are still leaving the country to look for quality work. Ireland is not a country with many Spanish professionals but it is interesting to know its particular Health care system. Ireland is one of the countries with a national health care system, although it has a mixture of private health care insurance schemes. People have a right to health care if they have been living in Ireland at least for a year. Access to the primary care health system depends on age and income: free of charge for Category 1 and co-payments for the rest. This division generates great inequalities among the population. Primary Care doctors are self-employed, and they work independently. However, since 2001 they have tended to work in multidisciplinary teams in order to strengthen the Primary Care practice. Salary is gained from a combination of public and private incomes which are not differentiated. The role of the General Practitioner consists in the treatment of acute and chronic diseases, minor surgery, child care, etc. There is no coordination between Primary and Secondary care. Access to specialised medicine is regulated by the price of consultation. Primary Care doctors are not gatekeepers. To be able to work here, doctors must have three years of training after medical school. After that, Continuing Medical Education is compulsory, and the college of general practitioners monitors it annually. The Irish health care system does not fit into the European model. Lack of a clear separation between public and private health care generates great inequalities. The non-existence of coordination between primary and specialised care leads to inefficiencies, which Ireland cannot allow itself after a decade of economic crisis.

  18. Primary appendiceal mucinous adenocarcinoma.

    PubMed

    Behera, Prativa Kumari; Rath, Pramod Kumar; Panda, Rabiratna; Satpathi, Sanghamitra; Behera, Rajan

    2011-04-01

    Primary Adenocarcinomas of the appendix are extremely rare tumor. We report a case of primary mucinous adenocarcinoma in a 40 year old lady misdiagnosed as having acute appendicitis. All the routine investigations were within normal limit. USG of abdomen showed dilated appendix with little fluid collection adjacent to it and no other abnormality was seen which suggested acute appendicitis. Appendicectomy was done and excised appendix was sent for histopathological examination. Mucinous Adenocarcinoma of the appendix was confirmed after histopathological examination. Right hemicolectomy was done as a second stage procedure. As some cases are incidentally discovered, this case emphasizes that histological examination of all appendicectomy specimens is mandatory.

  19. Does primary fibromyalgia exist?

    PubMed

    Forslind, K; Fredriksson, E; Nived, O

    1990-10-01

    Twenty-one of 25 consecutive primary fibromyalgia or fibrositis patients, identified during a 5-year period in a tertiary care day-ward for pain syndromes, were re-examined. Fifteen fulfilled criteria for fibromyalgia but unexpectedly, all cases had either psychiatric disturbance or thyroid dysfunction. Of the four patients not seen at follow-up, two had developed neurological diseases, another rheumatoid arthritis and one other hypothyroidism. Thus, after 5 years no patient fulfilled the criteria for primary fibromyalgia. Women occupied as manual workers were over-represented. Most patients reported beneficial effects of physiotherapy. None of the patients has been able to return to full time work.

  20. Fibrositis and primary hypothyroidism.

    PubMed

    Carette, S; Lefrançois, L

    1988-09-01

    The prevalence of fibrositis was determined in 100 patients with subclinical or biochemical primary hypothyroidism. Nineteen patients reported symptoms of joint and/or muscle pain with stiffness. Five of these patients presented 7 or more tender points on examination, thus allowing a diagnosis of fibrositis to be made in only 5% of the total group. Symptomatic improvement after thyroid hormone replacement occurred in 10 of the 19 patients, including 3 of those with fibrositis. There were no significant changes in tender points. Our data indicate that fibrositis is uncommon in patients with primary hypothyroidism despite the frequent occurrence of symptoms suggestive of this syndrome.

  1. Melatonin for primary insomnia?

    PubMed

    2009-07-01

    Melatonin, a hormone produced by the pineal gland, has a key role in regulating circadian rhythms, most importantly, the sleep-wake cycle. Melatonin's action has led to its being tried as a treatment for a wide range of sleep disorders, such as jet lag, primary insomnia, sleep-wake cycle disruption and sleep problems in children with neuro-developmental disorders. Until recently, it had not been licensed in the UK for any indication. Prolonged-release melatonin (Circadin - Lundbeck) has now been licensed as a treatment for primary insomnia. Here we consider whether this product has a place in the management of people with this condition.

  2. Primary Creative Writing.

    ERIC Educational Resources Information Center

    Wooten, Vida Jo

    1968-01-01

    Children will enjoy creative writing in the primary grades if they are given inspiration, time to write, and the opportunity to share their work with classmates. A second-grade class began a creative writing project by listening to poetry and selecting poems to memorize and recite. This stimulated and encouraged them to evaluate and to write…

  3. Healthcare is primary.

    PubMed

    Kumar, Raman

    2015-01-01

    India is undergoing a rapid transformation in terms of governance, administrative reforms, newer policy develoment, and social movements. India is also considered one of the most vibrant economies in the world. The current discourse in public space is dominated by issues such as economic development, security, corruption free governance, gender equity, and women safety. Healthcare though remains a pressing need of population; seems to have taken a backseat. In the era of decreasing subsidies and cautious investment in social sectors, the 2(nd) National Conference on Family Medicine and Primary Care 2015 (FMPC) brought a focus on "healthcare" in India. The theme of this conference was "Healthcare is Primary." The conference participants discussed on the theme of why healthcare should be a national priority and why strong primary care should remain at the center of healthcare delivery system. The experts recommended that India needs to strengthen the "general health system" instead of focusing on disease based vertical programs. Public health system should have capacity and skill pool to be able to deliver person centered comprehensive health services to the community. Proactive implementation of policies towards human resource in health is the need of the hour. As the draft National Health Policy 2015 is being debated, "family medicine" (academic primary care), the unfinished agenda of National Health Policy 2002, remains a priority area of implementation.

  4. Beginning Primary Teaching

    ERIC Educational Resources Information Center

    Jacklin, Angela; Griffiths, Vivienne; Robinson, Carol

    2006-01-01

    This book supports primary teachers' early professional development and learning, tackling key questions and concerns that new teachers might face in their early careers, such as: How will I get through the first term? When will I feel like a "real" teacher? What can I expect from my first years in teaching? Drawing on the experiences of beginning…

  5. New Primary School Syllabus.

    ERIC Educational Resources Information Center

    Ministry of Education and Culture (Trinidad and Tobago).

    This official syllabus of Trinidad and Tobago's primary schools gives detailed guidelines on the teaching objectives of each curriculum area and how these can best be realized, as well as descriptions of the subject matter. The curriculum is divided into three levels: Level I (5- to 7-year-olds), Level II (7- to 9-year-olds) and Level III (10+- to…

  6. Primary diffuse leptomeningeal gliosarcomatosis.

    PubMed

    Moon, Ju Hyung; Kim, Se Hoon; Kim, Eui Hyun; Kang, Seok-Gu; Chang, Jong Hee

    2015-04-01

    Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition with a fatal outcome, characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. In particular, PDLG histologically diagnosed as gliosarcoma is extremely rare, with only 2 cases reported to date. We report a case of primary diffuse leptomeningeal gliosarcomatosis. A 68-year-old man presented with fever, chilling, headache, and a brief episode of mental deterioration. Initial T1-weighted post-contrast brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement without a definite intraparenchymal lesion. Based on clinical and imaging findings, antiviral treatment was initiated. Despite the treatment, the patient's neurologic symptoms and mental status progressively deteriorated and follow-up MRI showed rapid progression of the disease. A meningeal biopsy revealed gliosarcoma and was conclusive for the diagnosis of primary diffuse leptomeningeal gliosarcomatosis. We suggest the inclusion of PDLG in the potential differential diagnosis of patients who present with nonspecific neurologic symptoms in the presence of leptomeningeal involvement on MRI.

  7. Pediatric primary gastric lymphoma.

    PubMed

    Harris, G J; Laszewski, M J

    1992-04-01

    Primary gastric lymphoma in the pediatric population is rare. We have described a case of non-Hodgkin's lymphoma (Burkitt's type) manifested as a gastric mass. Despite its rarity in children, this tumor should be treated aggressively, since long-term survival has been reported.

  8. Primary Biliary Cirrhosis

    MedlinePlus

    ... of liver cancer every 6 to 12 months. Health care providers use blood tests, ultrasound, or both to check for signs of ... make the diagnosis of primary biliary cirrhosis. A health care provider uses the test selectively when he or she is concerned that ...

  9. Primary Standards Laboratory report

    SciTech Connect

    Not Available

    1990-12-01

    Sandia National Laboratories operates the Primary Standards Laboratory (PSL) for the Department of Energy, Albuquerque Operations Office (DOE/AL). This report summarizes metrology activities that received emphasis in the first half of 1990 and provides information pertinent to the operation of the DOE/AL system-wide Standards and Calibration Program.

  10. Primary Premier for Belfast

    ERIC Educational Resources Information Center

    McAlister, Peter

    2009-01-01

    The author talks about the Association for Science Education (ASE) Primary Science Committee's (PSC) March 2009 meeting which was held in Belfast as guests of ASE Northern Ireland. To mark the auspicious occasion of a body that usually meets four times a year in the Hatfield HQ crossing the Irish Sea to be hosted by its Celtic cousins, a Lord…

  11. From Primary to Secondary

    ERIC Educational Resources Information Center

    Anderson, Lyn

    2011-01-01

    This author discusses her decision to move to secondary school to teach mathematics, after having taught and been a mathematics manager in primary schools for six years. She states that this was a valuable experience and sparked her interest in the transition experiences of students, particularly in mathematics. Research (Evangelou et al, 2008,…

  12. Primary Art Resource Guide.

    ERIC Educational Resources Information Center

    Newton Unified School District 373, KS.

    GRADES OR AGES: Primary Grades. SUBJECT MATTER: Art. ORGANIZATION AND PHYSICAL APPEARANCE: The guide begins with a list of topics for art expression. The main body of the guide contains 15 color-coded sections on the following subjects: 1) mobiles and folded paper; 2) collage and photo montage; 3) square paper and mosaics; 4) wax paper and…

  13. Primary ectopic frontotemporal craniopharyngioma

    PubMed Central

    Ortega-Porcayo, Luis Alberto; Ponce-Gómez, Juan Antonio; Martínez-Moreno, Mauricio; Portocarrero-Ortíz, Lesly; Tena-Suck, Martha Lilia; Gómez-Amador, Juan Luis

    2015-01-01

    Introduction Primary ectopic craniopharyngiomas have only rarely been reported. Craniopharyngiomas involve usually the sellar and suprasellar region, but can be originated from cell remnants of the obliterated craniopharyngeal duct or metaplastic change of andenohypophyseal cells. We present the first case of a primary ectopic frontotemporal craniopharyngioma. Presentation of case A 35-year old woman presented with a one-year history of headache and diplopia. MRI showed a large frontotemporal cystic lesion. Tumor resection was performed with a keyhole endoscopic frontal lateral approach. The pathological features showed an adamantinomatous craniopharyngioma with a cholesterol granuloma reaction. Discussion There have been reported different localizations for primary ectopic craniopharyngioma. Our case presented a lobulated frontotemporal cystic mass formed by a dense eosinophilic proteinaceous material dystrophic calcifications and cholesterol crystals, with epithelial remnants. No tumor regrowth was observed in the magnetic resonance image 27 months postoperatively. Conclusion Primary ectopic craniopharyngioma is a rare entity with a pathogenesis that remains uncertain. This is an unusual anatomic location associated with unique clinical findings. PMID:25725331

  14. Philosophy in Primary Schools?

    ERIC Educational Resources Information Center

    White, John

    2012-01-01

    The article is a critical discussion of the aims behind the teaching of philosophy in British primary schools. It begins by reviewing the recent Special Issue of the "Journal of Philosophy of Education" Vol 45 Issue 2 2011 on "Philosophy for Children in Transition", so as to see what light this might throw on the topic just…

  15. Multiple Primary Cancer Monograph

    Cancer.gov

    To identify groups of cancer survivors that are at increased risk for multiple primary cancers, investigators led an effort to provide the first comprehensive population-based analysis of the risk of subsequent cancer in the U.S., resulting in a monograph.

  16. Restoring primary anterior teeth.

    PubMed

    Waggoner, William F

    2002-01-01

    A variety of esthetic restorative materials are available for restoring primary incisors. Knowledge of the specific strengths, weakness, and properties of each material will enhance the clinician's ability to make the best choice of selection for each individual situation. Intracoronal restorations of primary teeth may utilize resin composites, glass ionomer cements, resin-modified ionomers, or polyacid-modified resins. Each has distinct advantages and disadvantages and the clinical conditions of placement may be a strong determining factor as to which material is utilized. Full coronal restoration of primary incisors may be indicated for a number of reasons. Crowns available for restoration of primary incisors include those that are directly bonded onto the tooth, which generally are a resin material, and those crowns that are luted onto the tooth and are some type of stainless steel crown. However, due to lack of supporting clinical data, none of the crowns can be said to be superior to the others under all circumstances. Though caries in the mandibular region is rare, restorative solutions for mandibular incisors are needed. Neither stainless steel crowns nor celluloid crown forms are made specifically for mandibular incisors. Many options exist to repair carious primary incisors, but there is insufficient controlled, clinical data to suggest that one type of restoration is superior to another. This does not discount the fact that dentists have been using many of these crowns for years with much success. Operator preferences, esthetic demands by parents, the child's behavior, and moisture and hemorrhage control are all variables which affect the decision and ultimate outcome of whatever restorative treatment is chosen.

  17. Primary gastrointestinal lymphoma

    PubMed Central

    Aledavood, Amir; Nasiri, Mohammad Reza Ghavam; Memar, Bahram; Shahidsales, Soodabeh; Raziee, Hamid Reza; Ghafarzadegan, Kamran; Mohtashami, Samira

    2012-01-01

    Background: Extranodal lymphoma may arise anywhere outside lymph nodes mostly in the gastrointestinal (GI) tract as non-Hodgkin's disease. We reviewed the clinicopathological features and treatment results of patients with primary GI lymphoma. Materials and Methods: A total number of 30 cases with primary GI lymphoma were included in this study. Patients referred to the Radiation Oncology Department of Omid Hospital (Mashhad, Iran) during a 5-year period (2006-11). Clinical, paraclinical, and radiological data was collected from medical records of the patients. Results: Out of the 30 patients with primary GI lymphoma in the study, 12 were female (40%) and 18 were male (60%) (male to female ratio: 3/2). B symptoms were present in 27 patients (90%). Antidiuretic hormone (LDH) levels were elevated in 9 patients (32.1%). The most common primary site was stomach in 14 cases (46.7%). Other common sites included small intestine and colon each in 8 patients (26.7%). All patients had histopathologically proven non-Hodgkin's lymphoma. The most common histologic subtype was diffuse large B-cell lymphoma (DLBL) in 16 patients (53.3%). In addition, 28 patients (93.3%) received chemotherapy with cyclophosphamide, vincristine, doxorubicin, prednisolone (CHOP regimen). The median course of chemotherapy was 6 cources. Moreover, 8 patients (26.7%) received radiotherapy with cobalt 60. The median follow-up time was 26 months. The overall 5-year survival rate was 53% and the median survival time was 60 months. Conclusion: Primary GI lymphoma is commonly seen in stomach and small intestine and mostly is DLBCL or mucosa-associated lymphoid tissue (MALT) lymphoma. PMID:23626617

  18. Pyrosequencing-Based Assays for Rapid Detection of HER2 and HER3 Mutations in Clinical Samples Uncover an E332E Mutation Affecting HER3 in Retroperitoneal Leiomyosarcoma

    PubMed Central

    González-Alonso, Paula; Chamizo, Cristina; Moreno, Víctor; Madoz-Gúrpide, Juan; Carvajal, Nerea; Daoud, Lina; Zazo, Sandra; Martín-Aparicio, Ester; Cristóbal, Ion; Rincón, Raúl; García-Foncillas, Jesús; Rojo, Federico

    2015-01-01

    Mutations in Human Epidermal Growth Factor Receptors (HER) are associated with poor prognosis of several types of solid tumors. Although HER-mutation detection methods are currently available, such as Next-Generation Sequencing (NGS), alternative pyrosequencing allow the rapid characterization of specific mutations. We developed specific PCR-based pyrosequencing assays for identification of most prevalent HER2 and HER3 mutations, including S310F/Y, R678Q, L755M/P/S/W, V777A/L/M, 774-776 insertion, and V842I mutations in HER2, as well as M91I, V104M/L, D297N/V/Y, and E332E/K mutations in HER3. We tested 85 Formalin Fixed and Paraffin Embbeded (FFPE) samples and we detected three HER2-V842I mutations in colorectal carcinoma (CRC), ovarian carcinoma, and pancreatic carcinoma patients, respectively, and a HER2-L755M mutation in a CRC specimen. We also determined the presence of a HER3-E332K mutation in an urothelial carcinoma sample, and two HER3-D297Y mutations, in both gastric adenocarcinoma and CRC specimens. The D297Y mutation was previously detected in breast and gastric tumors, but not in CRC. Moreover, we found a not-previously-described HER3-E332E synonymous mutation in a retroperitoneal leiomyosarcoma patient. The pyrosequencing assays presented here allow the detection and characterization of specific HER2 and HER3 mutations. These pyrosequencing assays might be implemented in routine diagnosis for molecular characterization of HER2/HER3 receptors as an alternative to complex NGS approaches. PMID:26287187

  19. Pyrosequencing-Based Assays for Rapid Detection of HER2 and HER3 Mutations in Clinical Samples Uncover an E332E Mutation Affecting HER3 in Retroperitoneal Leiomyosarcoma.

    PubMed

    González-Alonso, Paula; Chamizo, Cristina; Moreno, Víctor; Madoz-Gúrpide, Juan; Carvajal, Nerea; Daoud, Lina; Zazo, Sandra; Martín-Aparicio, Ester; Cristóbal, Ion; Rincón, Raúl; García-Foncillas, Jesús; Rojo, Federico

    2015-08-17

    Mutations in Human Epidermal Growth Factor Receptors (HER) are associated with poor prognosis of several types of solid tumors. Although HER-mutation detection methods are currently available, such as Next-Generation Sequencing (NGS), alternative pyrosequencing allow the rapid characterization of specific mutations. We developed specific PCR-based pyrosequencing assays for identification of most prevalent HER2 and HER3 mutations, including S310F/Y, R678Q, L755M/P/S/W, V777A/L/M, 774-776 insertion, and V842I mutations in HER2, as well as M91I, V104M/L, D297N/V/Y, and E332E/K mutations in HER3. We tested 85 Formalin Fixed and Paraffin Embbeded (FFPE) samples and we detected three HER2-V842I mutations in colorectal carcinoma (CRC), ovarian carcinoma, and pancreatic carcinoma patients, respectively, and a HER2-L755M mutation in a CRC specimen. We also determined the presence of a HER3-E332K mutation in an urothelial carcinoma sample, and two HER3-D297Y mutations, in both gastric adenocarcinoma and CRC specimens. The D297Y mutation was previously detected in breast and gastric tumors, but not in CRC. Moreover, we found a not-previously-described HER3-E332E synonymous mutation in a retroperitoneal leiomyosarcoma patient. The pyrosequencing assays presented here allow the detection and characterization of specific HER2 and HER3 mutations. These pyrosequencing assays might be implemented in routine diagnosis for molecular characterization of HER2/HER3 receptors as an alternative to complex NGS approaches.

  20. Spirometry in primary care

    PubMed Central

    Coates, Allan L; Graham, Brian L; McFadden, Robin G; McParland, Colm; Moosa, Dilshad; Provencher, Steeve; Road, Jeremy

    2013-01-01

    Canadian Thoracic Society (CTS) clinical guidelines for asthma and chronic obstructive pulmonary disease (COPD) specify that spirometry should be used to diagnose these diseases. Given the burden of asthma and COPD, most people with these diseases will be diagnosed in the primary care setting. The present CTS position statement was developed to provide guidance on key factors affecting the quality of spirometry testing in the primary care setting. The present statement may also be used to inform and guide the accreditation process for spirometry in each province. Although many of the principles discussed are equally applicable to pulmonary function laboratories and interpretation of tests by respirologists, they are held to a higher standard and are outside the scope of the present statement. PMID:23457669

  1. [Basics of primary immunodeficiencies].

    PubMed

    Hernández-Martínez, Claudia; Espinosa-Rosales, Francisco; Espinosa-Padilla, Sara Elva; Hernández-Martínez, Ana Rosa; Blancas-Galicia, Lizbeth

    2016-01-01

    Primary immunodeficiencies (PID) are a heterogeneous group of inherited disorders, the etiology are the defects in the development or function of the immune system. The principal PID manifestations are the infections in early age, malignancy and diseases of immune dysregulation as autoimmunity and allergy. PIDs are genetics disorders and most of them are inherited as autosomal recessive, also this group of diseases is more prevalent in males and in childhood. The antibody immunodeficiency is the PID more common in adults. The more frequent disorders are the infections in the respiratory tract, abscesses, candidiasis, diarrhea, BCGosis etc. Initial approach included a complete blood count and quantification of immunoglobulins. The delay in diagnosis could be explained due to a perception that the recurrent infections are normal process or think that they are exclusively of childhood. The early diagnosis of PID by primary care physicians is important to opportune treatment and better prognosis.

  2. Primary pulmonary artery sarcoma.

    PubMed

    Jin, Tao; Zhang, Chong; Feng, Zhiying; Ni, Yiming

    2008-08-01

    Primary pulmonary artery sarcoma is an uncommon tumor. We report a case of a 73-year-old male patient with a two-week history of palpitations and shortness of breath, aggravated for two days and was believed to be pulmonary hypertension. Emergency heart ultrasound after admission presented a massive pulmonary embolism in the pulmonary artery. The patient's condition was successfully managed with urgent pulmonary artery embolectomy. The patient demonstrated improvement in hemodynamics after the operation. Histologic and immunohistochemical assays were performed and a diagnosis was made as primary pulmonary artery sarcoma arising from the left pulmonary artery. Resection of the tumor is recommended for the treatment of this rare malignant tumor. The corresponding chemotherapy, follow-up and prognosis are described as well in this case report.

  3. Primary hyperparathyroidism and nephrolithiasis.

    PubMed

    Vestergaard, Peter

    2015-05-01

    Calcifications in the kidneys may occur in the parenchyma (nephrocalcinosis), pelvis renis (nephrolithiasis) or ureters (ureterolithiasis). Several factors may protect against stone formation or promote precipitation of stones. Most stones contain calcium, and the hypercalciuria seen in primary hyperparathyroidism is a contributing factor to stone formation in the kidneys and urinary tract. In early case series, renal stone formation was frequent, whereas the proportion of patients with symptomatic renal stones has declined in recent years. However, a substantial proportion of patients presents with asymptomatic nephrocalcinosis or nephrolithiasis. Before diagnosis and treatment of primary hyperparathyroidism, renal stone events are more frequent than in the general population. However, even after surgical cure, an increased rate of renal stone events may be seen. This may to some extent be the result of stones or calcifications already present at the time of diagnosis or sequelae to prior stones such as infections or ureter strictures.

  4. [Primary orthostatic tremor].

    PubMed

    Bottin, P; Sadzot, B; Hotermans, C

    2005-02-01

    Primary orthostatic tremor is a particular tremor exclusively present when a subject is standing. Patients experience a severe disabling sense of unsteadiness. Walking, sitting and lying down are unaffected Neurological examination and cerebral imagery are normal most of the time. Electromyography in standing position confirms the diagnosis in showing a regular rapid tremor with a frequency of 12 to 18 Hz. Its physiopathology is partially unknown. A few symptomatic therapies can be proposed.

  5. [Primary care in France].

    PubMed

    Sánchez-Sagrado, T

    2016-01-01

    The poor planning of health care professionals in Spain has led to an exodus of doctors leaving the country. France is one of the chosen countries for Spanish doctors to develop their professional career. The French health care system belongs to the Bismarck model. In this model, health care system is financed jointly by workers and employers through payroll deduction. The right to health care is linked to the job, and provision of services is done by sickness-funds controlled by the Government. Primary care in France is quite different from Spanish primary care. General practitioners are independent workers who have the right to set up a practice anywhere in France. This lack of regulation has generated a great problem of "medical desertification" with problems of health care access and inequalities in health. French doctors do not want to work in rural areas or outside cities because "they are not value for money". Medical salary is linked to professional activity. The role of doctors is to give punctual care. Team work team does not exist, and coordination between primary and secondary care is lacking. Access to diagnostic tests, hospitals and specialists is unlimited. Duplicity of services, adverse events and inefficiencies are the norm. Patients can freely choose their doctor, and they have a co-payment for visits and hospital care settings. Two years training is required to become a general practitioner. After that, continuing medical education is compulsory, but it is not regulated. Although the French medical Health System was named by the WHO in 2000 as the best health care system in the world, is it not that good. While primary care in Spain has room for improvement, there is a long way for France to be like Spain.

  6. [Multiple primary pulmonary carcinomas].

    PubMed

    Guitart, A C; Gómez, G; Estrada, G; Rodríguez, C; León, C; Cornudella, R

    1991-02-01

    Three cases of multiple simultaneous primary lung carcinomas are presented, in which diagnosis was established by post-surgery pathological exam. In all three cases, chest X-ray showed pulmonary masses suggestive or clinical malignancy, and pre-surgery pathological diagnosis or squamous lung carcinoma. During thoracotomy or in the resected segment, a second lesion we confirmed which made resection necessary being this second lesion classified as lung adenocarcinoma.

  7. [Primary care in Sweden].

    PubMed

    Sánchez-Sagrado, T

    2016-09-01

    Sweden was one of the first European Union countries that saw the opportunity in the free movement of professionals. First offers for jobs were managed in 2000. Since then, a large number of professionals have taken the opportunity of a decent job and have moved from Spain to Sweden. The Swedish health care model belongs to the group of national health systems. The right to health care is linked to legal citizenship. Health is financed through regional taxes, but there is a compulsory co-payment regardless of the financial situation of the patient. The provision of health care is decentralised at a regional level, and there is a mixture of private and public medical centres. Primary care is similar to that in Spain. Health professionals work as a team with a division of tasks. Like in Spain, waiting lists and coordination between primary and specialised care are a great problem. Patients may register with any public or private primary care centre and hospital provider within their region. Access to diagnostic tests and specialists are restricted to those selected by specialists. Doctors are salaried and their job and salary depend on their experience, professional abilities and regional needs. Medicine is curative. General practitioners are the gateway to the system, but they do not act as gatekeeper. Hospitals offer a number of training post, and the access is through an interview. Continuing medical education is encouraged and financed by the health centre in order to increase its revenues.

  8. Primary gastric lymphoma

    PubMed Central

    Al-Akwaa, Ahmad M; Siddiqui, Neelam; Al-Mofleh, Ibrahim A

    2004-01-01

    AIM: The purpose of this review is to describe the various aspects of primary gastric lymphoma and the treatment options currently available. METHODS: After a systematic search of Pubmed, Medscape and MDconsult, we reviewed and retrieved literature regarding gastric lymphoma. RESULTS: Primary gastric lymphoma is rare however, the incidence of this malignancy is increasing. Chronic gastritis secondary to Helicobacter pylori (H pylori) infection has been considered a major predisposing factor for MALT lymphoma. Immune histochemical marker studies and molecular biology utilizing polymerase chain reaction have facilitated appropriate diagnosis and abolished the need for diagnostic surgical resection. Advances in imaging techniques including Magnetic Resonance Imaging (MRI) and Endoscopic Ultrasonography (EUS) have helped evaluation of tumor extension and invasion. The clinical course and prognosis of this disease is dependent on histopathological sub-type and stage at the time of diagnosis. Controversy remains regarding the best treatment for early stages of this disease. Chemotherapy, surgery and combination have been studied and shared almost comparable results with survival rate of 70%-90%. However, chemotherapy possesses the advantage of preserving gastric anatomy. Radiotherapy alone has been tried and showed good results. Stage IIIE, IVE disease treatment is solely by chemotherapy and surgical resection has been a remote consideration. CONCLUSION: We conclude that methods of diagnosis and staging of the primary gastric lymphoma have dramatically improved. The modalities of treatment are many and probably chemotherapy is superior because of high success rate, preservation of stomach and tolerable complications. PMID:14695759

  9. Primary stabbing headache.

    PubMed

    Pareja, Juan A; Sjaastad, Ottar

    2010-01-01

    Primary stabbing headache is characterized by transient, cephalic ultrashort stabs of pain. It is a frequent complaint with a prevalence of 35.2%, a female preponderance, and a mean age of onset of 28 years (Vågå study). Attacks are generally characterized by moderate to severe, jabbing or stabbing pain, lasting from a fraction of a second to 3s. Attack frequency is generally low, with one or a few attacks per day. The paroxysms generally occur spontaneously, during daytime. Most patients exhibit a sporadic pattern, with an erratic, unpredictable alternation between symptomatic and non-symptomatic periods. Paroxysms are almost invariably unilateral. Temporal and fronto-ocular areas are most frequently affected. Attacks tend to move from one area to another, in either the same or the opposite hemicranium. Jabs may be accompanied by a shock-like feeling and even by head movement - "jolts" -or vocalization. On rare occasions, conjunctival hemorrhage and monocular vision loss have been described as associated features. Primary stabbing headache may concur, synchronously or independently, with other primary headaches. In contrast to what is the case in adults, in childhood it is not usually associated with other headaches. Treatment is rarely necessary. Indomethacin, 75-150 mg daily, may seem to be of some avail. Celecoxib, nifedipine, melatonin, and gabapentin have been reported to be effective in isolated cases and small series of patients. The drug studies need corroboration.

  10. Achieving Value in Primary Care: The Primary Care Value Model.

    PubMed

    Rollow, William; Cucchiara, Peter

    2016-03-01

    The patient-centered medical home (PCMH) model provides a compelling vision for primary care transformation, but studies of its impact have used insufficiently patient-centered metrics with inconsistent results. We propose a framework for defining patient-centered value and a new model for value-based primary care transformation: the primary care value model (PCVM). We advocate for use of patient-centered value when measuring the impact of primary care transformation, recognition, and performance-based payment; for financial support and research and development to better define primary care value-creating activities and their implementation; and for use of the model to support primary care organizations in transformation.

  11. Treatment of Primary Hyperoxaluria

    PubMed Central

    Dent, C. E.; Stamp, T. C. B.

    1970-01-01

    Nine patients with primary hyperoxaluria have been followed regularly for 1 to 11 years, and their treatment and progress are discussed in relation to the known natural history of the disease. 6 of them probably have the usual form of primary hyperoxaluria associated with increased glycollic acid excretion, while 3 who are sibs have the recently described variant associated with L-glyceric aciduria and normal glycollic acid excretion. All 9 patients have been on regimens designed to increase the urinary solubility of calcium oxalate, with or without the simultaneous lowering of urinary calcium and raising of urinary phosphate excretions. 8 patients have been treated for 1½-7½ years (average duration 4 years) with oral magnesium hydroxide, and 2 patients have been treated with sodium phosphate. One of the latter was changed after 3½ years to magnesium hydroxide and the other has been on sodium phosphate combined with a low calcium diet and cellulose phosphate continuously for 5½ years. 2, not at first diagnosed as hyperoxalurics, were first given sodium bicarbonate for their presumably secondary renal tubular acidosis. The over-all progress of the whole group is felt to have been better than could be expected from the known natural history of primary hyperoxaluria. They average 4¼ years on treatment during 5 years of our observation and all remain clinically well after an average of 9½ years since the onset of their first symptoms. Results warrant the recommendation that, until reliable means are available to decrease oxalate over-production, affected patients should be treated continuously with magnesium hydroxide. A more final opinion must await many more years of follow-up. The failure of several attempts to lower urinary oxalate excretion in these patients is also reported. ImagesFIG. 2FIG. 3FIG. 4FIG. 5 PMID:5491877

  12. Epigenetics and primary care.

    PubMed

    Wright, Robert; Saul, Robert A

    2013-12-01

    Epigenetics, the study of functionally relevant chemical modifications to DNA that do not involve a change in the DNA nucleotide sequence, is at the interface between research and clinical medicine. Research on epigenetic marks, which regulate gene expression independently of the underlying genetic code, has dramatically changed our understanding of the interplay between genes and the environment. This interplay alters human biology and developmental trajectories, and can lead to programmed human disease years after the environmental exposure. In addition, epigenetic marks are potentially heritable. In this article, we discuss the underlying concepts of epigenetics and address its current and potential applicability for primary care providers.

  13. Primary leptomeningeal melanoma.

    PubMed

    Xie, Zhao-Yu; Hsieh, Kevin Li-Chun; Tsang, Yuk-Ming; Cheung, Wing-Keung; Hsieh, Chen-Hsi

    2014-06-01

    Primary melanoma of the central nervous system is a rare melanocytic tumor typically located in the leptomeninges. We report a 57-year-old woman with an intracranial leptomeningeal melanoma who presented with myoclonic seizures. Brain CT scan and MRI revealed a hemorrhagic intracranial tumor. The tumor was completely removed and leptomeningeal melanoma was proven pathologically. Follow-up imaging studies up to 19 months showed no recurrence of the disease. Here we present radiological, gross, and pathological images of leptomeningeal melanoma, discuss its characteristics, and review the relevant literature.

  14. Primary amoebic (Naegleria) meningoencephalitis.

    PubMed

    Lam, A H; de Silva, M; Procopis, P; Kan, A

    1982-06-01

    The computed tomographic (CT) appearance of a case of primary meningoencephalitis due to the free living amoeba Naegleria fowleri is presented. The cisterns around and above the midbrain and the subarachnoid spaces were obliterated on precontrast CT. Marked enhancement in these regions was seen after intravenous contrast medium administration. The sulci and adjacent grey matter were also strongly enhanced. The ventricular size was normal. Pathological findings were those of arachnoiditis and invasion of the leptomeninges and brain substance by amoebae, especially at the base of the brain and cerebellum.

  15. Primary Ciliary Dyskinesia.

    PubMed

    Knowles, Michael R; Zariwala, Maimoona; Leigh, Margaret

    2016-09-01

    Primary ciliary dyskinesia (PCD) is a recessive genetically heterogeneous disorder of motile cilia with chronic otosinopulmonary disease and organ laterality defects in ∼50% of cases. The prevalence of PCD is difficult to determine. Recent diagnostic advances through measurement of nasal nitric oxide and genetic testing has allowed rigorous diagnoses and determination of a robust clinical phenotype, which includes neonatal respiratory distress, daily nasal congestion, and wet cough starting early in life, along with organ laterality defects. There is early onset of lung disease in PCD with abnormal airflow mechanics and radiographic abnormalities detected in infancy and early childhood.

  16. [Primary pancreatic plasmacytoma].

    PubMed

    Sánchez Acevedo, Z; Pomares Rey, B; Alpera Tenza, M R; Andrada Becerra, E

    2014-01-01

    Extramedullary plasmacytomas are uncommon malignant plasma cell tumors that present outside the bone marrow; 80% of extramedullary plasmacytomas are located in the upper respiratory tract, and gastrointestinal plasmacytomas are rare. We present the case of an asymptomatic 65-year-old man in whom a pancreatic mass was found incidentally. The lesion was determined to be a pancreatic plasmacytoma after fine-needle aspiration cytology and surgical resection. No clinical, laboratory, or imaging findings indicative of multiple myeloma or association with other plasmacytomas were found, so the tumor was considered to be a primary pancreatic plasmacytoma.

  17. [Primary esophageal lymphoma].

    PubMed

    Ximenes, Manoel; Piauilino, Marcos Amorim; Oliveira, Humberto Alves; Vaz Neto, Jorge Pinto

    2012-01-01

    We describe the case of a 54 year old woman seen with an esophageal mass diagnosed as a primary esophageal lymphoma. The main symptom was dysphagia of seven months duration. The treatment consisted in resection of the tumor, and reconstruction of the defect with a reversed pleural flap, followed by a chemotherapy regimen that consisted of five drugs, cyclophosphamid, prednisone, doxorubicin, rituximab and vincristine (R-CHOP). The patient developed an esophageal pleural fistula treated with pleural drainage and irrigation that closed in 45 days. Two and one half years later she is doing well and disease free.

  18. Primary testicular lymphoma.

    PubMed Central

    Vural, Filiz; Cagirgan, Seckin; Saydam, Guray; Hekimgil, Mine; Soyer, Nur Akad; Tombuloglu, Murat

    2007-01-01

    We evaluated clinical features, management and survival of 12 patients with primary testicular non-Hodgkin's lymphoma presented to our hematology unit between January 1992 and July 2006, retrospectively. The median age of patients was 47 years at presentation (range 29-78 years) and > 80% of them were < 50 years old. In the majority of cases, orchidectomy was performed as diagnostic and first-line therapeutic procedures. Dominant histological subtype was diffuse large B-cell non-Hodgkin's lymphoma. Seven patients out of 12 (58%) were Ann Arbor stages I and II, and the remaining five patients (42%) were stages III and IV. All the patients received doxorubicin-based chemotherapy and achieved complete remission. The addition of rituximab and central nervous system prophylaxis with intrathecal combined chemotherapy containing methotrexate, cytarabine and dexametasone were applied to three patients who were recently admitted. The rate of relapse was 8% and progression-free survival (PFS) at 10 years was 88%. Median duration of response was 84 months (range 14-173 months), median 97.5 months of follow-up. All patients are alive and in case remission. Because of the spreading nature and relapse probability at different sites, including central nervous system and contralateral testis, systemic treatment with doxorubicin-based chemotherapy with or without prophylaxis for contralateral testis and the central nervous system seems to improve the outcome of primary testicular lymphoma. PMID:18020104

  19. Update on primary hypobetalipoproteinemia.

    PubMed

    Hooper, Amanda J; Burnett, John R

    2014-07-01

    "Primary hypobetalipoproteinemia" refers to an eclectic group of inherited lipoprotein disorders characterized by low concentrations of or absence of low-density lipoprotein cholesterol and apolipoprotein B in plasma. Abetalipoproteinemia and homozygous familial hypobetalipoproteinemia, although caused by mutations in different genes, are clinically indistinguishable. A framework for the clinical follow-up and management of these two disorders has been proposed recently, focusing on monitoring of growth in children and preventing complications by providing specialized dietary advice and fat-soluble vitamin therapeutic regimens. Other recent publications on familial combined hypolipidemia suggest that although a reduction of angiopoietin-like 3 activity may improve insulin sensitivity, complete deficiency also reduces serum cholesterol efflux capacity and increases the risk of early vascular atherosclerotic changes, despite low low-density lipoprotein cholesterol levels. Specialist laboratories offer exon-by-exon sequence analysis for the molecular diagnosis of primary hypobetalipoproteinemia. In the future, massively parallel sequencing of panels of genes involved in dyslipidemia may play a greater role in the diagnosis of these conditions.

  20. Primary pulmonary choriocarcinoma

    PubMed Central

    Snoj, Ziga; Kocijancic, Igor

    2017-01-01

    Abstract Background The aim of the study was to establish whether there are different clinical entities of primary pulmonary choriocarcinoma (PPC) that deserve different diagnostic approach and the most optimal treatment. Patients and methods A systematic review with PubMed search was conducted to identify studies that reported cases of PPC. The eligibility criteria were histological diagnosis of pulmonary choriocarcinoma and thorough examination of the reproductive organs to exclude potential primary choriocarcinoma in the gonads. Furthermore, to illustrate the review we additionally present a patient referred at our institution. Results 55 cases (17 men) were included in the review with a median age of 34 years. Women with the history of gestational event showed better survival outcome than women without the history of gestational event. Patients treated with combined modality treatment (surgery and chemotherapy) survived longer than the patients without combined modality treatment. Furthermore, multivariate analysis of prognostic factors showed that the combined modality treatment had independent prognostic significance. Size of the tumour showed significant prognostic influence in univariate and multivariate analysis. Conclusions PPC is an extreme rarity with variable clinical characteristics and outcome. It is important to capture and treat patients in the early stages of the disease. Women with the history of gestational event may show better survival, therefore genetic examination could help us to predict patient’s prognosis. Surgery followed by adjuvant chemotherapy appears to represent the best treatment for PPC. PMID:28265226