Sample records for hemosiderotic fibrohistiocytic lipomatous

  1. Hemosiderotic fibrohistiocytic lipomatous lesion: clinical correlation with venous stasis.

    PubMed

    Kazakov, Dmitry V; Sima, Radek; Michal, Michal

    2005-07-01

    Hemosiderotic fibrohistiocytic lipomatous lesion (HFLL) is a recently proposed lipomatous entity. HFLL was originally suggested to be a benign reactive lesion arising due to an antecedent trauma. We report two patients with HFLL who also suffered from chronic vein insufficiency due to varicose involving deep veins of the low limbs. Both patients were middle-aged women with solitary, poorly circumscribed subcutaneous lesions on the lower extremities. Histopathological examination revealed typical features of HFLL. We think that the consistent clinical features such as advanced age, female sex predilection, and specific location along with distinctive histopathological features allow the suggestion that impaired blood circulation, to wit, venous stasis is involved in the pathogenesis of HFLL. We hypothesize that the proliferation of spindled fibroblastic and myofibroblastic cells and capillaries, erythrocyte extravasation, and hemosiderin deposition with lipomatous tissue of HFLL may simply represent an exaggerated tissue response to venous stasis in which elevated venous and capillary pressures, oxygen saturation, and edema stimulate the proliferation of the above mentioned elements and lead to erythrocyte extravasation. A similar histopathological pattern is seen in acroangiodermatitis of Mali and vascular transformation of lymph node sinuses, and these conditions are also associated with impaired blood circulation. PMID:15947948

  2. Mitotically Active Plexiform Fibrohistiocytic Tumor

    PubMed Central

    Zemheri, Ebru; Özkanl?, ?eyma; ?enol, Serkan; Ozen, Filiz; Ulukaya Durakba?a, Cigdem; Zindanc?, ?lkin; Okur, Hamit

    2013-01-01

    Plexiform fibrohistiocytic tumor is an intermediate malignant tumor situated in superficial soft tissues. It affects children and young adults. The tumor is most commonly located on upper extremities, whereas involvement of back region is rare. Mitotic activity is generally low (~3/10 HPF). It is rare, but it can exhibit aggressive behavior, so total excision with clear surgical margins and long-term followup is necessary to detect local recurrence and metastases. We report a child with a solid mass on back region which was found to be a mitotically active plexiform fibrohistiocytic tumor (6/10 HPF) after excision. Plexiform fibrohistiocytic tumor (PFT) is a mesenchymal neoplasm of children, adolescents, and young adults. It is characterized by fibrohistiocytic cytomorphology and multinodular growth pattern. Clinically it is usually a slow-growing mass of upper extremities with frequent local recurrence and rare regional lymphatic and systemic metastasis (Fletcher et al. (2002), Enzinger and Zhang (1988), Remstein et al. (1999)). PMID:23607025

  3. Lipomatous Pseudohypertrophy of the Pancreas

    PubMed Central

    Altinel, Deniz; Basturk, Olca; Sarmiento, Juan M.; Martin, Diego; Jacobs, Michael J.; Kooby, David A.; Adsay, N. Volkan

    2011-01-01

    Objectives Owing to the challenges in obtaining pancreatic biopsies, pancreatic resection for presumed malignancy is often performed without histological confirmation. As a result, benign lesions are sometimes surgically removed. One such condition, which is poorly defined in the literature, is referred to as lipomatous pseudohypertrophy (LPH) of the pancreas. Methods Five cases of LPH were analyzed. Results Four patients underwent surgical resection, 3 of which were diagnosed preoperatively by radiology as having ductal adenocarcinoma. The fourth case was correctly interpreted by magnetic resonance imaging as LPH, but the patient underwent resection because of the intractable pain due to pancreatitis. The fifth patient has been placed on watchful waiting. Two tumors were in the pancreatic head, one in the tail, one in the uncinate process, and one demonstrated diffuse involvement. Microscopically, they were characterized as having normal lipocytes without lipoblasts or inflammation. Within the adipose tissue, scattered microscopic foci of pancreatic parenchyma could be seen. Conclusion Lipomatous pseudohypertrophy of the pancreas is a distinct entity characterized by localized/diffuse replacement of pancreatic parenchyma with mature adipose tissue. It forms a pseudotumor that may be difficult to distinguish clinically from pancreatic adenocarcinoma. This entity should be considered when evaluating patients with a new diagnosis of a hypodense pancreatic neoplasm on imaging. PMID:19904221

  4. Fibrohistiocytic tumors and related neoplasms in children and adolescents.

    PubMed

    Black, Jennifer; Coffin, Cheryl M; Dehner, Louis P

    2012-01-01

    Fibrohistiocytic tumors (FHTs) in children and adolescents range from the benign fibrous histiocytoma, or dermatofibroma, to a variety of intermediate and malignant neoplasms, such as dermatofibrosarcoma protruberans and high-grade undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). Those tumors as a group are comprised of fibroblasts, myofibroblasts, and histiocytes-dendritic cells with a variably prominent inflammatory infiltrate consisting of lymphocytes and eosinophils. Dendritic cells are also a major constituent of another group of neoplasms that include Langerhans cell histiocytosis, follicular and interdigitating cell sarcomas, and juvenile xanthogranuloma. These latter tumors are considered in this discussion for the sake of differential diagnosis and their possible histogenetic relationship to FHTs. Recent studies have suggested that the relationship between the fibroblast and histiocyte in the FHTs may reflect the intrinsic capacity to transdifferentiate from one to the other morphologic and functional state. The so-called "facultative fibroblast," as a cell with fibroblastic and histiocytic properties, was discussed in the context of the fibrous xanthoma 50 years ago. Possibly the entire histogenetic concept of FHTs should be reconsidered in light of current studies. PMID:22420728

  5. Large Lipomatous Hypertrophy of the Interventricular Septum

    PubMed Central

    Ak, Koray; Isbir, Selim; Kepez, Alper; Turkoz, Kemal; Elci, Emre; Arsan, Sinan

    2014-01-01

    We present the case of a 58-year-old woman who had large lipomatous hypertrophy of the interventricular septum, a condition that is reported very infrequently. Preoperative cardiac magnetic resonance images revealed an inhomogeneous, infiltrating mass that was suppressed in fat-suppression mode. The extensive mass was causing right ventricular dysfunction, so we excised it through a right ventricular approach. The findings on histologic analysis of the mass were consistent with lipomatous hypertrophy. The patient died of septic shock on the 28th postoperative day. In addition to the patient's case, we discuss the characteristics and diagnosis of this rare entity. PMID:24808791

  6. Large lipomatous hypertrophy of the interventricular septum.

    PubMed

    Ak, Koray; Isbir, Selim; Kepez, Alper; Turkoz, Kemal; Elci, Emre; Arsan, Sinan

    2014-04-01

    We present the case of a 58-year-old woman who had large lipomatous hypertrophy of the interventricular septum, a condition that is reported very infrequently. Preoperative cardiac magnetic resonance images revealed an inhomogeneous, infiltrating mass that was suppressed in fat-suppression mode. The extensive mass was causing right ventricular dysfunction, so we excised it through a right ventricular approach. The findings on histologic analysis of the mass were consistent with lipomatous hypertrophy. The patient died of septic shock on the 28th postoperative day. In addition to the patient's case, we discuss the characteristics and diagnosis of this rare entity. PMID:24808791

  7. Huge lipomatous pseudohypertrophy of the pancreas.

    PubMed

    Nakamura, M; Katada, N; Sakakibara, A; Okumura, N; Kato, E; Takeichi, M; Kondo, T; Mano, H

    1979-08-01

    A rare case of lipomatous pseudohypertrophy of the pancreas associated with multiple cysts in an adult is presented. Endoscopic retrograde pancreatography showed a long, willow-like main pancreatic duct. Resected body and tail of the pancreas weighed 1,200 gm. PMID:474558

  8. [Cholestatic jaundice complicating lipomatous pseudo-hypertrophy of the pancreas].

    PubMed

    Henkinbrant, A; Khalek, W; Farchakh, E; Henry, M

    1990-01-01

    The authors describe a major pancreatic lipomatous infiltration, causing a "pseudo-hypertrophy" of the pancreas, in a 70-year-old man. This lesion was responsible of a hyperechogenic area at ultrasonography and of an empty pancreatic bed ("vanishing pancreas") at computed tomography, suggesting lipomatosis. This entity is a special modality of senescence of pancreatic tissue whose origin remains obscure. The progressive atrophy of the acinar lobules and the islets of Langerhans was finally responsible of a mild degree of malabsorption and of diabetes type II. The compression of the main bile duct by the enlarged lipomatous pancreas, demonstrated by percutaneous cholangiography, caused a cholestatic jaundice with abdominal pain, which was treated by a surgical derivation (hepatico-duodenostomy). This is the first description of a lipomatous pseudohypertrophy of the pancreas causing an obstruction of the common bile duct. PMID:2077797

  9. Lipomatous hypertrophy presenting as superior vena cava syndrome.

    PubMed

    Cheezum, Michael K; Jezior, Matthew R; Carbonaro, Salvatore; Villines, Todd C

    2014-01-01

    We report a rare case of invasive lipomatous hypertrophy of the interatrial septum presenting as superior vena cava syndrome requiring surgical resection. Lipomatous hypertrophy of the interatrial septum is a common incidental finding and is thought to be a relatively benign condition. Classically, its appearance on cardiac computed tomography is primarily fatty with low attenuation, sharp margins and minimal enhancement, involving the interatrial septum with relative sparing of the fossa ovalis. In masses of unclear etiology or in rare, extensive cases resulting in cavity obstruction, such as ours, cardiac computed tomography provides complementary information to define tumor extent and characterize classic mass features facilitating diagnosis and management. PMID:24939076

  10. Rare Lipomatous Tumor of the Posterior Mediastinum in Children

    PubMed Central

    Homma, Takahiro; Doki, Yoshinori; Senda, Kazutaka; Toge, Masayoshi; Yamamoto, Yutaka; Ojima, Toshihiro; Shimada, Yoshifumi; Yoshimura, Naoki

    2014-01-01

    The mediastinum is a unique anatomic area containing various structures and pluripotent cells, which allow for the development of a range of tumors. We report two pediatric cases of a lipomatous tumor of the posterior mediastinum. Complete surgical excision of the mass in each was achieved through a lateral thoracotomy. Histopathologic findings showed a lipoma in one case and a lipoblastoma in the other. A lipomatous tumor in the posterior mediastinum is extremely rare and preoperative diagnosis is difficult. When an operative plan is being considered, the presence of a potentially malignant tumor should be kept in mind. PMID:25755971

  11. Atypical lipomatous tumor of the cheek - a case report.

    PubMed

    Kaczmarczyk, Dariusz; Jesionek-Kupnicka, Dorota; Kubiak, Marcin; Morawiec-Sztandera, Alina

    2013-01-01

    Liposarcoma is the most common soft tissue malignant tumor. It mostly arises in the subcutaneous tissue of shoulders, limbs and neck, and retroperitoneal space, but head occurrence is very rare. Atypical lipomatous tumor (ALT) is a well-differentiated liposarcoma (WDLPS) and constitutes 40-45% of all liposarcoma cases. A case of 57-year-old woman with a tumor of the left cheek, causing a discreet face asymmetry is presented. The tumor was soft and caused no tenderness. The patient reported no previous injury of the region. Fine-needle aspiration biopsy (FNAB) revealed atypical cells suspected of liposarcoma. MR examination showed fascicles of adipose tissue, which made the left cheek prominent. The patient was operated under general anaesthesia. Adipose tissue of the left cheek was removed. Postoperative course was uneventful. The final histopathological diagnosis - was atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS). The patient remains under laryngological care. No recurrence of the disease has been observed during the 5 years follow-up. PMID:23911052

  12. Lipomatous pseudohypertrophy of the pancreas: further evidence of advanced hepatic lesion as the pathogenesis.

    PubMed

    Kuroda, Naoto; Okada, Mitsuo; Toi, Makoto; Hiroi, Makoto; Enzan, Hideaki

    2003-02-01

    An 80-year-old Japanese man with liver cirrhosis was suspected of having lipomatous pseudohypertrophy of the pancreas on the basis of results of ultrasonography and a computed tomography scan. He eventually died of hepatic failure. He had no obesity, diabetes mellitus or pancreatic symptoms during his entire clinical course. Autopsy results confirmed lipomatous pseudohypertrophy of the pancreas, cholecystolithiasis, and postnecrotic liver cirrhosis associated with submassive hepatic necrosis was suspected. Although the pathogenesis of lipomatous pseudohypertrophy of the pancreas is not clear, the findings in the present case provide further evidence to support the hypothesis that advanced hepatic lesions cause this lesion. PMID:12588437

  13. Squamous cell carcinoma and lipomatous pseudohypertrophy of the pancreas.

    PubMed

    Bralet, M P; Terris, B; Brégeaud, L; Ruszniewski, P; Bernades, P; Belghiti, J; Fléjou, J F

    1999-06-01

    A 68-year-old woman who had been treated for non-insulin-dependent diabetes mellitus for the past 20 years was admitted to hospital because of abdominal pain and weight loss. Radiological investigation revealed a tumour in the body of the pancreas and numerous intraductal calcifications in both the tail and the head of the pancreas. Left-sided pancreatectomy was performed to remove the tumour. The resection specimen showed fatty enlargement of the parenchyma and numerous intraductal calcifications in the tissue adjacent to the tumour, which was 7 cm in diameter and was found to be a primary squamous cell carcinoma with a spindle cell component. There was also lipomatous pseudohypertrophy. PMID:10394894

  14. Lipomatous pseudohypertrophy of the pancreas in a patient with cirrhosis due to chronic hepatitis B.

    PubMed

    Sasaki, M; Nakanuma, Y; Ando, H

    1998-07-01

    Lipomatous pseudohypertrophy of the pancreas was found at autopsy in a 52-year-old Japanese woman with cirrhosis due to chronic hepatitis B. Clinically, there were no clear symptoms of pancreatic insufficiency during the entire course. Marked atrophy and fat deposition of the pancreas had already been detected by computed tomography (CT) at least 6 years before her death. She died of hepatic failure due to decompensated cirrhosis. Autopsy revealed uniform enlargement of the pancreas due to massive fat replacement (lipomatous pseudohypertrophy): the exocrine glandular elements showed marked atrophy and loss, while the islets of Langerhans were preserved. Although the etiology and pathogenesis of lipomatous pseudohypertrophy is still unclear, this case suggests that this condition is causally related to chronic hepatitis B or other chronic advanced hepatic lesions. PMID:9701022

  15. A case report of "lipomatous hypertrophy of the cardiac interatrial septum", with a proposal for a new term "lipomatous hamartoma of the cardiac atrial septum".

    PubMed

    Inoue, T; Mohri, N; Nagahara, T; Takanashi, R

    1988-12-01

    An autopsy case of "lipomatous hypertrophy of the cardiac interatrial septum" (LHIS) combined with marked stenosis and calcification of the aortic and mitral valves due to fibrous valvular endocarditis, malignant lymphoma, and mucin-producing cancer of the pancreas is reported. LHIS and mucin-producing cancer of the pancreas were incidental findings at autopsy. Microscopically, the LHIS in this case consisted of proliferation of mature fat cells, brown fat cells and cardiac muscle cells. Since these three cell types are normal structural components of the interatrial septum, it was considered that the LHIS was a hamartomatous rather than hypertrophic lesion, of the cardiac atrial septum. Accordingly it was concluded that the term "lipomatous hamartoma of the cardiac atrial septum" was more appropriate for this lesion in the case presented here. As far as we know, this is the first reported case of LHIS in Japan. PMID:2467515

  16. Lipomatous hemangiopericytoma of the head and neck: immunohistochemical and dna ploidy analyses

    Microsoft Academic Search

    Sadir J. Alrawi; George Deeb; Richard Cheney; Paul Wallace; Thom Loree; Nestor Rigual; Wesley Hicks; Dongfeng Tan

    2004-01-01

    Background. Lipomatous hemangiopericytoma (LHPC) is a newly described rare soft tissue tumor with unpredictable biologic behavior and is difficult to diagnose by conventional histologic parameters. The molecular analyses of thisentitytodatearesparse.OnlyafewcasesofLHPChavebeen reported. Although one case of LHPC in the sinonasal region was briefly reported, this is the first case in the head and neck region with detailed clinicopathologic features and molecular

  17. [Pancreatic lipomatous hypoplasia associated with skeleton anomalies. Shwachman's syndrome (author's transl)].

    PubMed

    Guerrero, J; Gamallo, C; Agustí, B; Segurado, E; Vázquez, C

    1978-10-01

    A five month old girl with exocrine pancreatic insufficiency, skeletal deformities and a non constant neutropenia (Shwachman's syndrome) is presented. Infectious complications, mainly septic and pulmonary, which caused her death, were frequent. The pathologic findings showed a pancreatic lipomatous hypoplasia and absence of zymogen granulos, as well as anomalies of the growing cartilage where no radiological abnormalities could be seen. PMID:727604

  18. Lipomatous myofibroblastoma of the breast: case report with diagnostic and histogenetic considerations.

    PubMed

    Magro, G; Longo, F R; Salvatorelli, L; Vasquez, E; Vecchio, G M

    2014-06-01

    We report rare case of myofibroblastoma (MFB) of the breast comprised predominantly of a mature fatty component, representing approximately 70% of the entire tumour area. This tumour, designated "lipomatous MFB", should be interpreted as the morphological result of an unbalanced bidirectional differentiation of the precursor mammary stromal cell, with the adipocytic component overwhelming the fibroblastic/myofibroblastic one. Lipomatous MFB is a rare variant of mammary MFB, which can mimic malignancy because of the close juxtaposition of fibroblasts/myofibroblasts with mature adipocytes, resulting in a finger-like infiltrative growth pattern of the former towards the latter. Histogenetic considerations and differential diagnostic problems with other bland-looking spindle cell tumours containing infiltrating fat are provided. PMID:25291864

  19. Excessive nodular hyperplasia of Brunner glands associated with gastric hypersecretion and lipomatous atrophy of the pancreas.

    PubMed

    Tøttrup, A; Rokkjaer, M; Kruse, A; Poulsen, S S; Jacobsen, N O

    1999-05-01

    The case of a 34-year-old woman complaining of diarrhoea and abdominal pain is presented. Contrast radiography and endoscopy showed multiple polypoid tumours in the second part of the duodenum. Moreover, a severe fatty infiltration of the pancreas was shown by magnetic resonance and computed tomography scans. Due to pain, pancreatoduodenectomy (Whipple operation) was performed, and subsequent histopathologic examinations showed excessive Brunner gland hyperplasia of the duodenum and severe lipomatous atrophy of the pancreas. The occurrence of these two rare conditions in one patient has not been described previously, and it is conceivable that the lipomatous atrophy and exocrine insufficiency of the pancreas may have caused a compensatory stimulation of the submucosal structures of the duodenum. PMID:10423074

  20. The General Surgeon’s Quandary: Atypical Lipomatous Tumor vs Lipoma, Who Needs a Surgical Oncologist?

    PubMed Central

    Fisher, Sarah B; Baxter, Katherine J; Staley, Charles A; Monson, David K; Murray, Douglas R; Oskouei, Shervin V; Weiss, Sharon W; Kooby, David A; Maithel, Shishir K; Delman, Keith A

    2013-01-01

    Background Differentiating large lipomas from atypical lipomatous tumors (ALT) is challenging and preoperative management guidelines are not well-defined. The diagnostic ambiguity leads many surgeons to refer all patients with large lipomatous masses to an oncologic specialist, perhaps unneccessarily. Study Design In this retrospective cohort study of patients with nonretroperitoneal lipomatous tumors, preoperative characteristics discernible without invasive diagnostic procedures were evaluated for diagnostic predictive value. Results 319 patients (256 with lipomas, 63 with ALTs) treated between 1994 and 2012 were identified. Patients with ALTs were older (60.5 vs. 53.5 years, p<0.0001), had larger tumors (16.0 vs 8.3 cm, p<0.0001), had tumors more often located on an extremity (88.9% vs. 60.5% torso, p<0.0001), and more frequently had a history of prior operations at the same site, exclusive of excision leading to diagnosis and referral (20.6% vs. 5.9%, p=0.001). Local recurrence was observed in 2 patients with lipomas (0.8%) vs. 14 with ALTs (22.6%, p<0.0001). No patients with ALTs developed distant metastases or disease-specific mortality with a median follow-up of 27.4 months (range 0–164.6 months). On multivariate analysis age ?55 years, tumor size ?10 cm, extremity location, and history of prior operations were predictors for diagnosis of ALT (p<0.05). Conclusions Characteristics of lipomatous masses that are associated with a diagnosis of ALT include patient age ?55 years, tumor size ?10 cm, previous resection, and extremity location (vs torso). These easily identifiable traits may guide surgical management or referral to a specialist. PMID:24074812

  1. Lipomatous tumours of the head and neck: a spectrum of biological behaviour.

    PubMed

    de Bree, Eelco; Karatzanis, Alexander; Hunt, Jennifer L; Strojan, Primož; Rinaldo, Alessandra; Takes, Robert P; Ferlito, Alfio; de Bree, Remco

    2015-05-01

    Lipomatous tumours are rare in the head and neck region. Their biological behaviour varies greatly, from absolutely benign to histologically benign but locally infiltrative and, finally, invasive with metastatic potential. Each lipomatous tumour has to be treated accordingly. It is of paramount importance before eventual surgery is planned to perform adequate imaging, diagnostic biopsy and careful assessment. Only in small (<5 cm), superficial soft tissue tumours or when magnetic resonance imaging has demonstrated specific features of lipoma, may diagnostic biopsy be omitted. In these cases, expectant management or simple excision is appropriate. Adequate preoperative diagnosis is important to assure adequate tumour control as well as optimal functional and cosmetic outcome. The major problem in the treatment of lipomatous tumours of the head and neck region is the presence of nearby delicate structures. Especially, wide surgical excision of liposarcomas may be hindered by anatomic constraints and may result in impaired functional and cosmetic outcome. Neoadjuvant radiotherapy and specific systemic chemotherapy may be helpful in the treatment of liposarcoma, especially when unresectable or when primary surgery is expected to result in poor oncological, functional or cosmetic outcome. Greater emphasis placed on the underlying biology of individual sarcoma subtypes, development and evaluation of novel therapies and greater specificity in the selection of chemotherapy agents based on activity in individual histological subtypes are expected to lead to improved efficacy of systemic treatment. PMID:24800932

  2. Angiofibroma of soft tissue with fibrohistiocytic features and intratumor genetic heterogeneity of NCOA2 gene rearrangement revealed by chromogenic in situ hybridization: a case report.

    PubMed

    Fukuda, Yumiko; Motoi, Toru; Kato, Ikuma; Ikegami, Masachika; Funata, Nobuaki; Ohtomo, Rie; Horiguchi, Shinichiro; Goto, Takahiro; Hishima, Tsunekazu

    2014-05-01

    Angiofibroma of soft tissue is a recently described soft tissue tumor that is characterized by fibroblastic spindle tumor cells with arborizing capillary proliferation. Cytogenetically, it harbors a specific fusion gene involving the nuclear receptor coactivator 2 (NCOA2) gene. We report here additional new pathological and cytogenetic features. A soft tissue tumor in the left thigh of 73-year-old female was investigated. Microscopically, histiocytoid tumor cells were scattered in an edematous background with branching capillary proliferation. Immunohistochemically, we identified that the tumor cells were positive for histiocytic markers such as CD68 and CD163. Rearrangement of the NCOA2 gene was detected successfully by chromogenic in situ hybridization; however, abnormal signal patterns were observed in only a small subset of tumor cells. Unlike typical tumors with bland spindle cells, the present tumor needs to be distinguished from myxoid, dendritic and clear cell tumors. This case may suggest that angiofibroma of soft tissue is not in the center of the fibroblastic/myofibroblastic tumor group, but rather shows a fibrohistiocytic nature. We also found intratumor genetic heterogeneity, which is uncommon for a translocation-associated tumor. Therefore, careful evaluation is required to detect the gene rearrangement in this tumor entity. PMID:24888778

  3. Benign lipomatous masses of the heart: a comprehensive series of 47 cases with cytogenetic evaluation.

    PubMed

    Bois, Melanie C; Bois, John P; Anavekar, Nandan S; Oliveira, Andre M; Maleszewski, Joseph J

    2014-09-01

    Benign lipomatous lesions of the heart encompass an apparently etiologically diverse group of entities including neoplastic, congenital, and reparative phenomena. Among these, lipomas and lipomatous hypertrophy of the atrial septum (LHAS) represent 2 commonly encountered mass lesions. To date, no study has systematically and comparatively evaluated the morphologic and genetic characteristics of these lesions. Tissue registry archives of Mayo Clinic were queried for cases of cardiac lipoma and LHAS (1994-2011). Clinical, imaging, and pathologic findings were reviewed. Representative cases in each cohort were evaluated by fluorescence in situ hybridization (FISH) for HMGA1 and HMGA2 loci rearrangement and for MDM2/CPM locus amplification. Five cases of cardiac lipoma were identified (mean age, 67 years; range, 48-101; 3 men): 4 right atrial and 1 left ventricular. Forty-two cases of LHAS were identified (mean age, 75.6 years; range 45-95; 20 men), 39 of which were autopsy derived. The median size was 3.4 cm for lipomas and 2.8 cm for LHAS (n = 14). A single case each of cardiac lipoma and LHAS were found to harbor HMGA2 rearrangement, whereas no case showed cytogenetic abnormality of HMGA1 or CPM. This represents the largest series of histopathologically confirmed cardiac lipomas from a single institution. In addition, it is the first to evaluate cardiac lipomas and LHAS for genetic alterations associated with extracardiac lipomatous lesions. The genetic and morphologic similarities found provide evidence in support of the neoplastic classification of cardiac lipomas. A single case of LHAS contained an HMGA2 rearrangement, challenging the currently accepted hypothesis of pathogenesis for this lesion. PMID:24996689

  4. Lipomatous congenital melanocytic nevus presenting as a neck mass in a young adult.

    PubMed

    Patel, Kalyani R; Chernock, Rebecca; Lewis, James S; Raptis, Constantine A; Al Gilani, Maha; Dehner, Louis P

    2013-12-01

    Congenital melanocytic nevus (CMN) is a melanocytic proliferation that has its onset at birth or shortly thereafter and shows characteristic histopathologic features including symmetric proliferation of benign melanocytes, extension of nevus cells into the deep reticular dermis and subcutis, maturation of melanocytes with descent, tracking of melanocytes around and within adnexal structures, vessels, or nerves and splaying of collagen bundles by nevus cells arranged in single rows or cords. We report the case of a 34 year old previously healthy woman who presented with a progressively enlarging soft tissue mass in the right neck and back adjacent to a medium sized CMN. Magnetic resonance imaging showed multiple lipomatous masses within the soft tissues of the posterior superficial neck. Subsequent excision of the soft tissue mass showed a well circumscribed lipomatous lesion with diffuse infiltration by benign appearing melanocytes within the fat lobules. Excision of the mass was not accompanied by overlying skin and, thus, posed a diagnostic challenge. Sudden increase in the size of a CMN is worrisome for the development of a melanoma, however, this lesion lacked significant cytologic atypia and mitotic figures, and had a low proliferative index by Ki-67 immunohistochemistry. This case serves to illustrate the initial diagnostic dilemma as well as the plasticity of the neural crest cells. PMID:23649716

  5. Lipomatous tumors of the oral mucosa: histomorphological, histochemical and immunohistochemical features.

    PubMed

    Allon, Irit; Aballo, Sara; Dayan, Dan; Vered, Marilena

    2011-12-01

    We conducted a comprehensive study of all lipomatous tumors of the oral mucosa (1996-2008) accessioned at the Department of Oral Pathology, Tel Aviv University, collected demographic data and analyzed multiple histomorphological features. Furthermore, we examined the immunostaining of aP2 (adipocyte lipid binding protein) and the polarization colors of picrosirius red (PSR)-stained collagen fibers in order to test their potential in differentiating between benign and malignant tumors. All cases were immunohistochemically stained with aP2 antibody; only tumors with considerable collagenous stroma were selected for the PSR staining. A total of 77 tumors were included in the study, 91% benign and 9% malignant. Fibrolipoma (37.7%) and lipoma (36.4%) were the most frequent tumors. Atypical lipomatous tumor (ALT) was the only type of malignancy. The most common location for the benign tumors was the buccal mucosa and for ALT, the tongue. Histomorphological features characteristic of malignant tumors were occasionally present also in the benign entities. Expression of aP2 was similar in all tumors, while the polarization colors of the PSR-stained collagen fibers differed significantly between ALT and benign tumors (p<0.05). Benign and malignant tumors occasionally show overlapping histomorphological features that require a meticulous examination. PSR staining with polarization microscopy could aid in differentiating malignant from benign tumors in equivocal cases. PMID:21376375

  6. Atypical lipomatous tumor, its variants, and its combined forms: a study of 61 cases, with a minimum follow-up of 10 years.

    PubMed

    Evans, Harry L

    2007-01-01

    Sixty-one cases of neoplasms composed wholly or in part of atypical lipomatous tumor were reviewed. Minimum follow-up was 10 years. The cases were divided into 4 groups based on the findings in the initial excision specimen: conventional atypical lipomatous tumor (n=15), cellular atypical lipomatous tumor (n=21), dedifferentiated liposarcoma (n=24), and atypical lipomatous tumor with a pleomorphic liposarcoma-like component (n=1). The term "cellular atypical lipomatous tumor" was applied to atypical lipomatous tumors having areas of increased cellularity that when non-lipogenic lacked the 5 mitotic figures per 10 high-power fields (maximal rate) required for a dedifferentiated component and when lipogenic fell short of being truly pleomorphic liposarcoma-like. Myxoid regions within this spectrum sometimes had prominent or even plexiform vascularity, creating a resemblance to myxoid liposarcoma especially when interspersed small fat cells were present. The most important prognostic factor was tumor location, as none of the 12 patients with a subcutaneous or intramuscular neoplasm died of tumor. Among the 49 patients with neoplasms of central body sites (mostly retroperitoneum), those with dedifferentiated liposarcoma had significantly shorter survival (median 77 mo) than those with cellular (median 142 mo) or conventional (median 209 mo) atypical lipomatous tumor, whereas there was no statistically significant difference between the latter 2 categories. Patients with atypical lipomatous tumor (either cellular or conventional) in central body sites had significantly shorter survival if the tumor transformed into dedifferentiated liposarcoma in recurrence, and, conversely, those with central body site dedifferentiated liposarcoma had significantly longer survival if it recurred as atypical lipomatous tumor. Metastasis (7 cases) occurred only when the initial specimen or a recurrence demonstrated dedifferentiated liposarcoma. PMID:17197914

  7. Lipomatous tumors of the adrenals--a report on 18 cases and review of the literature.

    PubMed

    Lesbats-Jacquot, Virginie; Cucchi, Jean-Michel; Amoretti, Nicolas; Novellas, Sébastien; Chevallier, Patrick; Bruneton, Jean Noël

    2007-01-01

    The lipomatous tumors of the adrenals are hormonally inactive lesions, often of a benign nature, such as myelolipomas, lipomas, angiomyolipomas, or mature teratomas, and are rarely malignant, such as liposarcomas. The importance of recognizing their characteristic radiological images, which would lead to their correct treatment, is fundamental since there has been an increase in the demonstration of this lesion, often detected incidentally. The various imaging procedures, although not allowing to formulate a histological diagnosis, nonetheless permit to determine the volume of the tumoral mass and their evolution. These two factors are of significant importance in the planning of the correct surgical procedure, while a third important factor is the proper evaluation of the symptoms presented by the patient. Regarding the treatment, on the basis of our own experience, which agrees with that of other authors, the surgical removal of any lesion greater than 5 cm is mandatory. PMID:17825742

  8. Sudden death and lipomatous infiltration of the heart involved by fat necrosis resulting from acute pancreatitis.

    PubMed

    Roncati, Luca; Gualandri, Giorgio; Fortuni, Giuseppe; Barbolini, Giuseppe

    2012-04-10

    The possible causal link between damage to the heart and acute pancreatitis and other pancreatic diseases has been considered in both adults and children, particularly in cases of sudden, unexpected death. However, the cardiac pathological findings so far reported in the literature are neither specific enough, nor of a kind to prove a direct pancreatic pathogenesis. We describe the occurrence of steatonecrosis developed in areas of lipomatous infiltration of the heart following acute exacerbation of latent chronic pancreatitis. The presence of mature adipocytes in the myocardium is an adequate substrate for the pancreatic lipase to give rise to the steatonecrosis, which is a well-known marker of acute pancreatitis. As far as we are aware, this is the first reported case of heart steatonecrosis in the literature. PMID:22079999

  9. Atypical and malignant neoplasms showing lipomatous differentiation. A study of 111 cases.

    PubMed

    Azumi, N; Curtis, J; Kempson, R L; Hendrickson, M R

    1987-03-01

    One-hundred-eleven cases of histopathologically atypical or malignant lipomatous lesions in the somatic soft tissue and retroperitoneum were studied. These consisted of 48 differentiated fatty neoplasms of the somatic soft tissues (DFT-S), 21 fatty neoplasms of the retroperitoneum (DFT-R), 33 myxoid liposarcomas from various sites and nine pleomorphic liposarcomas. DFT-S were defined as lipomatous lesions composed of mature fat and containing atypical stromal cells or lipoblasts. In the somatic soft tissues, this group included lesions that would be classified using published criteria as "atypical lipoma", "pleomorphic lipoma", "well-differentiated lipoma-like liposarcoma", and "sclerosing liposarcoma". All of the DFT-R met previously published criteria for "well differentiated liposarcoma" or "sclerosing liposarcoma". We found no consistent histologic differences between the DFT-S and DRT-R. No pure "round cell" liposarcomas were encountered although many myxoid liposarcomas had "round cell" areas. Follow-up data were available in 80 cases (72%) with a mean follow-up period of over 7 years. Among the DFT-S there were no uncontrollable recurrences, distant metastases, or tumor-related deaths. The depth of the neoplasm correlated with the tendency for local recurrence; no neoplasms primary in the subcutis recurred; 29% of the tumors recurred when they originated in the deep soft tissues or within the muscle. None of the recurrent tumors demonstrated "dedifferentiation." DFT-R had a recurrence rate of 67% and, although there were no distant metastases, nine patients (43%) died of tumor. Five retroperitoneal tumors dedifferentiated but did not metastasize. In light of this experience, we believe that the term "atypical lipoma" is warranted for the DFT-S and "well differentiated liposarcoma" is an appropriate label for the DFT-R. The overall mean survival for the 52 cases of liposarcoma (excluding DFT-S) was 13.6 years. The mean survival in "well differentiated liposarcoma" (11.25 years) was between that for myxoid liposarcoma (16.25 years) and that for pleomorphic liposarcoma (7 years). Six patients (29%) with myxoid liposarcoma developed local recurrences and 6 patients (29%) developed distant metastases and died. Metastasis was always associated with a round cell (or pleomorphic) component with increased numbers of mitotic figures in either the primary tumor or a local recurrence. PMID:3826477

  10. Lipomatous hemangiopericytoma of the stomach: a case report and a review of literature.

    PubMed

    Jing, Hong-Biao; Meng, Qing-Da; Tai, Yan-Hong

    2011-11-21

    Lipomatous hemangiopericytomas (LHPCs) are rare soft-tissue tumors that are histologically characterized by hemangiopericytomatous vasculature and the presence of mature adipocytes. We present the clinicopathological features of a case of gastric LHPC in a 56-year-old female, along with a literature review. Endoscopy and endoscopic ultrasound showed a submucosal tumor 0.8 cm across in the greatest dimension in the lesser curvature side of the gastric antrum. Grossly, the well-defined mass had a solid and tan-white cut surface admixed with myxoid regions and yellowish areas. Histological examination revealed a submucosal well-circumscribed lesion composed of cellular nodules with the classic appearance of an hemangiopericytoma admixed with clusters and lobules of mature adipocytes. The ill-defined tumor cells had weakly eosinophilic cytoplasm and contained spindled nuclei with occasional small nucleoli. Nuclei atypia and mitoses were absent, and no cellular atypia, necrosis or vascular invasion was observed. Immunohistochemistry showed that the tumor cells were diffusely positive for CD34, CD99, and vimentin and were focally reactive for bcl-2. This is the first known report of an LHPC in the stomach. The patient was followed for 12 mo without any evidence of metastasis or recurrence. PMID:22147987

  11. Multifocal myocardial necrosis: a distinctive cardiac lesion in cystic fibrosis, lipomatous pancreatic atrophy, and Keshan disease.

    PubMed

    Nezelof, Christian; Bouvier, Raymonde; Dijoud, Frédérique

    2002-01-01

    Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber. PMID:12056507

  12. Lipomatous Pseudohypertrophy of the Pancreas Taking the Form of Huge Massive Lesion of the Pancreatic Head.

    PubMed

    Shimada, Masanari; Shibahara, Kazushige; Kitamura, Hirotaka; Demura, Yoshitaka; Hada, Masahiro; Takehara, Akira; Nozaki, Zensei; Sasaki, Masatoshi; Konishi, Kohji; Maeda, Yoshinobu

    2010-01-01

    A 70-year-old woman presented with hypogastric pain. Computed tomography and magnetic resonance imaging revealed a retroperitoneal tumor 18.0 cm in diameter with fatty tissue density, ventrally compressing the pancreatic head. We suspected a well-differentiated liposarcoma compressing the pancreas. At laparotomy, the tumor mass was the size of an infant's head; its center was located in the area corresponding to the pancreatic uncus. It was continuous with the pancreatic parenchyma through a poorly demarcated border, and we resected as much of the tumor mass as possible while conserving the pancreatic capsule. Histopathological examination indicated lipomatous pseudohypertrophy of the pancreas with proliferation of mature fatty tissue as the main constituent. At the periphery, islands of acinar tissue were retained among the fatty infiltration, which also contained branches of the pancreatic duct and islets of Langerhans. Previous reports have stated that this disorder only causes fatty replacements throughout the pancreas or in the pancreatic body and tail; however, in this patient, imaging and macroscopic examination revealed no fatty replacements in the pancreatic body and tail. We report this case, which we consider extremely rare, along with a brief review of the literature. PMID:21103205

  13. Lipomatous Pseudohypertrophy of the Pancreas Taking the Form of Huge Massive Lesion of the Pancreatic Head

    PubMed Central

    Shimada, Masanari; Shibahara, Kazushige; Kitamura, Hirotaka; Demura, Yoshitaka; Hada, Masahiro; Takehara, Akira; Nozaki, Zensei; Sasaki, Masatoshi; Konishi, Kohji; Maeda, Yoshinobu

    2010-01-01

    A 70-year-old woman presented with hypogastric pain. Computed tomography and magnetic resonance imaging revealed a retroperitoneal tumor 18.0 cm in diameter with fatty tissue density, ventrally compressing the pancreatic head. We suspected a well-differentiated liposarcoma compressing the pancreas. At laparotomy, the tumor mass was the size of an infant's head; its center was located in the area corresponding to the pancreatic uncus. It was continuous with the pancreatic parenchyma through a poorly demarcated border, and we resected as much of the tumor mass as possible while conserving the pancreatic capsule. Histopathological examination indicated lipomatous pseudohypertrophy of the pancreas with proliferation of mature fatty tissue as the main constituent. At the periphery, islands of acinar tissue were retained among the fatty infiltration, which also contained branches of the pancreatic duct and islets of Langerhans. Previous reports have stated that this disorder only causes fatty replacements throughout the pancreas or in the pancreatic body and tail; however, in this patient, imaging and macroscopic examination revealed no fatty replacements in the pancreatic body and tail. We report this case, which we consider extremely rare, along with a brief review of the literature. PMID:21103205

  14. YKL-40 expression in soft-tissue sarcomas and atypical lipomatous tumors

    PubMed Central

    2014-01-01

    Background and purpose YKL-40 is a glycoprotein that is expressed in many types of cancer cells. In some cancers, there is a correlation between high serum YKL-40 levels on the one hand and more aggressive disease and early death on the other. YKL-40 has never been studied in patients with soft-tissue sarcomas (STSs). We investigated whether YKL-40 is expressed in STS tissue and ascertained that the degree of expression is related to survival and/or the histological grade of the malignancy (FNCLCC). Patients and methods We included archived tissue from 49 patients (40 with STS and 9 with atypical lipomatous tumor, 20 female and 29 male, mean age 58 (4–89) years) who were treated with tumor resection in 2004 or 2005 at the Department of Orthopedics, Rigshospitalet. The minimum length of follow-up with respect to survival was 5–7 years. Immunohistochemical analysis with anti-YKL-40 antibody using tissue microarray was performed on resected tumors, and a semiquantitative measure of the intensity of YKL-40 staining was performed. Results 41 of the 49 tumors were positive for YKL-40, and of these, 36 had moderate to intense staining. 24 of the patients died within the follow-up period, and the intensity of YKL-40 staining was significantly higher in tumors from patients who had died in the follow-up period than in tumors from those who survived (p = 0.01). The staining intensity was different for the 3 grades of malignancy (p = 0.004): it was higher in highly malignant tumors (FNCLCC grade 2 and grade 3) than in low-malignancy tumors (grade 1). Interpretation YKL-40 is expressed in soft-tissue sarcomas. There is a correlation between expression of YKL-40 in STS and both histological grade of the malignancy and survival. Whether or not YKL-40 expression is an independent prognostic variable could not be determined in the present study. PMID:24650028

  15. The diagnosis and treatment of primary adrenal lipomatous tumours in Chinese patients: A 31-year follow-up study

    PubMed Central

    Zhao, Juping; Sun, Fukang; Jing, Xiaolong; Zhou, Wenlong; Huang, Xin; Wang, Haofei; Zhu, Yu; Yuan, Fei; Shen, Zhoujun

    2014-01-01

    Introduction: Adrenal lipomatous tumours (ALTs) are rarely encountered in clinical practice and consequently little is known about their clinical features. Methods: We analyze the clinical features, diagnosis and treatment of ALTs based on cases presenting at a single centre over a 31-year period. We reviewed clinical data from patients with primary adrenal tumours treated at the Ruijin Hospital, Shanghai between January 1980 and December 2010. Results: A total of 73 cases of primary ALTs in 22 men and 51 women (mean age 51.1±14.2 years) were reviewed. The ALTs included 65 myelolipomas (89.0%), 3 lipomas (4.1%), 2 angiomyolipomas (2.7%), 2 teratomas (2.7%), and 1 liposarcoma (1.4%). Of the total 73 patients, 24 of them had tumours in the left adrenal gland, 47 in the right gland and 2 had bilateral tumours. In total, 51 patients underwent open surgery and 22 laparoscopic surgery. Conclusion: Myelolipoma is predominant among the various types of lipomatous adrenal gland tumours; it accounts for about 90% of all cases. Surgery is recommended for tumours ?3.5 cm in diameter, for all cases of symptomatic tumour, and for cases of teratoma or liposarcoma identified by preoperative imaging. PMID:24678351

  16. Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.

    PubMed Central

    Fletcher, C. D.; Akerman, M.; Dal Cin, P.; de Wever, I.; Mandahl, N.; Mertens, F.; Mitelman, F.; Rosai, J.; Rydholm, A.; Sciot, R.; Tallini, G.; van den Berghe, H.; van de Ven, W.; Vanni, R.; Willen, H.

    1996-01-01

    Soft tissue tumors commonly show cytogenetic abnormalities, some of which are tumor specific. Lipomatous tumors represent the largest category of soft tissue neoplasms, and numerous karyotypic aberrations have been identified. However, clear-cut correlation between morphology and karyotype has not been undertaken on a systematic basis in a double-blind setting. The morphological features and histological diagnosis of 178 lipomatous neoplasms were reviewed independently without knowledge of the clinical data. The consensus diagnoses were then correlated with the clinical findings and compared with the tumors' karyotypes, using G-banded preparations from short-term cultures. The data were collated by a multicenter collaborative group of pathologists, geneticists, and surgeons. Clonal chromosomal abnormalities were identified in 149 cases studied (84%) and, to a large extent, the karyotype correlated with the morphological diagnosis. Specifically, 26 (96%) of 27 myxoid liposarcomas and its poorly differentiated variants showed a t(12;16); 29 (78%) of 37 atypical lipomatous tumors (including 5 dedifferentiated cases) showed ring chromosomes; 74 (80%) of 93 subcutaneous and intramuscular lipomas had karyotypic aberrations affecting mainly 12q, 6p, and 13q; 7 of 8 spindle cell and pleomorphic lipomas had aberrations of 16q; 3 lipoblastomas showed 8q rearrangements; and 2 hibernomas showed 11q abnormalities. We conclude that cytogenetic abnormalities are common in lipomatous tumors, correlate reliably with morphological sub-type in many cases, and can be of diagnostic value in histologically borderline or difficult cases. PMID:8579124

  17. Lipomatous hemangiopericytoma (adipocytic variant of solitary fibrous tumor) of the parotid gland: A case report and review of the literature

    PubMed Central

    CHEN, DAN; XUAN, JIZE; SUN, MEIYU; GUAN, HONGWEI

    2013-01-01

    The current study presents the first case of a lipomatous hemangiopericytoma (LHPC) developing in the parotid gland in a 33-year-old male. The patient presented with a 4-year history of a progressively growing painless and fixed mass in the left paratid gland region. The patient underwent radical parotidectomy and was followed-up for 12 months without any evidence of metastasis or recurrence. LHPC, a controversial rare HPC variant, is histologically characterized by a varying admixture of hemangiopericytomatous vasculature and the presence of mature adipocytes. To date, 51 cases of LHPC have been documented in the literature. Although the boundary between HPC and solitary fibrous tumors (SFTs) has become increasingly blurred, neither of these variant growth patterns has previously been recognized in the parotid gland. PMID:24179528

  18. Lipomatous hemangiopericytoma (adipocytic variant of solitary fibrous tumor) of the parotid gland: A case report and review of the literature.

    PubMed

    Chen, Dan; Xuan, Jize; Sun, Meiyu; Guan, Hongwei

    2013-11-01

    The current study presents the first case of a lipomatous hemangiopericytoma (LHPC) developing in the parotid gland in a 33-year-old male. The patient presented with a 4-year history of a progressively growing painless and fixed mass in the left paratid gland region. The patient underwent radical parotidectomy and was followed-up for 12 months without any evidence of metastasis or recurrence. LHPC, a controversial rare HPC variant, is histologically characterized by a varying admixture of hemangiopericytomatous vasculature and the presence of mature adipocytes. To date, 51 cases of LHPC have been documented in the literature. Although the boundary between HPC and solitary fibrous tumors (SFTs) has become increasingly blurred, neither of these variant growth patterns has previously been recognized in the parotid gland. PMID:24179528

  19. Lipomatous metaplasia identified in rabbits with reperfused myocardial infarction by 3.0 T magnetic resonance imaging and histopathology

    PubMed Central

    2013-01-01

    Background Cardiac lipomatous metaplasia (LM) occurs in patients with chronic ischemic heart disease and heart failure with unclear mechanisms. We studied coronary occlusion/reperfusion-induced myocardial infarction (MI) in rabbits during a 9-months follow-up using 3.0 T magnetic resonance scanner, and confirmed the presence of MI in acute phase and LM in chronic phase using histopathology. Methods MI was surgically induced in 10 rabbits by 90-min coronary artery occlusion and reperfusion. Forty-eight hours later, multiparametric cardiac magnetic resonance imaging (cMRI) was performed at a 3.0 T clinical scanner for MI diagnosis and cardiac function analysis. Afterwards, seven rabbits were scarified for histochemical staining with triphenyltetrazolium chloride (TTC), and hematoxylin-eosin (HE), and 3 were scanned with cMRI at 2 days, 2 weeks, 2 months and 9 months for longitudinal observations of morphological and functional changes, and the fate of the animals. Post-mortem TTC, HE and Masson's trichrome (MTC) were studied for chronic stage of MI. Results The size of acute MI correlated well between cMRI and TTC staining (r2=0.83). Global cardiac morphology-function analysis showed significant correlation between increasing acute MI size and decreasing ejection fraction (p<0.001). During 9 months, cMRI documented evolving morphological and functional changes from acute MI to chronic scar transformation and fat deposition with a definite diagnosis of LM established by histopathology. Conclusions Acute MI and chronic LM were induced in rabbits and monitored with 3.0 T MRI. Studies on this platform may help investigate the mechanisms and therapeutic interventions for LM. PMID:23815556

  20. Malignant fat-forming solitary fibrous tumor (so-called "lipomatous hemangiopericytoma"): clinicopathologic analysis of 14 cases.

    PubMed

    Lee, Jen-Chieh; Fletcher, Christopher D M

    2011-08-01

    Fat-forming solitary fibrous tumor is a rare variant of solitary fibrous tumor (SFT). Generally regarded as benign, very few fat-forming SFTs with malignant histologic features have been reported. Here, we report 14 histologically malignant fat-forming SFTs to better characterize this subset. Seven patients were female and 7 were male, with ages ranging 20 to 93 years (median, 57 y). Five tumors were located in the lower limb, 3 in the trunk, 3 in abdominopelvic locations, 2 in the head and neck region, and 1 in the upper limb. The tumor size ranged from 3.4 to 20 cm (median, 8.6 cm). Histologically, all exhibited at least focal hypercellularity; 12 tumors had mitoses >4/10 high-power fields (range, 2 to 37; median, 8), 12 showed at least moderate atypia, and 8 showed necrosis. It should be noted that 7 tumors contained only mature adipose tissue, whereas 5 contained multivacuolated lipoblasts and 2 had areas resembling atypical lipomatous tumor (ALT). Immunohistochemically, CD34 and CD99 were positive in most cases (11 of 14 and 8 of 10, respectively); MDM2 and CDK4 were both negative in all 4 cases tested (including both tumors with ALT-like areas). Follow-up data from 10 cases (median duration, 47.5 mo; range, 5 to 76) showed 2 patients with multiple metastases (both to lung and bones, and 1 each to breast and to soft tissue), both of whom died of disease. In conclusion, fat-forming SFTs exhibiting malignant histologic features have potential for aggressive behavior. The presence of lipoblasts and/or ALT-like areas, although described in some "benign" examples of fat-forming SFT, seems much more common in the malignant subset and may prompt a careful search for morphologic evidence of malignancy in any case of fat-forming SFT. PMID:21716088

  1. Diffuse Mallory bodies in the liver, diffuse Lewy bodies in the brain and diffuse fat replacement (lipomatous pseudohypertrophy) of the pancreas in a patient with juvenile Parkinson's disease.

    PubMed

    Yoshimura, N; Hayashi, S; Fukushima, Y

    1992-11-01

    A 38-year-old male patient with the juvenile variant of Parkinson's disease, in whom onset had occurred at the age of 24 yr, was autopsied. There were no clear symptoms of pancreatic or hepatic insufficiency during the entire clinical course. The only notable features were a slightly delayed decrease of the blood glucose level in an oral glucose tolerance test, slightly elevated levels of serum alkaline phosphatase and serum lactate dehydrogenase, and episodic loose stools. Autopsy revealed uniform enlargement of the pancreas due to massive fat replacement (lipomatous pseudohypertrophy): the exocrine glandular elements showed marked atrophy and loss, while the islets of Langerhans were preserved. The liver exhibited a histology closely mimicking alcoholic hepatitis associated with the diffuse presence of Mallory bodies (MBs), possibly indicative of a disturbance of protein metabolism. The nervous system showed the diffuse presence of Lewy bodies (LBs) in the cerebrum in addition to the ordinary lesions of Parkinson's disease. Although the etiopathogenesis of none of these three lesions has been well elucidated, common epitopes of MBs and LBs have recently been demonstrated. Therefore, the present case study suggests that a specific underlying toxic agent may cause diffuse LBs in the brain on the one hand, and diffuse MBs in the liver and lipomatous pseudohypertrophy of the pancreas on the other. PMID:1471530

  2. An Unexpected Cause of Hemoptysis: Endobronchial Lipomatous Hamartoma

    PubMed Central

    Sarioglu, Nurhan; Susur, Alev; Goksel, Tuncay; Paksoy, Serpil; Erel, Fuat

    2014-01-01

    ABSTRACT Hamartomas are the most common benign tumors of the lung. Endobnronchial hamartomas are even rarer and infrequently causes hemoptysis. We report a case of endobronchial hamartoma that was originating from a segment bronchus and invisible in chest X-ray. A 63-year-old man was admitted to hospital with hemoptysis. A CT scan revealed endobronchial mass obstructing anterior bronchus of the right lower lob of the right lung. It wasn’t radiographically presented. Flexible bronchoscopy detected a polypoid mass (1.5x1.0 cm) that arising from the posterior wall of the anterior segment of right lower lob. Histopathologic examination revealed lipoumatous hamartoma. It was resected with an electro-surgical snare. Cryotherapy was applied to residual lesion on surface of the bronchus. The patient was successfully recovered. In conclusion, lipoumatous hamartoma may presented as rare cause of hemoptysis. Endoscopic treatment is safe and currently modality used for select cases. PMID:24783918

  3. Giant Submucosal Lipomatous Polyp Causing Perforation of Sigmoid Colon: A Case Report and Review of Literature

    PubMed Central

    Laksman, Triza Kumar

    2015-01-01

    Colonic lipomas are rare benign tumours that are usually asymptomatic. Large colonic lipomas can present with symptoms such as constipation, abdominal pain, bleeding per rectum, and intussusception. We report the case of an 82-year-old man presenting with sudden onset lower abdomen pain and features of peritonitis. An exploratory laparotomy revealed a perforation of sigmoid colon with a fleshy mass protruding through the perforation. No case of spontaneous perforated sigmoid colon by submucous giant lipoma is reported yet in the literature. We discuss the details of first such case, our management, the outcome and also briefly review the literature. PMID:25738034

  4. Spindle cell myoepithelial tumours of the parotid gland with extensive lipomatous metaplasia

    Microsoft Academic Search

    Alena Skálová; Ivo Stárek; Roderick HW Simpson; Vladislava Ku?erová; Jana Dvo?á?ková; Romuald ?u?ík; Milada Dušková

    2001-01-01

    We report four cases of parotid gland tumours composed predominantly of spindle-shaped myoepithelial cells and mature adipocytes. The central portion of one tumour showed extensive adipose differentiation, whereas in the peripheral parts there were small foci of ductal epithelium arranged in cords and tubules within an abundant myxoid stroma. The other cases were adipose spindle cell myoepitheliomas without an obvious

  5. Malignant fat-forming solitary fibrous tumor (lipomatous hemangiopericytoma) in the neck: Imaging and histopathological findings of a case

    PubMed Central

    de Carvalho, Alice Duarte; Abrahão-Machado, Lucas Faria; Viana, Cristiano Ribeiro; de Castro Capuzzo, Renato; Mamere, Augusto Elias

    2013-01-01

    Fat-forming solitary fibrous tumor (SFT) is a rare variant of solitary fibrous tumor, a mesenchymal fibroblastic neoplasia with a particular branching hypervascular pattern. This tumor is usually classified as benign and only very few fat-forming SFTs with malignant histologic features have been reported. We report a histologically malignant fat-forming solitary fibrous tumor in a 61-year-old man, located in his neck. Ultrasonography examination was first performed showing a heterogeneous lesion, predominantly hyperechoic, with sound beam attenuation, containing two hypoechoic solid nodules. Magnetic resonance imaging and computed tomography examinations demonstrated a heterogeneous and predominantly adipose mass, containing post contrast enhancing solid nodules and thin septations. Treatment consisted of total removal of the lesion. Histologically, the tumor showed hypercellularity, numerous mitoses and cytological atypia, fulfilling the criteria for malignancy. The patient had no metastasis. This rare tumor may be confused with other fat-containing lesions on imaging examinations, mainly liposarcoma. PMID:23705040

  6. Malignant fat-forming solitary fibrous tumor (lipomatous hemangiopericytoma) in the neck: Imaging and histopathological findings of a case.

    PubMed

    de Carvalho, Alice Duarte; Abrahão-Machado, Lucas Faria; Viana, Cristiano Ribeiro; de Castro Capuzzo, Renato; Mamere, Augusto Elias

    2013-03-01

    Fat-forming solitary fibrous tumor (SFT) is a rare variant of solitary fibrous tumor, a mesenchymal fibroblastic neoplasia with a particular branching hypervascular pattern. This tumor is usually classified as benign and only very few fat-forming SFTs with malignant histologic features have been reported. We report a histologically malignant fat-forming solitary fibrous tumor in a 61-year-old man, located in his neck. Ultrasonography examination was first performed showing a heterogeneous lesion, predominantly hyperechoic, with sound beam attenuation, containing two hypoechoic solid nodules. Magnetic resonance imaging and computed tomography examinations demonstrated a heterogeneous and predominantly adipose mass, containing post contrast enhancing solid nodules and thin septations. Treatment consisted of total removal of the lesion. Histologically, the tumor showed hypercellularity, numerous mitoses and cytological atypia, fulfilling the criteria for malignancy. The patient had no metastasis. This rare tumor may be confused with other fat-containing lesions on imaging examinations, mainly liposarcoma. PMID:23705040

  7. Unusual Thymic Hyperplasia Mimicking Lipomatous Tumor in an Eight-Year-Old Boy with Concomitant Pericardial Lipomatosis and Right Facial Hemihypertrophy

    PubMed Central

    Kim, Yoo Jin; Cheon, Jung-Eun; Lim, Yun-Jung; Kim, In-One; Yeon, Kyung Mo; Jung, Kyeong Cheon; Byun, Sun-Ju

    2011-01-01

    We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion. PMID:21603297

  8. CLOVE Syndrome (Congenital Lipomatous Overgrowth, Vascular Malformations, and Epidermal Nevi): CNS Malformations and Seizures may be a Component of this Disorder

    PubMed Central

    Gucev, Zoran S.; Tasic, Velibor; Jancevska, Aleksandra; Konstantinova, Marina Krstevska; Pop-Jordanova, Nada; Trajkovski, Zoran; Biesecker, Leslie G.

    2010-01-01

    A newborn girl was found to have a massive lymphatic truncal vascular malformation with overlying cutaneous venous anomaly associated with overgrown feet and splayed toes. These manifestations comprise the recently described CLOVE syndrome. She also had cranial asymmetry and developed generalized seizures, which were treated with anticonvulsants. Cranial CT showed encephalomalacia, widening of the ventricles and the sulci, hemimegalencephaly (predominantly white matter) and partial agenesis of corpus callosum. Review of the literature identified several other patients with CLOVE syndrome, some of whom were misdiagnosed as having Proteus syndrome, with strikingly similar manifestations. We conclude that CNS manifestations including hemimegalencephaly, dysgenesis of the corpus callosum, neuronal migration defects, and the consequent seizures, may be a more common manifestation of CLOVE syndrome than is presently appreciated. PMID:18816642

  9. Hemihyperplasia-multiple lipomatosis syndrome: an underdiagnosed entity in children with asymmetric overgrowth.

    PubMed

    Boybeyi, Ozlem; Alanay, Yasemin; Kayikçio?lu, Aycan; Karnak, Ibrahim

    2010-01-01

    Hemihyperplasia can be found as an isolated abnormality, or as a predominant associated feature of an asymmetric overgrowth syndrome. This report describes 2 patients with hemihyperplasia-multiple lipomatosis syndrome. The finding of hemihyperplasia prompts careful examination for associated lipomatous lesions. Close follow-up is required because progressive growth of lipomatous lesions can be encountered as well as recurrence after excision. PMID:20105568

  10. FNA of cutaneous juvenile xanthogranuloma--a case report.

    PubMed

    Mohindroo, Shobha; Sharma, Jai Shree

    2005-07-01

    A case of cutaneous juvenile xanthogranuloma of the left shoulder in a three years old female which was diagnosed by FNA is presented here. Juvenile xanthogranuloma is a benign lesion of fibrohistiocytic proliferation, often regresses spontaneously, is a self-limiting lesion and should be treated conservatively. PMID:16761754

  11. [Computed tomography of pancreatic cystic fibrosis].

    PubMed

    Brachlow, M; Zaunbauer, W; Haertel, M

    1984-02-01

    The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas is recommended, particularly in differential diagnostic aspects of cystic fibrosis. PMID:6420278

  12. Bone tumors: Diagnosis, treatment and prognosis. Second edition

    SciTech Connect

    Huvos, A.G.

    1987-01-01

    This book presents treatment modalities of all skeletal neoplasms with special emphasis on clinicopathologic correlations and differential diagnosis. This describes the clinical, radiographic, and pathologic features, plus interdisciplinary approaches to treatment for each tumor type and also covers benign and malignant bone-forming and cartilage-forming tumors, tumors of connective tissue origin, tumors of histoiocytic or fibrohistiocytic origin, and tumors and tumor-like lesions of blood vessels arising in the skeletal system.

  13. [Extrarenal retroperitoneal angiomyolipoma: description of a case and review of the literature].

    PubMed

    Cavazza, A; Giunta, A; Pedrazzoli, C; Putrino, I; Serra, L; De Marco, L; Gardini, G

    2001-02-01

    A case of retroperitoneal extrarenal angiomyolipoma is described. The patient, a 61-year-old woman, presented with a large retroperitoneal lipomatous mass. Histologically, it consisted of lipomatous tissue. Focally, there was interstitial and perivascular proliferation of bland oval and epithelioid cells with a large, eosinophilic or clear cytoplasm. Immunohistochemically, these elements were positive for smooth muscle actin and HMB-45. The literature on retroperitoneal extrarenal angiomyolipoma is briefly reviewed. It is important that the pathologist recognize this lesion, even when it is located in the retroperitoneum outside the kidney, and differentiate it from a liposarcoma. PMID:11294019

  14. Superficial Acral Fibromyxoma involving the nail's apparatus. Case report and literature review*

    PubMed Central

    García, Ana Márquez; Mendonça, Francisco Manuel Ildefonso; Cejudo, Manuel Perea; Martínez, Francisco M. Camacho; Martín, Juan José Ríos

    2014-01-01

    Superficial Acral Fibromyxoma is a rare tumor of soft tissues. It is a relatively new entity described in 2001 by Fetsch et al. It probably represents a fibrohistiocytic tumor with less than 170 described cases. We bring a new case of SAF on the 5th toe of the right foot, in a 43-year-old woman. After surgical excision with safety margins which included the nail apparatus, it has not recurred (22 months of follow up). We carried out a review of the location of all SAF published up to the present day. PMID:24626661

  15. Pathology Case Study: Metastasizing Tumor

    NSDL National Science Digital Library

    Rao, Uma N. M.

    This is a case study presented by the University of Pittsburgh Department of Pathology in which a woman presented with a low-grade sarcoma with features of plexiform fibrohistiocytic tumor in the subcutaneous soft tissue of left posterior thigh. Visitors can view both gross and microscopic descriptions, including images, and have the opportunity to diagnose the patient. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to introduce or test students of soft tissue pathology.

  16. Encephalocraniocutaneous Lipomatosis (Haberland Syndrome)

    Microsoft Academic Search

    Sergiusz Jó?wiak; Ignacio Pascual-Castroviejo

    Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is a rare, congenital, neurocutaneous disorder. It is characterized\\u000a by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye and ipsilateral neurologic malformations.\\u000a Mental retardation and epilepsy may compromise the clinical status.

  17. Giant lipoblastoma of the thigh: a rare soft tissue tumor in an infant.

    PubMed

    Akhtar, Tanveer; Alladi, Anand; Ahmed, Siddiq M; Siddappa, Odda S

    2012-01-01

    Lipoblastoma is a rare lipomatous tumor encountered almost exclusively in infants and young children. It arises from embryonic white fat. The common site of involvement is the extremities. In spite of their potential for local invasion, they are benign tumors. We report a case of a lipoblastoma in an infant and review the literature pertaining to clinical management of these tumors. PMID:22531541

  18. Giant fibrolipoma in the mediastinum: an unusual case.

    PubMed

    Hsu, Jui-Sheng; Kang, Wan-Yi; Liu, Gin-Chung; Kao, Eing-Long; Chuang, Ming-Tsung; Chou, Shah-Hwa

    2005-10-01

    A 50-year-old man presented with a middle and posterior mediastinal mass on chest radiograph and computed tomography. Surgical exploration revealed a large dumbbell-shaped lipomatous lesion. Histologic examination confirmed this to be a fibrolipoma. This is the first reported case of fibrolipoma in the mediastinum but outside of the esophagus. PMID:16181831

  19. Lumbar spinal angiolipoma: case report and review of the literature

    Microsoft Academic Search

    Deniz Konya; Serdar Ozgen; Ozlem Kurtkaya; Necmettin M. Pamir

    2006-01-01

    Spinal angiolipomas are extremely rare benign tumors composed of mature lipomatous and angiomatous elements. Most are symptomatic due to progressive spinal cord or root compression. This article describes the case of a 60-year-old woman who presented with a 6-month history of low back pain radiating to her right leg. The pain was multisegmental. The condition had worsened with time. Lumbar

  20. Lipomatosis of the pancreas. A morphometrical investigation.

    PubMed

    Schmitz-Moormann, P; Pittner, P M; Heinze, W

    1981-12-01

    The study was performed in 50 human autoptic pancreata. After postmortem ductography and formalin fixation the organs were cut in slices of 8 mm thickness. After paraffinembedding histological sections were made from these slices. In photographical magnification (20 X) of these sections the amount of adipose tissue and pancreatic parenchyma were measured by morphometric means. The length of the pancreatic duct was determined in the radiographs. The data were evaluated by statistical methods. The share of the adipose tissue and the total weight of the pancreata are positively correlated depending on age and body weight. In contrast the weight of the pancreatic parenchyma shows no correlation to age, body length and weight. The amount of lipomatosis is the same in the various parts of the dorsal pancreatic anlage. On the other hand, the amount is clearly diminished in the ventral anlage. In the lipomatous pancreata the main duct is longer than in the normal. The lipomatosis of the pancreas is not correlated with a loss of parenchyma, which means, that there is not a true lipomatous atrophy. Even if the parenchyma in the lipomatous pancreata is replaced by fat tissue, which includes the undestroyed pancreatic islets, the parenchymal weight of the pancreas remains unchanged. This and the lengthening of the pancreatic duct in lipomatous organs allow the conclusion that the loss of parenchyma may be compensated by new parenchymal growth. PMID:7335549

  1. Soft tissue giant cell tumour of low malignant potential: a rare tumour at a rare site.

    PubMed

    Bhat, Amoolya; V, Geethamani; C, Vijaya

    2013-12-01

    "Soft tissue giant cell tumour of low malignant potential" is considered as the soft tissue counterpart of osteoclastoma of the bone. It is a primary soft tissue tumour which is classified under the category of fibrohistiocytic tumours of intermediate malignancy.Seventy percent of the tumours involve the extremities and only about seven percent of them arise in head and neck region. They are composed of nodules of histiocytes in a vascular stroma, with multinucleated osteoclast-like giant cells positive for vimentin, smooth muscle actin (SMA), CD68 and Tarterate Resistant Acid Phosphatase (TRAP). We are presenting a case of a 75-year-old man who had a nodule on the ala of the nose. Histopathology showed a histiocytic lesion. Benign fibrous histiocytoma, plexiform fibrohistiocytic tumour, solitary reticulohistiocytoma and histioid leprosy were ruled out by using special stains and immunostains. Expression of smooth muscle actin and CD68 confirmed the diagnosis of a soft tissue giant cell tumour with a low malignant potential. PMID:24551690

  2. Extrapulmonary silicosis: a clinical, morphologic, and ultrastructural study

    SciTech Connect

    Slavin, R.E.; Swedo, J.L.; Brandes, D.; Gonzalez-Vitale, J.C.; Osornio-Vargas, A.

    1985-04-01

    A variety of silicotic lesions derived from thoracic silicosis via lymphohematogenous spread to the liver, spleen, bone marrow, and extrathoracic lymph nodes are described. The morphologic features of these lesions depend on the extent of macrophage aggregation, the occurrence of fibrogenesis, and the development of necrosis and degradative changes in macrophages and adjacent extracellular matrix, presumably caused by lysosomal enzymes released from macrophages. Ultrastructurally, the degenerative alterations of matrix material include longitudinal splitting and breakage of collagen fibrils into segments one and three quarters the length of the original fibrils and deposition of flocculent electron-dense material either focally or diffusely around collagen fibrils. The corresponding changes viewed light microscopically are those of fibrinoid necrosis. The sclerohyaline nodule, the characteristic lesion of silicosis, includes all of these features as it evolves through nodular histiocytic and subsequent fibrohistiocytic phases. Its ultimate morphology appears to be determined by the reassembly of the degraded matrix into non-native, fibrous long-spacing collagen via a spiny collagen intermediary. The sclerohyaline nodule occurs infrequently in the spleen and liver, although less typical lesions caused by silica alone or admixed with other dusts seem to occur more commonly in these organs. These lesions appeared as loose or nodular histiocytic or fibrohistiocytic aggregates.

  3. Pediatric Synovial Sarcoma in the Retropharyngeal Space: A Rare and Unusual Presentation

    PubMed Central

    Vaid, Sanjay; Vaid, Neelam; Desai, Sanjay; Vaze, Varada

    2015-01-01

    Synovial sarcomas in the head and neck are extremely rare tumors, especially in the pediatric population. 3–5% of synovial sarcomas occur in the head and neck region displaying varied imaging and histopathological features resulting in frequent misdiagnosis. These tumors have a poor prognosis; hence early diagnosis and accurate classification based on imaging, histopathology, and immunohistochemistry are critical for prompt treatment. To the best of our knowledge, imaging findings of pediatric retropharyngeal lipomatous synovial sarcoma have not been reported to date in English medical literature. We report, for the first time, a rare case of retropharyngeal lipomatous synovial sarcoma in a ten-year-old child and discuss the case-specific imaging findings in our patient using magnetic resonance imaging and computed tomography. PMID:25632364

  4. A Neonate with CLOVES Syndrome

    PubMed Central

    Akin, Mustafa Ali; Kurtoglu, Selim; Tubas, Filiz; Sarici, Serdar Umit

    2014-01-01

    Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet). PMID:25400966

  5. A Neonate with CLOVES Syndrome.

    PubMed

    Sarici, Dilek; Akin, Mustafa Ali; Kurtoglu, Selim; Tubas, Filiz; Sarici, Serdar Umit

    2014-01-01

    Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet). PMID:25400966

  6. Intramuscular Chondroid Lipoma: Magnetic Resonance Imaging Diagnosis by ‘Fat Ring Sign’

    PubMed Central

    Y?ld?z, Adalet Elçin; Ayd?ngöz, Üstün; Sökmensüer, Cenk; Karçaalt?ncaba, Mu?turay

    2015-01-01

    Background: Chondroid lipoma is an extremely rare variant of benign lipomatous lesions that is composed of lipoblasts, mature fat, and chondroid matrix. Although benign lipomatous lesions are the most common soft tissue tumors and imaging findings are often pathognomonic, there have been few reports describing the imaging features of chondroid lipoma. Case Report: We present magnetic resonance imaging (MRI) findings of a pelvic intramuscular chondroid lipoma in a 59 year-old man and describe a “fat ring sign” that may be useful to diagnose this rare tumor radiologically. Conclusion: Magnetic resonance imaging findings of a chondroid lipoma may be heterogenous according to the distribution of the fatty and chondroid tissue. However, in the presence of “fat ring sign,” radiologists should consider a diagnosis of chondroid lipoma preoperatively.

  7. Potential pitfall of FDG PET/CT in assessing residual masses postchemotherapy.

    PubMed

    Lu, Suat-Jin; Wong, Alvin Seng Cheong; Seet, Ju-Ee; Hao, Cheng; Sinha, Arvind

    2013-04-01

    A 22-year-old man with primary mediastinal choriocarcinoma and bilateral lung metastases underwent an FDG PET/CT scan after completing chemotherapy. Serum beta-human chorionic gonadotropin had normalized. PET/CT showed increased FDG uptake in the anterior mediastinal and lung lesions, suspicious for residual disease. After resection of the mediastinal and lung lesions, histopathology revealed necrosis and fibrohistiocytic reaction with no viable tumor. There was discordance between beta-human chorionic gonadotropin (negative) and FDG PET/CT (positive) findings, with PET/CT findings being false positive. Awareness of this potential pitfall of FDG PET/CT is important, and caution should be exercised when using FDG PET/CT to assess residual masses after chemotherapy. PMID:23455529

  8. Localized Pigmented Villonodular Synovitis of the Hip: Sudden-Onset Pain Caused by Torsion of the Tumor Pedicle

    PubMed Central

    Kaneuji, Ayumi; Kinoshita, Eriko; Numata, Yuhei; Nojima, Takayuki; Matsumoto, Tadami

    2013-01-01

    Pigmented villonodular synovitis is a rare, benign, but potentially locally aggressive disease that should be considered in younger patients who present with monoarticular joint symptoms and pathology. We present the case of a 33-year-old woman with a mass arising from her right hip joint that was examined using a multimodal radiological approach. Because her clinical presentation mimicked that of synovial osteochondromatosis of the hip, surgical dislocation was performed. Histopathological examination of the resected specimen confirmed the diagnosis of localized pigmented villonodular synovitis, with the mass consisting of proliferation of fibrohistiocytic cells, abundant hemosiderin, foamy histiocytes, and occasional giant cells. Because of the presence of tumor necrosis, we hypothesize that torsion of the tumor pedicle was the cause of acute presentation. PMID:24324906

  9. Atypical Fibroxanthoma

    PubMed Central

    Sakamoto, Akio

    2008-01-01

    Atypical fibroxanthoma (AFX) is a nodular dermal ulcerative lesion with a favorable prognosis. AFX most commonly occurs on sun-exposed skin in elderly individuals. AFX is characterized by its association with ultraviolet radiation, not only from a clinical aspect, but also from a molecular aspect. Making a diagnosis of AFX is challenging, and it is important to differentiate it from squamous cell carcinoma and malignant melanoma. Histological features and combined immunohistochemical markers are necessary for a definitive diagnosis (i.e., an absence of immunostaining for cytokeratins, S100 and HMB45 in AFX is helpful for excluding both squamous cell carcinoma and malignant melanoma). AFX, as well as MFH (malignant fibrous histiocytoma), is a fibrohistiocytic lesion with myofibroblastic differentiation. AFX is considered to be a different lesion from MFH. AFX and MFH might share the same pathway which determines their morphology. However, they may have different pathways in development which determine their biological behavior. PMID:21892274

  10. Images of liposarcoma using technetium-99m bleomycin and technetium (V)-99m DMSA

    SciTech Connect

    Ohta, H.; Shane, F.I.; Endo, K.; Torizuka, K.; Horiuchi, K.; Yokoyama, A.; Ishii, M.

    1986-12-01

    The effectiveness of Tc-99m bleomycin (BLM) and Tc(V)-99m DMSA are compared with that of Ga-67 citrate, which is currently the most widely used agent. In four patients with lipomatous tumors, the clinical significance of tumor imaging with each of these three agents is discussed and compared. Results indicate that both Tc-99m BLM and Tc(V)-99m DMSA are superior in detecting the extension or localization of liposarcomas.

  11. Syndromes with focal overgrowth in infancy: Diagnostic approach and surgical treatment.

    PubMed

    Mirastschijski, Ursula; Altmann, Silke; Lenz-Scharf, Olivia; Muschke, Petra; Schneider, Wolfgang

    2010-02-17

    Abstract Syndromes with focal overgrowth are sporadic diseases and comprise Proteus syndrome and congenital lipomatous overgrowth, vascular malformations, and epidermal naevi (CLOVE) syndrome, and isolated hemihyperplasia. We describe 3 children classified according to standard criteria with dysregulated growth of various tissues that was excised, together with excess toes, and tumours. Correct classification facilitates diagnosis and operations. Interdisciplinary treatment and follow-up are recommended to prevent disfiguration. PMID:19634071

  12. Syndromes with focal overgrowth in infancy: diagnostic approach and surgical treatment.

    PubMed

    Mirastschijski, Ursula; Altmann, Silke; Lenz-Scharf, Olivia; Muschke, Petra; Schneider, Wolfgang

    2012-02-01

    Syndromes with focal overgrowth are sporadic diseases and comprise Proteus syndrome and congenital lipomatous overgrowth, vascular malformations, and epidermal naevi (CLOVE) syndrome, and isolated hemihyperplasia. We describe 3 children classified according to standard criteria with dysregulated growth of various tissues that was excised, together with excess toes, and tumours. Correct classification facilitates diagnosis and operations. Interdisciplinary treatment and follow-up are recommended to prevent disfiguration. PMID:21446802

  13. Dedifferentiated liposarcoma with extensive lymphoid component

    Microsoft Academic Search

    Cornelius Kuhnen; Thomas Mentzel; Raf Sciot; Marcus Lehnhardt; Heinz-Herbert Homann; Maria Debiec-Rychter

    2005-01-01

    An unusual variant of dedifferentiated liposarcoma with extensive lymphocytic component is described. A 71-year-old patient suffered from a relapse of an atypical lipomatous tumor\\/well-differentiated liposarcoma with early micronodular (low-grade) dedifferentiation, which had been resected 4 years before. The relapse revealed features of a dedifferentiated liposarcoma with spindle-cell, partly pleomorphic dedifferentiation and osseous metaplasia. Clearly separated from the spindle-cell areas, an

  14. [Myocardial fibrosis in Shwachman's syndrome (author's transl)].

    PubMed

    Guerrero, J; López Barea, F; Calvo, C; Moreno, F; Solas, I

    1979-01-01

    A case of a child affected by Shwachman's syndrome with a chronic diarrhea, cyclic neutropenia and bone defects is described. The child died at the age of eight months from an acute cardiac failure which showed analytical alterations compatible to an acute myocardial infarction. Pathologic study showed a pancreatic lipomatous hypoplasia, myocardial fibrosis and chondrometaphyseal dysplasia. Main hypothesis that relate pancreatic pathology and development of myocardial fibrosis are discussed, although there is a lack of definitive evidence for confirming such relation. PMID:484946

  15. Infarction of lesser omental fat mimicking an exophytic pancreatic tumor by sonography.

    PubMed

    Kim, Hyun Cheol; Yang, Dal Mo; Jin, Wook; Joh, Jin Hyun

    2011-09-01

    We report a case of infarction of lesser omental fat in a 30-year-old woman who presented with epigastric pain. Sonography revealed a painful echogenic mass in the epigastrium that could not be separated from the adjacent hyperechoic pancreas and mimicked an exophytic pancreatic tumor. On CT, the mass was identified as an inflammatory lipomatous mass in the lesser sac, leading to the diagnosis of infarction of lesser omental fat. The patient was successfully treated conservatively. PMID:21449000

  16. Respiratory Toxicity of Diacetyl in C57BI/6 Mice

    PubMed Central

    Morgan, Daniel L.; Flake, Gordon P.; Kirby, Patrick J.; Palmer, Scott M.

    2009-01-01

    Diacetyl, a component of artificial butter flavoring, is a potential etiological agent of obliterative bronchiolitis (OB); however, the toxic dose and mechanisms of toxicity remain controversial. We evaluated the respiratory toxicity of diacetyl in a murine model using several exposure profiles relevant to workplace conditions at microwave popcorn packaging plants. Male C57B1/6 mice were exposed to inhaled diacetyl across several concentrations and duration profiles, or by direct oropharyngeal aspiration. Effects of diacetyl on the respiratory tract were evaluated by histopathology and BALF analyses. Subacute exposure to 200 or 400 ppm diacetyl for 5 days caused deaths, necrotizing rhinitis, necrotizing laryngitis and bronchitis. Reducing the exposure to 1 h/day (100, 200, 400 ppm) for 4 weeks resulted in less nasal and laryngeal toxicity, but led to peribronchial and peribronchiolar lymphocytic inflammation. A similar pattern was observed with intermittent high-dose exposures at 1200 ppm (15 min, twice a day, 4 weeks). Subchronic exposures to 100 ppm (6 h/day, 12 weeks) caused moderate nasal injury, and peribronchial lymphocytic inflammation accompanied by epithelial atrophy, denudation, and regeneration. Treatment with 400 mg/kg by oropharyngeal aspiration to bypass the nose caused foci of fibrohistiocytic proliferation with little or no inflammation at the junction of the terminal bronchiole and alveolar duct. Depending on the route and duration of exposure, diacetyl causes significant epithelial injury, peribronchial lymphocytic inflammation, or fibrohistiocytic lesions in the terminal bronchioles. Collectively these results indicate that clinically relevant diacetyl exposures result in a pattern of injury that replicates features of human OB. PMID:18227102

  17. Laparoscopic resection of a retroperitoneal myolipoma presenting in a right inguinal hernia?

    PubMed Central

    Dan, Dilip; Bascombe, Nigel; Harnanan, Dave; Naraynsingh, Vijay

    2013-01-01

    INTRODUCTION Myolipoma of soft tissue is an extremely rare benign lipomatous lesion. The lesions are most commonly located in the abdominal cavity, retroperitoneum, and inguinal areas. Despite their large size, myolipomas are cured by surgical resection. PRESENTATION OF CASE We present the case of a 79 year-old man who presented with bilateral reducible inguinal hernias (right larger than left). After reducing the right inguinal hernia (RIH), the sensation of a palpable mass was noted in the right iliac fossa. CT scan suggested the content of the right inguinal hernia (RIH) to be small bowel mesentery and no other mass was noted in the right iliac fossa (possibly missed on CT scan). DISCUSSION A very large 1.8 kg retroperitoneal lipomatous lesion, measuring 22 cm × 16 cm × 8 cm, attached to the right spermatic cord was found and excised laparoscopically during a trans-abdominal pre-peritoneal (TAPP) approach to repair the hernias. The lesion was pathologically defined as a myolipoma. CONCLUSION The laparoscopic TAPP approach to repair inguinal hernias allows the surgeon to inspect the peritoneal cavity, and in this case it was possible to safely dissect and remove a large, lipomatous, retroperitoneal lesion laparoscopically. To the best of our knowledge, there are no reports of local recurrence, metastatic disease, or malignant transformation of myolipomas, and the laparoscopic approach to resect such a lesion has not been reported. PMID:23995475

  18. Utility of fluorescence in situ hybridization to detect MDM2 amplification in liposarcomas and their morphological mimics

    PubMed Central

    Kimura, Hiroaki; Dobashi, Yoh; Nojima, Takayuki; Nakamura, Hiroyuki; Yamamoto, Norio; Tsuchiya, Hiroyuki; Ikeda, Hiroko; Sawada-Kitamura, Seiko; Oyama, Takeru; Ooi, Akishi

    2013-01-01

    The atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDLS) and the de-differentiated liposarcoma (DDLS) represent the most common category of liposarcomas. ALT/WDLSs and DDLSs are often difficult to distinguish from other tumors with similar morphological characteristics. In this study, we investigated whether the detection of amplified or overexpressed murine double-minute 2 (MDM2) can be a useful diagnostic ancillary aid. We used fluorescent in situ hybridization (FISH) and immunohistochemistry (IHC) to detect MDM2 amplification and protein overexpression, respectively, in 49 WDLSs, 5 DDLSs, 23 myxoid liposarcomas, 25 benign lipomatous tumors, and 75 spindle and pleomorphic sarcomas. MDM2 amplification was detected in 48 of 49 WDLSs, 5 of 5 DDLSs, 2 of 9 malignant peripheral nerve sheath tumors, and 2 of 10 myxofibrosarcomas. We did not detect MDM2 amplification in any of the benign lipomatous tumors. FISH-mediated detection of MDM2 amplification was the most valuable diagnostic aid for ALT/WDLS, as determined by using the Fisher exact test to compare two different diagnoses of 19 biopsies. On the contrary, unequivocal nuclear overexpression of MDM2 was found in only 10 of 50 ALT/WDLSs. The sensitivity and specificity of MDM2 amplification in distinguishing a DDLS from spindle and pleomorphic sarcomas were 100% and 95%, respectively, while those of MDM2 overexpression were 100% and 87%, respectively. In conclusion, our results indicate that FISH-mediated detection of MDM2 amplification is the most useful adjunct in the diagnosis of both ALT/WDLS and DDLS. However, IHC-mediated detection of MDM2 protein is useful only for the diagnosis of DDLS. PMID:23826411

  19. Myolipoma of Mesentery: A Case Report

    PubMed Central

    Kim, Hyun Sung; Kim, Suk; Kim, Kyungbin; Choi, Kyung Un; Kim, Joo Youn

    2013-01-01

    Myolipomas are very rare benign lipomatous soft tissue tumors which are usually located in retroperitoneum, abdominal and pelvic cavity, and the abdominal wall. They can be diagnosed histologically by the presence of irregularly admixed mature adipose tissue and smooth muscle fibers. The correct diagnosis of myolipoma is important, because it should be considered in the differential diagnosis of fat-containing lesions of the soft tissue and should follow a benign clinical course despite its frequently large size and deep location. We report here a case of myolipoma arising in the mesentery of the jejunum. PMID:24459598

  20. Identical twins with lumbosacral lipomyelomeningocele.

    PubMed

    Hanaei, Sara; Nejat, Farideh; Mortazavi, Abolghasem; Habibi, Zohreh; Esmaeili, Arash; El Khashab, Mostafa

    2015-01-01

    Lipomyelomeningocele, a congenital spine defect, is presented as skin-covered lipomatous tissue that attaches to the cord in different ways according to its subtypes. Unlike other types of neural tube defects, the exact cause of this birth defect has not been confirmed yet, but it is proposed to be a multifactorial disease with involvement of both genetic and environmental factors. The authors describe identical twins with lipomyelomeningocele of the same subtype and location without any familial history of similar abnormality. Therefore, the same genetic and/or environmental risk factors could have played a part in their condition. PMID:25396701

  1. Surgical removal of a canine orbital lipoma.

    PubMed

    Williams, D L; Haggett, E

    2006-01-01

    A 10-year-old, female, neutered Cairn terrier was presented with a progressively enlarging, cream-white fluctuant subconjunctival swelling in the right eye. A fine-needle aspirate performed under topical anaesthesia showed that the mass contained lipomatous tissue. Orbital ultrasonography showed the mass to have a distinct border and to extend into the posterior orbit. The mass was removed via a conjunctival incision. It had a distinct capsule anteriorly, while the border of the mass was less readily identified in the posterior orbit. Histopathological examination showed the mass to be a lipoma. The dog recovered uneventfully from surgery, and no recurrence has been noted after 12 months. PMID:16417609

  2. Cervical hibernoma in a two year old boy

    PubMed Central

    Khattala, Khalid; Elmadi, Aziz; Bouamama, Hanane; Rami, Mohamed; Bouabdallah, Youssef

    2013-01-01

    Hibernomas are uncommon benign soft tissue tumours mimicking brown fat. They are mostly seen in the fourth and fifth decades of life. Only few cases in the cervical area have been reported. Because of its rarity in pediatrics and diffcult diagnosis, we report a tow year-old patient with a cervical tumor. Ultrasound and computed tomography exams showed an infiltrative, with hypervascular and lipomatous features. After tumor excision, histopathological exam confirmed the diagnosis of hibernoma or brown fat tumor. This presentation describes the characteristics of this type of tumor, rare in children. PMID:24570788

  3. Schwannomatous components in a recurrent lipomyelocele: Report of a rare case.

    PubMed

    Mondal, Santosh Kumar

    2013-10-01

    Congenital lipomatous malformations of spinal cord constitute a diverse group of lesions and lipomyelocele is one of them. Here, we report a case of congenital lipomyelocele in a male child who presented at 7 years of age. Magnetic resonance imaging (MRI) revealed a lesion in lumbosacral region. The patient was operated and histologic examination diagnosed the case to be lipomyelocele. But after 8 years, recurrence of the lesion occurred and MRI revealed the lesion at the same location. This time histologic examination detected another new component (schwannomatous areas) in the lesion. The child was on 1-year follow-up which was uneventful. PMID:24551006

  4. Shwachman-Bodian-Diamond syndrome: metaphyseal chondrodysplasia in children with pancreatic insufficiency and neutropenia.

    PubMed

    Levin, Terry L; Mäkitie, Outi; Berdon, Walter E; Lachman, Ralph S

    2014-11-23

    Shwachman-Bodian-Diamond syndrome (OMIM 260400) was identified in 1964 by pediatricians Harry Shwachman, a leader in cystic fibrosis, and Louis K. Diamond, a hematologist, along with pediatrician and morbid anatomist Martin Bodian. Initially the syndrome's clinical presentation included exocrine pancreatic insufficiency (lipomatous replacement of the pancreas) and neutropenia. In 1967 skeletal changes of metaphyseal chondrodysplasia were also described, completing the triad of findings; these abnormalities are present in all affected children and should be viewed as an integral feature of the syndrome, also called Shwachman-Diamond syndrome. PMID:25416932

  5. Perirenal myelolipoma diagnosed on imprint: case report and review of the literature.

    PubMed

    Brandler, Tamar C; Reder, Ilan; Kahn, Leonard

    2015-03-01

    Myelolipomas are benign tumors containing adipose tissue and hematopoeitic elements which occur most commonly in the adrenal glands. Extra-adrenal myelolipomas are uncommon with few cases of renal and perirenal myelolipomas in the literature. These cases may be difficult to diagnose based on radiology alone and intraoperative diagnosis can be extremely important in determining the course of surgery. Due to the lipomatous component of the tumor it may be technically difficult to prepare adequate frozen sections; this problem can be obviated by utilization of cytologic imprint preparations. We present a case of perirenal myelolipoma diagnosed intraoperatively utilizing cytologic imprint preparations stained with toluidine blue and hematoxylin and eosin. PMID:24895157

  6. Congenital infiltrating lipomatosis of the face with exophytic temporomandibular joint ankylosis: a case report and review of the literature

    PubMed Central

    Sahai, S; Rajan, S; Singh, N; Arora, H

    2013-01-01

    Congenital infiltrating lipomatosis of the face (CIL-F) is a rare lipomatous lesion with diffuse fatty infiltration of tissues and hyperplasia of underlying bone. We report clinical and CT findings in an unusual case of CIL-F presenting with progressive hemifacial asymmetry, manifesting as severely restricted mouth opening owing to exophytic temporomandibular joint ankylosis. The role of imaging in diagnosis is presented with a review of the literature. Differential diagnosis of CIL-F and its exclusion as a cause of hemifacial hyperplasia are also discussed. PMID:22241871

  7. Labile iron in cells and body fluids: physiology, pathology, and pharmacology

    PubMed Central

    Cabantchik, Zvi Ioav

    2014-01-01

    In living systems iron appears predominantly associated with proteins, but can also be detected in forms referred as labile iron, which denotes the combined redox properties of iron and its amenability to exchange between ligands, including chelators. The labile cell iron (LCI) composition varies with metal concentration and substances with chelating groups but also with pH and the medium redox potential. Although physiologically in the lower ?M range, LCI plays a key role in cell iron economy as cross-roads of metabolic pathways. LCI levels are continually regulated by an iron-responsive machinery that balances iron uptake versus deposition into ferritin. However, LCI rises aberrantly in some cell types due to faulty cell utilization pathways or infiltration by pathological iron forms that are found in hemosiderotic plasma. As LCI attains pathological levels, it can catalyze reactive O species (ROS) formation that, at particular threshold, can surpass cellular anti-oxidant capacities and seriously damage its constituents. While in normal plasma and interstitial fluids, virtually all iron is securely carried by circulating transferrin (Tf; that renders iron essentially non-labile), in systemic iron overload (IO), the total plasma iron binding capacity is often surpassed by a massive iron influx from hyperabsorptive gut or from erythrocyte overburdened spleen and/or liver. As plasma Tf approaches iron saturation, labile plasma iron (LPI) emerges in forms that can infiltrate cells by unregulated routes and raise LCI to toxic levels. Despite the limited knowledge available on LPI speciation in different types and degrees of IO, LPI measurements can be and are in fact used for identifying systemic IO and for initiating/adjusting chelation regimens to attain full-day LPI protection. A recent application of labile iron assay is the detection of labile components in intravenous iron formulations per se as well as in plasma (LPI) following parenteral iron administration. PMID:24659969

  8. Combined liposuction and excision of lipomas: long-term evaluation of a large sample of patients.

    PubMed

    Copeland-Halperin, Libby R; Pimpinella, Vincenza; Copeland, Michelle

    2015-01-01

    Background. Lipomas are benign tumors of mature fat cells. They can be removed by liposuction, yet this technique is seldom employed because of concerns that removal may be incomplete and recurrence may be more frequent than after conventional excision. Objectives. We assessed the short- and long-term clinical outcomes and recurrence of combined liposuction and limited surgical excision of subcutaneous lipomas. Methods. From 2003 to 2012, 25 patients with 48 lipomas were treated with liposuction followed by direct excision through the same incision to remove residual lipomatous tissue. Initial postoperative follow-up ranged from 1 week to 3 months, and long-term outcomes, complications, and recurrence were surveyed 1 to 10 years postoperatively. Results. Lipomas on the head, neck, trunk, and extremities ranged from 1 to 15?cm in diameter. Early postoperative hematoma and seromas were managed by aspiration. Among 23 survey respondents (92%), patients were uniformly pleased with the cosmetic results; none reported recurrent lipoma. Conclusions. The combination of liposuction and excision is a safe alternative for lipoma removal; malignancy and recurrence are uncommon. Liposuction performed through a small incision provides satisfactory aesthetic results in most cases. Once reduced in size, residual lipomatous and capsular tissue can be removed without expanding the incision. These favorable outcomes support wider application of this technique in appropriate cases. PMID:25694827

  9. Hemihyperplasia-multiple lipomatosis syndrome (HHML): a challenge in spinal care.

    PubMed

    Schulte, Tobias L; Liljenqvist, Ulf; Görgens, Heike; Hackenberg, Lars; Bullmann, Viola; Tinschert, Sigrid

    2008-10-01

    A 15-year-old girl developed a progressive paraparesis over a period of six months, secondary to spinal cord compression by a lipomatous mass and anomalies of the vertebral column. Clinically, a right hemihyperplasia affecting the trunk and lower limb was evident, as well as a right convex lumbar scoliosis. CT and MRI demonstrated severe spinal cord compression resulting from intraspinal lipomatosis, overgrowth of right facet joints (T8 to L5), and kyphoscoliosis. Surgical decompression was undertaken. A lumbar scoliosis of 48 degrees was partially corrected by means of dual-rod instrumentation. The neurological deficit improved significantly, and ambulation was progressively restored. The patient carried the diagnosis of Proteus syndrome for several years, but reevaluation of clinical features prompted the diagnosis of Hemihyperplasia Multiple Lipomatosis syndrome (HHML). This rare sporadic disorder is often confused with Proteus syndrome. As in Proteus syndrome, spinal cord compression in patients with HHML can result from lipomatous infiltration and/or significant spinal abnormalities including kyphoscoliosis and overgrowth. HHML and Proteus syndrome are discussed and compared with special emphasis on spinal and orthopaedic pathologies. PMID:19058713

  10. Combined Liposuction and Excision of Lipomas: Long-Term Evaluation of a Large Sample of Patients

    PubMed Central

    Copeland-Halperin, Libby R.; Pimpinella, Vincenza

    2015-01-01

    Background. Lipomas are benign tumors of mature fat cells. They can be removed by liposuction, yet this technique is seldom employed because of concerns that removal may be incomplete and recurrence may be more frequent than after conventional excision. Objectives. We assessed the short- and long-term clinical outcomes and recurrence of combined liposuction and limited surgical excision of subcutaneous lipomas. Methods. From 2003 to 2012, 25 patients with 48 lipomas were treated with liposuction followed by direct excision through the same incision to remove residual lipomatous tissue. Initial postoperative follow-up ranged from 1 week to 3 months, and long-term outcomes, complications, and recurrence were surveyed 1 to 10 years postoperatively. Results. Lipomas on the head, neck, trunk, and extremities ranged from 1 to 15?cm in diameter. Early postoperative hematoma and seromas were managed by aspiration. Among 23 survey respondents (92%), patients were uniformly pleased with the cosmetic results; none reported recurrent lipoma. Conclusions. The combination of liposuction and excision is a safe alternative for lipoma removal; malignancy and recurrence are uncommon. Liposuction performed through a small incision provides satisfactory aesthetic results in most cases. Once reduced in size, residual lipomatous and capsular tissue can be removed without expanding the incision. These favorable outcomes support wider application of this technique in appropriate cases. PMID:25694827

  11. Objective parameters aid the prediction of fistulas in pancreatic surgery.

    PubMed

    Felix, Klaus; Schuck, Anna; Gaida, Matthias M; Hinz, Ulf; Dovzhanskiy, Dmitriy; Werner, Jens

    2014-09-01

    Insufficiency of pancreatic anastomosis with leakage from the pancreatic stump and the development of fistulas account for the majority of surgical complications following pancreatic resection, which are often life threatening. The cause of pancreatic fistulas of the remnant tissue on a molecular level remains unclear. Thus, the aim of the present study was to investigate risk factors associated with postoperative pancreatic fistula (POPF) formation and to define parameters that may predict the resection outcome. Pancreatic resection margins were selected from 31 patients, including 16 individuals without and 15 patients with POPF, to analyze the degree of fibrosis, lipomatous atrophy, inflammatory activity and infiltration. Wound healing factors were assessed by luminex technology using tissue homogenates, while the distribution in situ was assessed using immunohistochemistry. Increased chronic inflammatory infiltration, a higher degree of fibrosis and a reduction in lipomatous atrophy were observed in the samples without anastomotic fistulas. Multiplex analysis of 38 wound healing factors demonstrated significantly higher levels of interleukin (IL)-6, -8 and -12, glucagon-like peptide-1 and matrix metalloproteinase (MMP)-1, -2, -3 and -12 in the group without fistulas, while lower concentrations of IL-10, IL-17 and gastric inhibitory polypeptide were observed. Therefore, the observations of the present study indicated that increased inflammatory infiltration and inflammatory activity, as well as higher concentrations of proinflammatory cytokines and higher MMP levels at the resection margins, predisposed individuals to a lower fistula incidence rate following pancreatic resection. PMID:25120588

  12. Objective parameters aid the prediction of fistulas in pancreatic surgery

    PubMed Central

    FELIX, KLAUS; SCHUCK, ANNA; GAIDA, MATTHIAS M.; HINZ, ULF; DOVZHANSKIY, DMITRIY; WERNER, JENS

    2014-01-01

    Insufficiency of pancreatic anastomosis with leakage from the pancreatic stump and the development of fistulas account for the majority of surgical complications following pancreatic resection, which are often life threatening. The cause of pancreatic fistulas of the remnant tissue on a molecular level remains unclear. Thus, the aim of the present study was to investigate risk factors associated with postoperative pancreatic fistula (POPF) formation and to define parameters that may predict the resection outcome. Pancreatic resection margins were selected from 31 patients, including 16 individuals without and 15 patients with POPF, to analyze the degree of fibrosis, lipomatous atrophy, inflammatory activity and infiltration. Wound healing factors were assessed by luminex technology using tissue homogenates, while the distribution in situ was assessed using immunohistochemistry. Increased chronic inflammatory infiltration, a higher degree of fibrosis and a reduction in lipomatous atrophy were observed in the samples without anastomotic fistulas. Multiplex analysis of 38 wound healing factors demonstrated significantly higher levels of interleukin (IL)-6, -8 and -12, glucagon-like peptide-1 and matrix metalloproteinase (MMP)-1, -2, -3 and -12 in the group without fistulas, while lower concentrations of IL-10, IL-17 and gastric inhibitory polypeptide were observed. Therefore, the observations of the present study indicated that increased inflammatory infiltration and inflammatory activity, as well as higher concentrations of proinflammatory cytokines and higher MMP levels at the resection margins, predisposed individuals to a lower fistula incidence rate following pancreatic resection. PMID:25120588

  13. Lymphohistiocytoid mesothelioma: a rare lymphomatoid variant of predominantly sarcomatoid mesothelioma

    SciTech Connect

    Henderson, D.W.; Attwood, H.D.; Constance, T.J.; Shilkin, K.B.; Steele, R.H.

    1988-01-01

    Of 394 ''definite'' mesotheliomas entered in the Australian Mesothelioma Surveillance Program, three bore a striking resemblance to malignant lymphoma by conventional light microscopy, and each was misinterpreted at some stage as lymphoma. The lymphoma-like morphology was a combined result of intense lymphoplasmacytic infiltration and the histiocytoid appearances of the underlying neoplastic cell population. Immunocytochemical analysis demonstrated cytokeratins coexpressed with vimentin within the tumor cells, whereas immunoreactivity for leukocyte common antigen was confined to the smaller lymphoid cells. Electron microscopy of two cases revealed a polymorphous population of fibrohistiocytic cells resembling those typical of malignant fibrous histiocytoma, admixed with lymphocytes and plasma cells, but sporadic cells expressed mesothelial properties in the form of sinuous villiform processes, intracytoplasmic neolumina lined by microvilli, and intermediate filaments that were aggregated into tonofilament bundles in some cells. The ultrastructural appearances, the localization of the tumors to the pleura, with effusion, and absence of anterior mediastinal mass lesions facilitated exclusion of lymphocyte-rich thymoma. In addition, a history of prior occupational exposure to asbestos was elicited in each instance. There was no apparent response to radiotherapy or chemotherapy, and the patients died at 4, 5, and 8 months after presentation. Our observations suggest that immunocytochemical or ultrastructural evaluation is mandatory for accurate diagnosis of all pleura-based lymphomatoid lesions with a mixed large and small cell pattern. 42 references.

  14. Congenital diffuse infiltrating facial lipomatosis

    PubMed Central

    Balaji, S. M.

    2012-01-01

    Congenital diffuse infiltrating lipomatosis of the face (CDIL-F) is a rare pathological entity belonging to the subgroup of lipomatous tumors. Till date only a handful of cases has been documented and known to occur exclusively in infancy. On microscopical examination, it is characterized by diffuse infiltration of mature adipose tissue over normal muscle fibers, rapid growth, associated osseous hyperplasia, and a high recurrence rate after surgical intervention. An attempt has been made to identify and characterize all the 49 documented cases of CDIL-F in literature along with describing a report of a male child with CDIL-F. Follow-up of 8 years has been documented. The pathogenesis and spectrum of treatment modality are discussed with identified clinical features. PMID:23483013

  15. Enlarged pancreas: not always a cancer.

    PubMed

    Calculli, Lucia; Festi, Davide; Pezzilli, Raffaele

    2015-02-01

    Pancreatic fat accumulation has been described with various terms including pancreatic lipomatosis, pancreatic steatosis, fatty replacement, fatty infiltration, fatty pancreas, lipomatous pseudohypertrophy and nonalcoholic fatty pancreas disease. It has been reported to be associated with type 2 diabetes mellitus, acute pancreatitis, pancreatic cancer and the formation of pancreatic fistula. The real incidence of this condition is still unknown. We report a case of pancreatic steatosis in a non-obese female patient initially diagnosed with a mass in the head of the pancreas. Magnetic resonance imaging (MRI) was carried out to define the characteristics of the pancreatic mass. MRI confirmed the diagnosis of fat pancreas. Enlarged pancreas is not always a cancer, but pancreatic steatosis is characterized by pancreatic enlargement. MRI could give a definite diagnosis of pancreatic steatosis or cancer. PMID:25655299

  16. Idiopathic renal replacement lipomatosis: a rare renal pseudo tumor.

    PubMed

    Wahal, Shailja P; Mardi, Kavita

    2014-01-01

    Renal replacement lipomatosis (RRL) is a rare condition that occurs as an end result of renal atrophy or destruction of renal parenchyma by excessive lipomatous tissue in renal sinus and perinephric space. We report a case of RRL presenting as a right renal mass clinically. Intra-venous pyelography and renal scan revealed a left non-functioning kidney. A left nephrectomy was performed. After histopathological examination and extensive literature search, a diagnosis of RRL was given. In majority of cases, it is associated with renal calculi. Idiopathic variety is not common. It is a rare entity and the diagnosis may be missed due to lack of experience. It has to be differentiated from other fat-containing tumors such as renal lipoma, xanthogranulomatous pyelonephritis, angiomyolipoma and liposarcoma. PMID:25022415

  17. Myelolipomatous adrenal masses causing Cushing's syndrome.

    PubMed

    Lamas, C; López, L M; Lozano, E; Atienzar, M; Ruiz-Mondéjar, R; Alfaro, J J; Botella, F

    2009-09-01

    Adrenal myelolipomas are uncommon benign tumors, composed of mature adipose tissue and haematopoietic elements in varying proportions. They are usually asymptomatic, non-functioning adrenal incidentalomas, but there have been a few reports of myelolipomatous masses associated with adrenocortical hypersecretion. We report two cases of large mixed adrenal tumors, with heterogeneous appearance and areas of fat density in imaging techniques, and with autonomous cortisol production leading to Cushing's syndrome. Both underwent adrenalectomy and the histological study showed an adrenocortical adenoma with widespread myelolipomatous metaplasia. Hypercortisolism resolved in the one patient that could be evaluated after surgery. We review all the previous reported cases of hypercortisolism associated with adrenal myelolipomas. We also discuss the recommended diagnostic approach and therapeutic management of adrenal masses of lipomatous appearance. PMID:19373749

  18. Liposarcoma in clownfish, Amphiprion ocellaris Cuvier, produced in indoor aquaculture.

    PubMed

    Sharon, G; Benharroch, D; Kachko, L; Reis-Hevlin, N; Zilberg, D

    2015-06-01

    Clownfish, Amphiprion ocellaris Cuvier, produced and grown in an experimental indoor aquaculture facility, presented with lipomatous tumours. A total of 14 affected fish were examined. Based on the total number of fish at the aquaculture facility at the time of outbreak of this pathology, the scope of the incident is estimated to be 1 of 300 fish. The tumours were characterized by the presence of mature adipocytes of variable sizes, lipoblasts and by an invasive behaviour, which affected internal organs, muscle, central nervous system and, in one case, an eye. Detailed macroscopic and histopathological features are presented. The suggested diagnosis is that of a well-differentiated liposarcoma, a diagnosis so far never applied to fish. The limited outbreak of the neoplasm lasted a few months in 2011 and did not recur. Possible factors leading to this phenomenon, notably the metastasis, are discussed. PMID:24917512

  19. Angiolymphoid hyperplasia with eosinophilia--an incidental finding after surgical excision.

    PubMed

    Tchernev, Georgi; Taneva, Teodora; Ananiev, Julian; Cardoso, José Carlos; Gulubova, Maya; Velev, Valentin; Karashmalakov, Anatoli; Zisova, Liliya; Geilen, Christoph C

    2012-10-01

    Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vasoproliferative lesion. Although it is a benign disease, lesions are often persistent and difficult to eradicate. ALHE typically presents clinically as papules or nodules, tan, brown, pink or dull red in colour, located predominantly in the head and neck region, especially around the ears and on the forehead and scalp.All races can be affected and no gender predominance exists. The disease also has nonspecific clinical features, hence it requires in most of the cases biopsy for accurate diagnosis. We present an uncommon clinical presentation of the disease, mimicking clinically a subcutaneous lipomatous mass, which has been treated successfully with surgical excision. PMID:22956004

  20. Compression of the Superior Vena Cava by an Interatrial Septal Lipoma: A Case Report

    PubMed Central

    Grech, R.; Mizzi, A.; Grech, S.

    2013-01-01

    Primary cardiac tumours are rare; their prevalence ranges from 0.0017% to 0.28% in various autopsy series. Cardiac lipomas are well-encapsulated benign tumours typically composed of mature fat cells, and their reported size ranges from 1 to 15?cm. They are usually seen in the left ventricle and the right atrium. Lipomas are true neoplasms, as opposed to lipomatous hypertrophy of the interatrial septum, which is a nonencapsulated hyperplastic accumulation of mature and foetal adipose tissue. Cardiac lipomas occur in patients of all ages, and the frequency of occurrence has been found to be equal in both sexes. Patients are usually asymptomatic, although the manifestation of symptoms depends upon both size and location of the tumour. We present the case of a patient with an interatrial septal lipoma, causing obstruction of the superior vena cava. PMID:23984155

  1. Thymolipoma with sebaceous differentiation: a hitherto unreported variant of thymolipoma.

    PubMed

    Ajaz, Bushra; Tran, Tien Anh Nguyen; Truong, Tuan; Manoucheri, Manoucher

    2013-10-01

    Thymolipoma is a very rare anterior mediastinal mass of thymic origin, accounting for only 2% to 10% of all thymic neoplasm. Histologically, the tumor is usually composed of adipose tissue interspersed with thymic tissues. We report a case of a 58-year-old man who presented with a few weeks history of shortness of breath, cough, and chest pain, found to have a retrocardiac infiltrate on chest X-ray. Computed tomography scan of the chest showed a large anterior mediastinal mass. Surgical excision revealed an ovoid fatty mass, which on histological examination displayed thymic tissue intermixed with lipomatous tissue consistent with a thymolipoma. Of note was the presence of a microscopic nodule of thymic epithelial proliferation with sebaceous differentiation and focal cylindroma-like architecture. Although sebaceous tissue has been rarely reported in thymic tissue, to the best of our knowledge, this is the first case report of sebaceous differentiation in a thymolipoma. PMID:23358435

  2. Neoplasms involving the heart, their simulators, and adverse consequences of their therapy

    PubMed Central

    2001-01-01

    Primary cardiac tumors involving the heart may be either benign or malignant. Most of the benign tumors are myxomas, which are most commonly located in the left atrium. Primary malignant neoplasms usually involve the myocardium and the interior of the cardiac cavities, whereas neoplasms metastatic to the heart most commonly involve pericardium, and pericardial effusion and constriction are the most common consequences. Computed tomography and magnetic resonance imaging are becoming the most useful instruments of precision for the diagnosis of cardiac tumors. Pericardial cysts, teratomas, lipomatous hypertrophy of the atrial septum, papillary fibroelastomas, thrombi, and sarcoid are frequently mistaken for cardiac neoplasms. There are a number of cardiac consequences of malignancy, including radiation heart disease, cardiac hemorrhages, cardiac infection, cardiac adiposity or the corticosteroidtreated heart, cardiac hemosiderosis, and toxicity due to anthracycline chemotherapy. PMID:16369647

  3. Functional Genomics Analysis Reveals a MYC Signature Associated with a Poor Clinical Prognosis in Liposarcomas.

    PubMed

    Tran, Dat; Verma, Kundan; Ward, Kristin; Diaz, Dolores; Kataria, Esha; Torabi, Alireza; Almeida, Anna; Malfoy, Bernard; Stratford, Eva W; Mitchell, Dianne C; Bryan, Brad A

    2015-03-01

    Liposarcomas, which are malignant fatty tumors, are the second most common soft-tissue sarcomas. Several histologically defined liposarcoma subtypes exist, yet little is known about the molecular pathology that drives the diversity in these tumors. We used functional genomics to classify a panel of diverse liposarcoma cell lines based on hierarchical clustering of their gene expression profiles, indicating that liposarcoma gene expression profiles and histologic classification are not directly correlated. Boolean probability approaches based on cancer-associated properties identified differential expression in multiple genes, including MYC, as potentially affecting liposarcoma signaling networks and cancer outcome. We confirmed our method with a large panel of lipomatous tumors, revealing that MYC protein expression is correlated with patient survival. These data encourage increased reliance on genomic features in conjunction with histologic features for liposarcoma clinical characterization and lay the groundwork for using Boolean-based probabilities to identify prognostic biomarkers for clinical outcome in tumor patients. PMID:25622542

  4. Somatic Mosaic Activating Mutations in PIK3CA Cause CLOVES Syndrome

    PubMed Central

    Kurek, Kyle C.; Luks, Valerie L.; Ayturk, Ugur M.; Alomari, Ahmad I.; Fishman, Steven J.; Spencer, Samantha A.; Mulliken, John B.; Bowen, Margot E.; Yamamoto, Guilherme L.; Kozakewich, Harry P.W.; Warman, Matthew L.

    2012-01-01

    Congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) is a sporadically occurring, nonhereditary disorder characterized by asymmetric somatic hypertrophy and anomalies in multiple organs. We hypothesized that CLOVES syndrome would be caused by a somatic mutation arising during early embryonic development. Therefore, we employed massively parallel sequencing to search for somatic mosaic mutations in fresh, frozen, or fixed archival tissue from six affected individuals. We identified mutations in PIK3CA in all six individuals, and mutant allele frequencies ranged from 3% to 30% in affected tissue from multiple embryonic lineages. Interestingly, these same mutations have been identified in cancer cells, in which they increase phosphoinositide-3-kinase activity. We conclude that CLOVES is caused by postzygotic activating mutations in PIK3CA. The application of similar sequencing strategies will probably identify additional genetic causes for sporadically occurring, nonheritable malformations. PMID:22658544

  5. Spindle cell lipoma of the parapharyngeal space: first report of a case.

    PubMed

    Baumann, I; Dammann, F; Horny, H P; Plinkert, P K

    2001-04-01

    Spindle cell lipomas are usually located in the subcutaneous tissue of the back, shoulders, and neck. To our knowledge, the presence of such a tumor in the parapharyngeal space has not yet been described. We evaluated a 45-year-old man with a tender swelling of the right parotid area that had reached the submandibular area. Clinical examination and magnetic resonance imaging revealed the presence of a tumor that coated the parotid area laterally and extended into the center of the parapharyngeal space, thus causing a dislocation of the pharyngeal muscles and mucosa. We performed a total parotidectomy and submandibulectomy on the right side and extirpated the parapharyngeal tumor. We were able to spare the facial nerve, and no facial paralysis occurred. Histologic examination revealed an atypical lipomatous tumor with a remarkably large portion of spindles. PMID:11338650

  6. [Johanson-Blizzard's syndrome: another cause of pancreatic lipomatosis (author's transl)].

    PubMed

    Bresson, J L; Schmitz, J; Saudubray, J M; Lesec, G; Hummel, J A; Rey, J

    1980-01-01

    A new case of Johanson-Blizzard's syndrome is reported. It concerns a boy born to consanguineous parents and who died at the age of 10 months from malnutrition. Anal imperforation, alar agenesia, hair anomalies, mental retardation and external pancreatic failure were associated. Neither deafness nor hypothyroidism appeared to be present. Autopsy revealed lipomatous hypoplasia of the exocrine pancreas, hitherto unobserved in this syndrome, and probably responsible for the external pancreatic failure noted in published cases. The variability within a given family of the Johanson-Blizzard malformative syndrome is illustrated by two other cases reported in the anamnesis, one involving a brother who had died earlier with cutaneous aplasia at the fontanella and lacrimal canal malposition and one involving a second cousin who presented with isolated anal imperforation. PMID:7469679

  7. Shwachman's syndrome and leukaemia.

    PubMed

    Caselitz, J; Klöppel, G; Delling, G; Grüttner, R; Holdhoff, U; Stern, M

    1979-12-01

    The clinical and morphological characteristics of Shwachman's syndrome (exocrine pancreatic insufficiency, pancytopenia, skeletal changes) were observed in a boy who, at the age of 8 years, developed a juvenile form of chronic myeloic leukemia which did not respond to cytostatic treatment. Autopsy revealed a striking lipomatous atrophy of the pancreas, defects in the ossification zones of the bones and marked dwarfism. In addition there was leukaemic infiltration of the pancreas, the spleen, the liver and the lymph nodes. The association of Shwachman's syndrome with leukaemia is a rare, but remarkable complication of this entity because of its relationship to the preceeding pancytopenia. Thorough follow-up of the haematological status of patients with Shwachman's syndrome is recommended. PMID:162096

  8. Dedifferentiated liposarcoma of thigh with chondrosarcomatous dedifferentiated component.

    PubMed

    Yoon, Richard S; Benevenia, Joseph; Beebe, Kathleen S; Hameed, Meera

    2010-11-01

    Liposarcomas are common soft-issue sarcomas arising predominantly in deep soft tissue and the retroperitoneum with varied mortality and recurrence rates, largely dependent on histologic type. Thought to arise de novo, liposarcomas typically are classified into 5 types based on strict morphologic characteristics: well-differentiated, dedifferentiated, myxoid, round cell, and pleomorphic. More specifically, dedifferentiated liposarcoma, a common type most prevalent in the retroperitoneum, often has 2 distinct components, a well-differentiated lipomatous component and a dedifferentiated nonlipomatous component composed of sarcomas, such as myxofibrosarcomas or other spindle-cell sarcomas. Morphology typically ranges from low- to high-grade components, most commonly exhibiting myxofibrosarcoma and malignant fibrous histiocytoma components. However, the case reported in this article is unique-the dedifferentiated component exhibited only chondrosarcomatous differentiation-and it is, to our knowledge, the first such case to be described. PMID:21623423

  9. Retroperitoneal Liposarcoma With Leiomyosarcomatous Differentiation

    PubMed Central

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

    2014-01-01

    We herein describe a 60-year-old Japanese man with a giant retroperitoneal liposarcoma undergoing leiomyosarcomatous differentiation. He was admitted to our hospital because of a 5-month history of dysphagia and abdominal distention. Abdominal computed tomography showed a giant tumor that occupied the entire retroperitoneal space. The majority of the mass was lipomatous and low density; both a heterogenous and solid mass were also present. A giant retroperitoneal liposarcoma was diagnosed, and tumor resection was performed. At surgery, the tumor was mostly isolated from the retroperitoneum and other organs. Histopathologically, the tumor comprised well-differentiated and dedifferentiated liposarcoma with heterologous differentiation of the leiomyosarcomatous components, which is a rare phenomenon in liposarcoma. The patient was alive 3 years after the first treatment, although he has had 3 local recurrences (approximately one recurrence yearly) and has been treated by repeated resection and radiotherapy. PMID:24444269

  10. Ultrasound-guided fine-needle aspiration of a posterior neck dedifferentiated liposarcoma with MDM2 fluorescence in situ hybridization performed on a Pap-stained smear.

    PubMed

    Zreik, Riyam; Soyalp, Krystal; Ruiz, Steve; Ward, Russell; Dobin, Sheila; Chen, Xiangbai; Liu, Lina; Rao, Arundhati

    2015-04-01

    Head and neck liposarcomas, while rare, tend to be subcutaneous and well-differentiated. Dedifferentiated liposarcomas of the head and neck are exceedingly rare in the literature. We present a case of a dedifferentiated liposarcoma arising in the soft tissue of the posterior neck of an 86-year-old man and diagnosed by fine-needle aspiration. Aspirate smears showed a dual population of atypical lipomatous and spindled cells. MDM2 (murine double minute 2) amplification was demonstrated on a Pap-stained smear using fluorescence in situ hybridization (FISH). To the best of our knowledge, this is the first report of MDM2 FISH amplification in a liposarcoma performed on an aspirate smear. Diagn. Cytopathol. 2015;43:320-324. © 2014 Wiley Periodicals, Inc. PMID:25132684

  11. Myxolipoma of the renal capsule: A case report

    PubMed Central

    Cakiroglu, Basri; Tas, Tuncay; Esen, Tar?k; Ates, Lora; Aksoy, Suleyman Hilmi

    2014-01-01

    INTRODUCTION Although lipomas are the most common mesenchymal tumors of the human body, primary intrarenal lipomas are quite rare. In this report we present a case of benign mesenchymal tumor with lipomatous and myxoid components. PRESENTATION OF CASE A sixty one years old male patient was admitted to our outpatient clinic for a general control since he had a right radical nephrectomy operation due to renal cell carcinoma (RCC) eight years ago and he did not have any urological control for last 3 years. However the urinary ultrasound revealed a mass lesion on left kidney and then on axial contrast-enhanced computed tomography (CT) scan, there were two masses on the left kidney. In the magnetic resonance imaging (MRI), the tumor on cortex was depicted as a homogeneous low-signal intensity on the T1-weighted pulse sequence and as a heterogeneous high-signal intensity on the T2-weighted pulse sequence. In pathological evaluation, the biopsy material of the cortical mass was a tumoral lesion containing lipomatous and mixoid areas without atypia, mitosis or necrosis which was diagnosed as myxolipoma. DISCUSSION Myxolipoma, an uncommon type of lipoma, is a benign tumor composed mainly of fat cells with myxoid (mucus-like) components. In our case, the tumor was composed of mature adipocytes together with areas rich in mucoid substances and there were no malignant features including lipoblasts, mitosis or abundant capillary network. CONCLUSION Herein we present a case of a fatty tumor originating from the renal capsule with the histologic diagnosis of myxolipoma. To the best of our knowledge, myxolipoma, a very rare form of lipoma, is not reported in kidney, in the literature before. PMID:25524301

  12. Large benign submucosal lipoma presented with descending colonic intussusception in an adult

    PubMed Central

    Allos, Ziad; Zhubandykova, Dina

    2013-01-01

    Patient: Female, 34 Final Diagnosis: Lipoma of the large intestine Symptoms: Abdominal pain • bloating • blood in stool • constipation • lose of appetite • nausea Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Lipoma of the large intestine is rare, account for only 5% of all gastrointestinal tumors. Lipomas are usually asymptomatic but rarely may cause bleeding, obstruction and intussusception. We present a case of a giant colonic lipoma causing descending-colonic intussusception. Case Report: 34 yo F presented with the intermittent left lower quadrant abdominal pain for 3 weeks. The pain initially was associated with bloating and constipation and for the last several days frank blood in stool, nausea and decreased appetite. CT scan of the abdomen revealed descending colonic obstruction by a 5.3 cm colonic lipomatous mass with resultant intussusception. Patient initially underwent colonoscopy that revealed polypoid lesion at 3–40 similar to lipoma with intussusception that was reduced. Patient subsequently underwent laparoscopic segmental left colectomy for the descending colonic intussusception due to large colonic lipomatous mass. Pathology confirmed the histology of lipoma. Conclusions: Adult bowel intussusception is a rare but challenging condition to diagnose in a timely manner. Preoperative diagnosis is usually missed or delayed because of nonspecific and often subacute symptoms. Lipoma is a rare cause of the intussusception. A high index of suspicion and appropriate investigations (abdominal ultrasound, CT scan and colonoscopy) can result in prompt diagnosis. Lipoma of the large intestine is very rare. Submucosal lipomas are usually asymptomatic but may cause bleeding, obstruction, intussusception, or abdominal pain. Accurate preoperative diagnosis is difficult and lipoma is often mistaken for adenomatous polyp or carcinoma. Treatment usually requires formal resection of the involved bowel segment due to high suspicion for malignancy and subsequent complications due to obstruction. PMID:23869248

  13. Cellular angiofibroma with atypia or sarcomatous transformation: clinicopathologic analysis of 13 cases.

    PubMed

    Chen, Eleanor; Fletcher, Christopher D M

    2010-05-01

    Cellular angiofibroma is a mesenchymal neoplasm that is characterized by a bland spindle cell component, morphologically reminiscent of spindle cell lipoma, and thick-walled vessels. The tumor occurs equally in men and women and usually arises in the inguino-scrotal or vulvovaginal regions. An earlier study of 51 cases from our group showed that the tumor follows a benign course without any tendency for recurrence. In 1 case, an intralesional microscopic nodule of pleomorphic liposarcoma was observed. The biologic significance of atypia or sarcomatous transformation in cellular angiofibroma remains uncertain. In this study, we characterized clinicopathologic features in 13 cases of cellular angiofibroma with morphologic atypia or sarcomatous transformation. Thirteen cases with atypia or sarcomatous transformation among 154 usual cellular angiofibromas identified between 1993 and 2009 were retrieved from consultation files. There were 12 females and 1 male ranging in age from 39 to 71 years (median age, 46 y). Tumor size ranged from 1.2 to 7.5 cm. In 11 cases, the tumors occurred in the vulva. One case each occurred in the paratesticular and hip regions. Most tumors were located in subcutaneous tissue. There were 4 cases of cellular angiofibroma with atypia. Three showed severely atypical cells as scattered foci within the cellular angiofibroma. One case showed a discrete nodule of atypical cells. There were 9 cases of cellular angiofibroma with morphologic features of sarcomatous transformation. In each case, abrupt transition to a discrete sarcomatous component was seen. Of these 9 cases, the sarcomatous component in 2 cases showed features of pleomorphic liposarcoma with multivacuolated lipoblasts readily identified. Three of these 9 cases showed discrete nodule(s) closely resembling atypical lipomatous tumor within usual cellular angiofibroma. In the remaining 4 cases, the sarcomatous component was composed of pleomorphic spindle cells arranged in various patterns. By immunohistochemistry, atypical cells and sarcomatous areas showed either multifocal or more diffuse p16 expression compared with either scattered or negative expression in the conventional cellular angiofibroma. The 3 cases with atypical lipomatous tumor-like areas were negative for MDM-2 and CDK4. Follow-up information was available for 7 patients (range from 2 to 75 mo; median: 14 mo). Six patients did not develop recurrence or metastasis. One patient died of metastatic carcinoma of unknown primary site 27 months after the diagnosis of cellular angiofibroma with sarcomatous transformation. Cellular angiofibroma with atypia or morphologic sarcomatous transformation occurs predominantly in the subcutaneous tissue of the vulva and, as yet, shows no evident tendency to recur based on limited clinical follow-up available for 7 cases. The sarcomatous component can show variable features including atypical lipomatous tumor, pleomorphic liposarcoma, and pleomorphic sarcoma NOS. Overexpression of p16 in the atypical cells and sarcomatous component suggests a possible underlying molecular mechanism. PMID:20305534

  14. Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma.

    PubMed

    Weaver, Joshua; Goldblum, John R; Turner, Sondra; Tubbs, Raymond R; Wang, Wei-Lein; Lazar, Alexander Jf; Rubin, Brian P

    2009-01-01

    Inflammatory liposarcoma is a variant of well-differentiated liposarcoma/atypical lipomatous tumor that consists of a mixture of lymphocytes, histiocytes, scattered atypical stromal cells, mature adipocytes, and rarely lipoblasts. When the inflammatory infiltrate predominates, the morphological features overlap with various fibroinflammatory disorders including sclerosing mesenteritis and retroperitoneal fibrosis, making the diagnosis difficult. Well-differentiated liposarcoma/atypical lipomatous tumor and dedifferentiated liposarcoma have characteristic molecular markers in the form of giant marker and ring chromosomes consisting of amplicons of 12q13-15, which includes MDM2. MDM2 immunohistochemistry (IHC) (Zymed; clone IF2) and dual color fluorescence in situ hybridization utilizing MDM2 (12q15) and chromosome 12 centromeric probes were performed on formalin-fixed and paraffin-embedded specimens from inflammatory well-differentiated liposarcoma (17 cases), sclerosing mesenteritis (14 cases), and idiopathic retroperitoneal fibrosis (10 cases). MDM2 expression as detected by IHC is a very sensitive tool in recognizing inflammatory well-differentiated liposarcoma (17 of 17); however, 21% (3 of 14) and 10% (1 of 10) of sclerosing mesenteritis and retroperitoneal fibrosis, respectively, displayed weak MDM2 immunoexpression. The MDM2 fluorescence in situ hybridization assay was very specific for inflammatory well-differentiated liposarcoma as 15 of 17 (88%) cases showed MDM2 amplification, whereas none of the cases of sclerosing mesenteritis or idiopathic retroperitoneal fibrosis showed amplification. Five cases of retroperitoneal fibrosis were noncontributory secondary to autofluorescence, potentially limiting the usefulness of the assay in certain situations such as inappropriate fixation. Increased MDM2 expression and/or MDM2 amplification can be employed to aid discrimination of inflammatory well-differentiated liposarcoma from fibroinflammatory mimics. MDM2 fluorescence in situ hybridization is a very specific method (100%), but less sensitive (88%), whereas MDM2 expression by IHC is very sensitive (100%), but less specific (83%). Therefore, a positive screen of difficult cases with MDM2 IHC would require confirmation by the fluorescence in situ hybridization. However, lack of MDM2 immunoexpression would rule out the possibility of inflammatory well-differentiated liposarcoma. PMID:18836421

  15. Fluorescence in situ analysis of soft tissue tumor associated genetic alterations in formalin-fixed paraffin-embedded tissue.

    PubMed

    Horn, Heike; Allmanritter, Jan; Doglioni, Claudio; Marx, Alexander; Müller, Justus; Gattenlöhner, Stefan; Staiger, Annette M; Rosenwald, Andreas; Ott, German; Ott, M Michaela

    2014-12-01

    No prospective studies are available to date evaluating the combined analysis of chromosomal alterations via interphase FISH in different soft tissue sarcoma (STS) subtypes. We tested 64 consecutive sarcoma specimens with FISH probes to detect aberrations specific for a given STS subtype. We first determined the translocation frequency in the specific STS subtypes in 48 tumors, with the primary pathological diagnosis as the gold standard. Subsequently, to evaluate sensitivity and specificity, all FISH probes were hybridized to 16 STS of hitherto unknown diagnosis. DDIT3 translocations occurred in 8/10 (80%) of myxoid liposarcomas. FOXO1 translocations were noted in 4/4 (100%) of alveolar but in none of 7 embryonal rhabdomyosarcomas. All 15 (100%) Ewing sarcomas/PNET and 4 clear cell sarcomas (4/4) harbored EWSR1 translocations. SS18 rearrangements were demonstrated in 8/9 (89%) synovial sarcomas. MDM2 amplification was noted in 7/8 (88%) atypical lipomatous tumors/well-differentiated and 3/3 (100%) dedifferentiated liposarcomas, respectively, but not in four pleomorphic liposarcomas. Sensitivities and specificities ranged from 80% to 100% and from 93% to 100%, respectively, with the highest values observed for FOXO1 (100% each). We conclude, therefore, that is possible to accurately predict the STS subtype using a panel of different subtype-specific FISH probes, thereby greatly facilitating the differential diagnosis of these tumors. PMID:25446247

  16. Pancreatic steatosis: Is it related to either obesity or diabetes mellitus?

    PubMed Central

    Pezzilli, Raffaele; Calculli, Lucia

    2014-01-01

    The accumulation of fat in the pancreatic gland has been referred to using various synonyms, such as pancreatic lipomatosis, fatty replacement, fatty infiltration, fatty pancreas, lipomatous pseudohypertrophy, non-alcoholic fatty pancreatic disease and pancreatic steatosis We believe that pancreatic steatosis is the best description of fat accumulation in the pancreatic gland without fat replacement, and this term also describes the possibility that the fat accumulation is a reversible process. A review of the existing literature was carried out, and it was found that there was notable evidence from both the pathological and the imaging point of view that pancreatic steatosis is an increasing problem due to the increasing incidence of obesity. The conclusion was that pancreatic steatosis was easily detectable using modern imaging techniques, such as ultrasonography, endoscopic ultrasonography, computed tomography and magnetic resonance imaging. Pancreatic steatosis was not due to the presence of diabetes mellitus but was highly associated with the metabolic syndrome. The possible presence of steatopancreatitis should be better evaluated, especially regarding the inflammatory cascade, and additional studies are needed which are capable of assessing whether non-alcoholic steatopancreatitis really exists as does non-alcoholic steatohepatitis. Finally, the presence of exocrine pancreatic function should be extensively evaluated in patients with pancreatic steatosis. PMID:25126389

  17. Pancreatic steatosis: Is it related to either obesity or diabetes mellitus?

    PubMed

    Pezzilli, Raffaele; Calculli, Lucia

    2014-08-15

    The accumulation of fat in the pancreatic gland has been referred to using various synonyms, such as pancreatic lipomatosis, fatty replacement, fatty infiltration, fatty pancreas, lipomatous pseudohypertrophy, non-alcoholic fatty pancreatic disease and pancreatic steatosis We believe that pancreatic steatosis is the best description of fat accumulation in the pancreatic gland without fat replacement, and this term also describes the possibility that the fat accumulation is a reversible process. A review of the existing literature was carried out, and it was found that there was notable evidence from both the pathological and the imaging point of view that pancreatic steatosis is an increasing problem due to the increasing incidence of obesity. The conclusion was that pancreatic steatosis was easily detectable using modern imaging techniques, such as ultrasonography, endoscopic ultrasonography, computed tomography and magnetic resonance imaging. Pancreatic steatosis was not due to the presence of diabetes mellitus but was highly associated with the metabolic syndrome. The possible presence of steatopancreatitis should be better evaluated, especially regarding the inflammatory cascade, and additional studies are needed which are capable of assessing whether non-alcoholic steatopancreatitis really exists as does non-alcoholic steatohepatitis. Finally, the presence of exocrine pancreatic function should be extensively evaluated in patients with pancreatic steatosis. PMID:25126389

  18. Resection of soft tissue tumors extending through the obturator ring.

    PubMed

    Starks, Alexandria; Guo, Lifei; Abraham, John A

    2013-09-01

    Pelvic soft tissue sarcomas are rare tumors often presenting larger than other soft tissue sarcomas and can extend into the thigh through various anatomic routes. Surgical resection is the main modality of curative treatment. En bloc tumor excision with wide, negative margins may reduce the risk of local recurrence. Soft tissue sarcomas extending through the obturator foramen create unique challenges to operative management. This case report describes 2 cases of lipomatous lesions that extend through the obturator foramen, presenting as dumbbell-shaped lesions with large intra- and extrapelvis portions. One possible surgical approach performed in both patients is detailed with long-term follow-up. Postoperatively, 1 incidence of infection was reported. Functional outcomes were acceptable, with full restoration of ambulation without assistive devices in both cases and no hernia observed. Oncologic outcomes included locoregional recurrence in 1 patient at 24 months outside the radiation field. The ideal primary treatment for all localized soft tissue sarcomas, including those extending through the obturator foramen, is resection. However, the unique subgroup of obturator ring soft tissue sarcomas has undefined outcomes and complications. The authors' goal was to achieve en bloc resection with wide negative margins while preserving ipsilateral limb function. The surgical approach described in this case study offers a description of feasibility and discussion of theoretical and observed complications. PMID:24025018

  19. Posterior mediastinal ganglioneuroma with peripheral replacement by white and brown adipocytes resulting in diagnostic fallacy from a false-positive 18F-2-fluoro-2-deoxyglucose-positron emission tomography finding: a case report

    PubMed Central

    2014-01-01

    Introduction Ganglioneuroma is a rare tumor in the posterior mediastinum; fat-containing ganglioneuromas are rarely reported. The present case report documents a brown fat-containing, posterior mediastinal ganglioneuroma, which has not been reported previously. Radiological examination, in particular 18F-2-fluoro-2-deoxyglucose-positron emission tomography, suggested that the tumor had low-grade malignant potential. This led to uncertainty at preoperative diagnosis. Case presentation An asymptomatic 66-year-old Japanese woman with no significant past medical history was referred for the evaluation of a posterior mediastinal mass. Although its size had not changed in the past 5 years, a malignant lipomatous tumor could not be excluded due to the presence of intratumoral fat and increased 18F-2-fluoro-2-deoxyglucose uptake observed by positron emission tomography imaging. A computed tomography-guided core-needle biopsy revealed a mixture of mature adipocytes, spindle-shaped cells, and fibrotic stroma. Definite diagnosis was not possible, and surgical resection was performed. Three years after the surgery, she remains disease-free. Conclusions Histological diagnosis of the surgically resected mass confirmed ganglioneuroma with substantial amounts of white and brown adipose tissues in peripheral areas. The existence of both ganglion cells and brown fat tissue intensified the accumulation of 18F-2-fluoro-2-deoxyglucose, resulting in a false-positive result by positron emission tomography. Considering this, ganglioneuroma should not be excluded either clinically or pathologically in fat-containing, posterior mediastinal tumors. PMID:25319096

  20. Fine needle aspiration biopsy diagnosis of dedifferentiated liposarcoma: Cytomorphology and MDM2 amplification by FISH

    PubMed Central

    Al-Maghraby, Hatem Q.; Khalbuss, Walid E.; Rao, Uma N. M.; Cieply, Kathleen; Dacic, Sanja

    2010-01-01

    Lipomatous mesenchymal tumors constitute the most common type of soft tissue tumors. Well-differentiated liposarcoma (WDLS) can undergo dedifferentiation to a nonlipogenic sarcoma of variable histologic grade. In the recent literature, amplification of the murine double minute 2 (MDM2) oncogene, which has a role in cell cycle control, has been successful in distinguishing WDLS from benign lesions. We present a case of dedifferentiated liposarcoma diagnosed by fine-needle aspiration (FNA), using cytomorphology and ancillary studies (immunocytochemistry and fluorescent in-situ hybridization). An 85-year old female presented to our institution with a firm soft tissue mass of the right buttock. The FNA showed atypical spindle cells, osteoclast-like giant cells and extracellular dense matrix material. The cell block showed cellular groups of highly atypical spindle cells with osteoid and adipose tissue. Fluorescence in situ hybridization (FISH) studies performed on the cell block demonstrated amplification of the MDM2 gene. In addition, the findings were morphologically compatible with the previously resected retroperitoneal dedifferentiated liposarcoma with areas of osteosarcoma. This rare case illustrates the usefulness of FNA and ancillary studies in the diagnosis and subclassification of soft tissue tumors. To the best of our knowledge, this is the first report of MDM2 FISH positivity in a liposarcoma diagnosed by FNA. PMID:20436789

  1. Left Supraclavicular Spindle Cell Lipoma

    PubMed Central

    Olaleye, Oladejo; Fu, Bertram; Moorthy, Ram; Lawson, Charles; Black, Myles; Mitchell, David

    2010-01-01

    Background. Spindle cell lipoma (SCL) is a benign lipomatous tumour, typically occurring in the posterior neck, shoulder or upper back of elderly males. They compose of fat, CD34 positive spindle cells, and ropey collagen on a myxoid matrix. This case highlights a rare presentation of SCL and the need for pre-operative diagnosis. Case Report. A 63-year-old gentleman presented with a pre-existing left supraclavicular mass that had recently increased in size. FNA and CT Scans were performed and results discussed in the mutidisciplinary team meeting. Excisional biopsy was recommended. Radiology. CT neck showed a left supraclavicular mass of fatty density with fine internal septations. A low-grade liposarcoma could not be excluded. Histopathology. FNA was indeterminate. Histology of specimen showed bland spindle cells with no evidence of malignancy. Immuno-histochemistry showed SCL with CD34 positivity and negative staining on CDK4 and p16. Management. Excision biopsy of the mass was performed which was technically difficult as the mass invaginated around the brachial plexus. The patient recovered well post-operatively with no neurological deficits. Conclusion. Spindle cell lipoma is a rare benign tumour and a pre-operative diagnosis based on the clinical context, imaging and immuno-histochemistry is crucial to management. PMID:20508829

  2. MRI Features of Spinal Epidural Angiolipomas

    PubMed Central

    Hu, Su; Hu, Xiao-yun; Wang, Xi-ming; Dai, Hui; Fang, Xiang-ming; Cui, Lei

    2013-01-01

    Objective To describe the MRI findings in ten patients of spinal epidural angiolipoma for differentiated diagnosis presurgery. Materials and Methods Ten surgically proved cases of spinal epidural angiolipomas were retrospectively reviewed, and the lesion was classified according to the MR findings. Results Ten tumors were located in the superior (n = 4), middle (n = 2), or inferior (n = 4) thoracic level. The mass, with the spindle shape, was located in the posterior epidural space and extended parallel to the long axis of the spine. All lesions contained a fat and vascular element. The vascular content, correlating with the presence of hypointense regions on T1-weighted imaging (T1WI) and hyperintense signals on T2-weighted imaging, had marked enhancement. However, there were no flow void signs on MR images. All tumors were divided into two types based on the MR features. In type 1 (n = 3), the mass was predominantly composed of lipomatous tissue (> 50%) and contained only a few small angiomatous regions, which had a trabeculated or mottled appear. In type 2 (n = 7), the mass, however, was predominantly composed of vascular components (> 50%), which presented as large foci in the center of the mass. Conclusion Most spinal epidural angiolipomas exhibit hyperintensity on T1WI while the hypointense region on the noncontrast T1WI indicates to be vascular, which manifests an obvious enhancement with gadolinium administration. PMID:24043978

  3. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

    PubMed

    Guo, Juan; Zhou, Shengmei; Rao, Nagesh P; Pez, Gholam H

    2010-10-01

    Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later. PMID:20505510

  4. The right atrium: gateway to the heart--anatomic and pathologic imaging findings.

    PubMed

    Malik, Sachin B; Kwan, Damon; Shah, Amar B; Hsu, Joe Y

    2015-01-01

    Knowledge of right atrial anatomic and pathologic imaging findings and associated clinical symptoms is important to avoid false-positive diagnoses and missed findings. Complete evaluation of the heart often requires a multimodality approach that includes radiography, echocardiography, computed tomography (CT), magnetic resonance (MR) imaging, and invasive angiography. In general, CT provides the highest spatial resolution of these modalities at the cost of radiation exposure to the patient. Echocardiography and MR imaging offer complementary and detailed information for functional evaluation without added radiation exposure. The advantages and disadvantages of each modality for the evaluation of right atrial anatomic structure, size, and pathologic findings are discussed. Cardiac MR imaging is the reference standard for evaluation of right atrial size and volume but often is too time consuming and resource intensive to perform in routine clinical practice. Therefore, established reference ranges for two-dimensional transthoracic echocardiography are often used. Right atrial pathologic findings can be broadly categorized into (a) congenital anomalies (cor triatriatum dexter, Ebstein anomaly, and aneurysm), (b) disorders of volume (tricuspid regurgitation, pathologic mimics such as a pseudoaneurysm, and atrial septal defect), (c) disorders of pressure (tricuspid stenosis, restrictive cardiomyopathy, and constrictive pericarditis), and (d) masses (pseudomasses, thrombus, lipomatous hypertrophy of the interatrial septum, lipoma, myxoma, sarcoma, and metastatic disease). Familiarity with each pathologic entity and its treatment options is essential to ensure that appropriate imaging modalities are selected. Online supplemental material is available for this article. PMID:25590385

  5. DDIT3 Expression in Liposarcoma Development

    PubMed Central

    Engström, Katarina

    2014-01-01

    Liposarcomas are mesenchymal tumors containing variable numbers of lipoblasts or adipocytes. The most common entities, well differentiated/dedifferentiated liposarcoma (WDLS/DDLS) and myxoid/round cell liposarcoma (MLS/RCLS), are both characterized by genetic rearrangements that affect the expression of the transcription factor DDIT3. DDIT3 induces liposarcoma morphology when ectopically expressed in a human fibrosarcoma. The role of DDIT3 in lipomatous tumors is, however, unclear. We have analyzed the expression of DDIT3 in 37 cases of liposarcoma (WDLS/DDLS n = 10, MLS/RCLS n = 16, and pleomorphic liposarcomas (PLS) n = 11) and 11 cases of common benign lipomas. Major cell subpopulations of WDLS/DDLS and MLS/RCLS tumors were found to express DDIT3 or the derived fusion protein, whereas PLS cases showed only a few positive cells. The lipomas contained large subpopulations expressing DDIT3. No correlation between numbers of DDIT3 expressing cells and numbers of lipoblasts/adipocytes was found. In vitro adipogenic treatment of two DDIT3 expressing cell lines induced lipid accumulation in small subpopulations only. Our results suggest a dual, promoting and limiting, role for DDIT3 in the formation of lipoblasts and liposarcoma morphology. PMID:24790523

  6. The tip of the iceberg: a giant pelvic atypical lipoma presenting as a sciatic hernia

    PubMed Central

    Skipworth, Richard JE; Smith, Graeme HM; Stewart, Ken J; Anderson, David N

    2006-01-01

    Background This case report highlights two unusual surgical phenomena: lipoma-like well-differentiated liposarcomas and sciatic hernias. It illustrates the need to be aware that hernias may not always simply contain intra-abdominal viscera. Case presentation A 36 year old woman presented with an expanding, yet reducible, right gluteal mass, indicative of a sciatic hernia. However, magnetic resonance imaging demonstrated a large intra- and extra-pelvic fatty mass traversing the greater sciatic foramen. The tumour was surgically removed through an abdomino-perineal approach. Subsequent pathological examination revealed an atypical lipomatous tumour (synonym: lipoma-like well-differentiated liposarcoma). The patient remains free from recurrence two years following her surgery. Conclusion The presence of a gluteal mass should always suggest the possibility of a sciatic hernia. However, in this case, the hernia consisted of an atypical lipoma spanning the greater sciatic foramen. Although lipoma-like well-differentiated liposarcomas have only a low potential for recurrence, the variable nature of fatty tumours demands that patients require regular clinical and radiological review. PMID:16790047

  7. [The new WHO classification of tumors of the nervous system 2000. Pathology and genetics].

    PubMed

    Radner, H; Blümcke, I; Reifenberger, G; Wiestler, O D

    2002-07-01

    New developments in neuro-oncology have prompted an update of the World Health Organization (WHO) classification of tumors of the nervous system. Major changes include the addition of new entities and the refinement of criteria for the diagnosis and grading of various neoplasms, in particular the meningiomas. As novel clinico-pathological entities, the chordoid glioma of the third ventricle, the atypical teratoid/rhabdoid tumor (AT/RT), the solitary fibrous tumor, and the perineurioma have been listed. The former lipomatous medulloblastoma of the cerebellum, previously incorporated in the family of embryonal tumors, is now classified as cerebellar liponeurocytoma. The term mixed pineocytoma/pineoblastoma has been replaced by pineal parenchymal tumor of intermediate differentiation. Furthermore, the large cell medulloblastoma and the tanycytic ependymoma were established as novel tumor variants. A separate chapter on the peripheral neuroblastic tumors has now been included in the classification. Substantial revisions were introduced in the meningioma chapter. For both atypical meningioma WHO grade II and anaplastic meningioma WHO grade III, histopathological criteria are now precisely defined. An important new addition to the WHO 2000 classification of nervous system tumors is the inclusion of molecular pathology findings. With this combination of pathology and genetics it has set the stage for a new format of the WHO tumor classification series. PMID:12185780

  8. Spinal extradural angiolipoma: report of two cases and review of the literature

    PubMed Central

    García-Allut, Alfredo

    2009-01-01

    Spinal angiolipomas are benign uncommon neoplasm composed of mature lipocytes admixed with abnormal blood vessels. They account for only 0.04–1.2% of all spinal tumors. We report two cases of lumbar extradural angiolipoma and review previously reported cases. We found 118 cases of spinal epidural angiolipoma (70 females and 48 males; age range 1.5–85 years, mean 44.03) spanning from 1890 to 2006. Prior to diagnosis 40.6% of the patients had weakness of the lower limbs. The interval between the initial symptoms and tumor diagnosis ranged from 1 day to 17 years (mean 20.2 months). Except for four cases diagnosed at autopsy, 109 patients underwent surgery and gross-total resection was performed in 79 cases (72.4%). Spinal angiolipomas are tumors containing angiomatous and lipomatous tissue, predominantly located in the mid-thoracic region. All angiolipomas show iso- or hyperintensity on T1-weighted images and hyperintensity on T2-weighted images and most lesions enhance with gadolinium administration. The treatment for spinal extradural angiolipomas is total surgical resection and no adjuvant therapy should be administered. PMID:19127373

  9. Soft Tissue Tumours of the Retroperitoneum

    PubMed Central

    Van Roggen, J. Frans Graadt

    2000-01-01

    Purpose. This review summarizes the more prevalent soft tissue tumours arising in the retroperitoneum and highlights some recent fundamental and diagnostic developments relevant to mesenchymal tumours. Discussion. The retroperitoneum is an underestimated site for benign and malignant neoplastic disease, and represents the second most common site of origin of primary malignant soft tissue tumours (sarcomas) after the deep tissues of the lower extremity. In contrast to the predominance of benign soft tissue lesions over malignant sarcomas elsewhere, retroperitoneal mesenchymal lesions are far more likely to be malignant. The differential diagnosis is primarily with the more common lymphoproliferative and parenchymatous epithelial lesions arising in this area, and with metastatic disease from known or unknown primary sites elsewhere.The most prevalent mesenchymal tumours at this site are of a lipomatous, myogenic or neural nature.Their generally late clinical presentation and poorly accessible location provides numerous clinical challenges; optimal radiological imaging and a properly performed biopsy are essential cogs in the management route. Histopathological diagnosis may be complicated, but has been aided by developments in the fields of immunohistochemistry and tumour (cyto)genetics. Despite significant advances in oncological management protocols, the prognosis remains generally less favourable than for similar tumours at more accessible sites. PMID:18521430

  10. [Hypoechogenic aspects of the ventral embryonic cephalic pancreas: a large prospective clinical study].

    PubMed

    Coulier, B

    1996-06-01

    Global echogenicity of the normal pancreas increases essentially with lipomatous involution which is common with aging. However, the two embryologic different parts of the cephalic pancreas may present a different echogenicity: the "embryologic ventral" pancreas-posterior cephalic pancreas + uncinate process-is then hypoechoic because of less fatty involution. It is important to distinguish such normal variant from a pathological process. The clinical prevalence and morphologic characteristics of this normal variant have been prospectively studied in a large series of 446 patients including 275 females and 171 males. The study confirms the proportional increase of pancreatic echogenicity with age. The variant has a sharp female predominance: 27.5% of males vs 43.3% females, corresponding to a sex ratio of 1:1.52. Hypoechoic ventral embryologic cephalic pancreas is never encountered before the age of 25 and is most frequent in middle aged females with moderately echoic pancreas. There is not any difference in global cephalic pancreatic thickness between our positive group and a negative group of reference of 66 patients. These observations rather plead a genetic uneven fatty involution in positive cases instead of ventral pancreas atrophy in negative cases. The morphological and biometric characteristics of the hypoechoic embryologic ventral pancreas are rather stable. These are relatively independent from age, sex, and global pancreatic echogenicity. PMID:8765075

  11. [Intraductal ultrasound of the pancreas--a procedure with diagnostic prospects? Initial preclinical experiences].

    PubMed

    Menzel, J; Foerster, E C; Kerber, S; Keller, R; Domschke, W

    1993-12-01

    For the first time, human pancreas specimens (18 autopsy specimens, three resection specimens) were examined with high-resolution, flexible ultrasound catheters (20 MHz; 3.5 F, 4.8 F, 5.0 F and 6.0 F external diameters; mechanical and electronic systems). The ultrasound catheter was easily inserted into the pancreatic duct in all specimens. The sonographic tissue texture was correlated with its histological picture at defined positions. A high resolution was thus achieved in the sonomorphological differentiation of blood vessels, duct system elements, fibrotic tissue, fatty tissue and pancreas tissue with varying lipomatous composition. In a radius of an average of 5.5 mm, structures 0.1 mm large were recognised. Contrast media studies of the ductal system were carried out before and after ultrasound examination. No evidence of trauma due to catheterisation was found either with these studies or by histology. In one case, a 15 mm serous microcystic adenoma of the pancreatic head was found at intraductal examination of the resection specimen. Clinical examinations within the framework of endoscopic retrograde pancreaticography must clarify if the intraductal ultrasonography technique presented here can truly enlarge the diagnostic, repertoire used in the search of tiny focal pancreatic lesions. PMID:8128207

  12. Blood flow in the atrophied pancreas.

    PubMed

    Weaver, F C

    1990-01-01

    Pancreatic and islet blood flow was measured by quantitative determination of microspheres within atrophied pancreases of rats rendered dietarily copper-deficient. The deficient state effects selective acinar necrosis and its replacement with non-inflammatory lipomatosis. While blood flow to lipomatous glands remained intact, it was significantly less (0.41 +/- 0.02 ml/min X g pancreas) compared to normal control glands (0.50 +/- 0.01 ml/min X g pancreas). Blood flow to islets, which remain unaffected by selective acinar atrophy of the copper-deficient state, was not significantly altered (50.8 +/- 2.13 microliters/min X g pancreas) compared to controls (55.7 +/- 2.66 microliters/min X g pancreas). The percent of islet blood flow (IBF) within atrophied glands (12.2 +/- 0.31) was found to be significantly greater than control glands (10.7 +/- 0.21). It is suggested that the maintenance of IBF and IBF percentage in the atrophic pancreas may reflect the parallel arrangement of islet angioarchitecture which based on morphological data is not appreciably altered with attenuation of acinar plexuses. Microspheres, although not quantitated, were also found distributed within the adventitia of ducts as well as in vessels supplying intrapancreatic nerves and ganglia which remain intact in copper deficiency-induced acinar atrophy. PMID:2188848

  13. [Pancreaticomediastinal fistula as a rare cause of recurrent pleural effusions].

    PubMed

    Sandmann, M; Fähndrich, M; Heike, M

    2007-10-01

    A 45-year-old male with chronic alcoholism was transferred to our hospital with progressive dyspnoea, bilateral pleural effusions and ascites. Serum concentrations of lipase and CRP were increased. Pleural effusions recurred despite repeated thoracentesis, antibiotic therapy and total parenteral nutrition. A CT scan of chest and abdomen showed a lipomatous pancreas without signs of necrosis. Endosonography (EUS) demonstrated as signs of chronic pancreatitis an increased lobularity of the parenchyma without calcifications and two small cystic lesions in the pancreatic head. The pleural fluid lipase levels were markedly increased compared to a low lipase concentration in ascites. As an explanation for the pleural effusions ERCP showed a retroperitoneal fistula in the head/body region into the mediastinum and a stenosis of the pancreatic duct in the pancreatic head. For the ascites an inflammatory pathogenesis due to the exacerbation of chronic pancreatitis had to be assumed because the analysis showed an exudate with leukocytosis. After papillotomy via the pancreatic duct a 10 French wide 9 cm long stent was inserted into the pancreatic duct bridging the pancreatic fistula. Within 10 days pleural effusions had completely receded. After three weeks pancreaticography showed a total regression of the fistula and the stent was exchanged. After 6 weeks the pancreatic stent was removed with a lasting remission of the pleural effusions. PMID:17924302

  14. Angioarchitecture of the atrophic pancreas.

    PubMed

    Weaver, C

    The pancreas has a complex vasculature which comprises both exocrine and endocrine structures. Copper deficiency induces highly selective acinar cell degeneration and progressive noninflammatory lipomatosis in pancreas while Langerhans islets, ducts, and nerves remain unaffected. Pancreatic vasculature was examined in rats that had dietary copper deficiency to characterize changes in the angioarchitecture of the gland. This model was used to assess the degree to which the vasculature of non-acinar components of the gland are potentially altered under conditions of exocrine atrophy. Ultrastructure of pancreas was examined by histology, enzyme histochemistry and immunohistochemistry, corrosion casting and scanning electron microscopy, in situ vascular staining, microsphere injection, biochemical analysis, and morphometry in copper-deficient rats. Results show that no acute angiopathic changes indicative of vascular disorganization accompany atrophy. Only a reduction in the complexity of the capillary beds, which normally vascularize the dense acinar parenchyma, was found. Microsphere quantitation also showed that blood flow to the lipomatous gland remains intact. Furthermore, analysis of the angioarchitecture of the atrophied pancreas supports a largely autonomous blood supply to islets and ducts. These observations support the hypothesis that while the vasculature of the atrophied gland is modified in vascular regions severely targeted by acinar necrosis, the overall structural features of the angioarchitecture are preserved. The atrophied gland thus provides an experimental model to study the vascular routes supplying islet and ductal blood flow within the complex pancreatic circulation. PMID:9220429

  15. Preduodenal portal vein in adult with polysplenia syndrome revisited with a case report.

    PubMed

    G A, Latha; Kagali, Nagaraj A; M, Shridhar; Prasad, B S Satish

    2014-04-01

    Preduodenal portal vein (PDPV) is a rare developmental anomaly. In infants, this is often associated with duodenal obstruction or biliary atresia. It is generally asymptomatic in adults (Ooshima et al., Hepato-Biliary-Pancreat Surg 5(4):455-458, 1998). Here, we report a singular case of adult PDPV that was discovered accidentally during emergency laparotomy for peritonitis due to gastric perforation in a 38-year-old lady. A plethora of congenital anomalies was uncovered, which is consistent with the reported cases of classical polysplenia syndrome, viz., multiple spleens of equal volume, visceral heterotaxia, right (Rt.)-sided stomach, a left (Lt.)-sided or a large midline liver, malrotation of intestine, a short pancreas, PDPV and IVC abnormalities (Gayer et al., Abdom Imaging 24:178-184, 1999). In addition, abnormalities like anamolous origin of Lt. gastric and splenic arteries from the abdominal aorta with absent celiac trunk, hepatic artery arising from the superior mesenteric artery, hepatic veins draining directly to Rt. atrium, etc. along with hypertrophic and lipomatous interatrial septum have also been detected during further investigations. As of now, we have come across 29 cases of adult PDPV reported in world literature and we are reporting our unique case with a review of literature on anomalies of visceral organs associated with PDPV. PMID:24891779

  16. Intra-articular synovial lipoma of the knee joint (located in the lateral recess): a case report and review of the literature.

    PubMed

    Hirano, Kenichi; Deguchi, Masao; Kanamono, Toshihisa

    2007-01-01

    This report describes a rare intra-articular synovial lipoma of the knee joint which developed in a 66-year-old female. The patient suffered from sudden knee pain and a catching or slight interruption of normal motion of the left knee and then noticed the tumor. The tumor was located in the lateral recess of the knee joint and showed a signal intensity similar to subcutaneous fat on T1 and T2()-weighted magnetic resonance images. The arthroscopy revealed a smooth, globular, yellowish, encapsulated tumor extending into the lateral recess from the surface of the lateral condyle and the tumor was totally excised under arthroscopic guidance. Histologic examination of the specimen revealed a tumor composed of mature adipose cells covered by a thin fibrous layer of varying thickness and normal synovial lining cells. The diagnosis was intra-articular synovial lipoma. Intra-articular synovial lipomas should be distinguished from other similar lipomatoid conditions such as Hoffa disease and villous lipomatous proliferation of the synovial membrane (lipoma arborescens). Intra-articular synovial lipoma should be considered in the differential diagnoses when examining a patient with sudden knee pain, and a catching or locking knee. PMID:17127064

  17. An overview on development and application of an experimental platform for quantitative cardiac imaging research in rabbit models of myocardial infarction

    PubMed Central

    Feng, Yuanbo; Bogaert, Jan; Oyen, Raymond

    2014-01-01

    To exploit the advantages of using rabbits for cardiac imaging research and to tackle the technical obstacles, efforts have been made under the framework of a doctoral research program. In this overview article, by cross-referencing the current literature, we summarize how we have developed a preclinical cardiac research platform based on modified models of reperfused myocardial infarction (MI) in rabbits; how the in vivo manifestations of cardiac imaging could be closely matched with those ex vivo macro- and microscopic findings; how these imaging outcomes could be quantitatively analyzed, validated and demonstrated; and how we could apply this cardiac imaging platform to provide possible solutions to certain lingering diagnostic and therapeutic problems in experimental cardiology. In particular, tissue components in acute cardiac ischemia have been stratified and characterized, post-infarct lipomatous metaplasia (LM) as a common but hardly illuminated clinical pathology has been identified in rabbit models, and a necrosis avid tracer as well as an anti-ischemic drug have been successfully assessed for their potential utilities in clinical cardiology. These outcomes may interest the researchers in the related fields and help strengthen translational research in cardiovascular diseases. PMID:25392822

  18. Immunohistochemical, cytogenetic, and molecular cytogenetic characterization of both components of a dedifferentiated liposarcoma: implications for histogenesis.

    PubMed

    Nishio, Jun; Iwasaki, Hiroshi; Nabeshima, Kazuki; Naito, Masatoshi

    2015-01-01

    Dedifferentiated liposarcoma (DDLS) is a malignant adipocytic tumor showing transition from an atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDLS) to a non-lipogenic sarcoma of variable histological grades. We present the immunohistochemical, cytogenetic, and molecular cytogenetic findings of DDLS arising in the right chest wall of a 76-year-old man. Magnetic resonance imaging exhibited a large mass composed of two components with heterogeneous signal intensities, suggesting the coexistence of a fatty area and another soft tissue component. The grossly heterogeneous mass was histologically composed of an ALT/WDLS component transitioning abruptly into a dedifferentiated component. Immunohistochemistry was positive for murine double-minute 2 (MDM2), cyclin-dependent kinase 4 (CDK4), and p16 in both components, although a more strong and diffuse staining was found in the dedifferentiated area. The MIB-1 labeling index was extremely higher in the dedifferentiated area compared to the ALT/WDLS area. Cytogenetic analysis of the ALT/WDLS component revealed the following karyotype: 46,X,-Y,+r. Notably, cytogenetic analysis of the dedifferentiated component revealed a similar but more complex karyotype. Spectral karyotyping demonstrated that the ring chromosome was entirely composed of material from chromosome 12. Interphase fluorescence in situ hybridization analysis revealed amplification of MDM2 and CDK4 in both components. These findings suggest that multiple abnormal clones derived from a single precursor cell would be present in DDLS, with one or more containing supernumerary rings or giant marker chromosomes. PMID:25550570

  19. Fine-Needle Aspiration Cytology of Soft Tissue Sarcoma: Benefits and Limitations

    PubMed Central

    1998-01-01

    Purpose. Examine the benefits and limitations of fine-needle aspiration cytology (FNA) used as the definitive diagnostic method before treatment. Method. Review of the 25 year experience at a multidisciplinary musculo-skeletal centre where FNA is the primary diagnostic approach to soft tissue sarcoma in the extremities and trunk wall and the experience of various experts in the field. Results. FNA has several benefits compared with coarse needle or open surgical biopsy. The most important are rapid preliminary diagnosis, no need for hospitalization and anaesthesia, negligible complications and fear for tumour cell spread. With the collected experience gained during the years a reliable diagnosis of sarcoma is the rule in general and specific-type diagnoses are possible in many histotypes, especially when the cytologic examination is supplemented with ancillary diagnostics. The most important limitations are inability to hit small deep-seated sarcoma and some diagnostic pitfalls such as the correct diagnosis of spindle cell neoplasms, variants of benign lipomatous tumours and ‘new soft tissue tumour entities’. Discussion. Optimal use of FNA calls for certain requirements such as centralization, experience in soft tissue tumour cytology–histopathology, the FNA technique and close co-operation between the orthopaedic surgeon and cytopathologist. PMID:18521248

  20. Clinical delineation and natural history of the PIK3CA-related overgrowth spectrum.

    PubMed

    Keppler-Noreuil, Kim M; Sapp, Julie C; Lindhurst, Marjorie J; Parker, Victoria E R; Blumhorst, Cathy; Darling, Thomas; Tosi, Laura L; Huson, Susan M; Whitehouse, Richard W; Jakkula, Eveliina; Grant, Ian; Balasubramanian, Meena; Chandler, Kate E; Fraser, Jamie L; Gucev, Zoran; Crow, Yanick J; Brennan, Leslie Manace; Clark, Robin; Sellars, Elizabeth A; Pena, Loren D M; Krishnamurty, Vidya; Shuen, Andrew; Braverman, Nancy; Cunningham, Michael L; Sutton, V Reid; Tasic, Velibor; Graham, John M; Geer, Joseph; Henderson, Alex; Semple, Robert K; Biesecker, Leslie G

    2014-07-01

    Somatic mutations in the phosphatidylinositol/AKT/mTOR pathway cause segmental overgrowth disorders. Diagnostic descriptors associated with PIK3CA mutations include fibroadipose overgrowth (FAO), Hemihyperplasia multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, Scoliosis/skeletal and spinal (CLOVES) syndrome, macrodactyly, and the megalencephaly syndrome, Megalencephaly-Capillary malformation (MCAP) syndrome. We set out to refine the understanding of the clinical spectrum and natural history of these phenotypes, and now describe 35 patients with segmental overgrowth and somatic PIK3CA mutations. The phenotypic data show that these previously described disease entities have considerable overlap, and represent a spectrum. While this spectrum overlaps with Proteus syndrome (sporadic, mosaic, and progressive) it can be distinguished by the absence of cerebriform connective tissue nevi and a distinct natural history. Vascular malformations were found in 15/35 (43%) and epidermal nevi in 4/35 (11%) patients, lower than in Proteus syndrome. Unlike Proteus syndrome, 31/35 (89%) patients with PIK3CA mutations had congenital overgrowth, and in 35/35 patients this was asymmetric and disproportionate. Overgrowth was mild with little postnatal progression in most, while in others it was severe and progressive requiring multiple surgeries. Novel findings include: adipose dysregulation present in all patients, unilateral overgrowth that is predominantly left-sided, overgrowth that affects the lower extremities more than the upper extremities and progresses in a distal to proximal pattern, and in the most severely affected patients is associated with marked paucity of adipose tissue in unaffected areas. While the current data are consistent with some genotype-phenotype correlation, this cannot yet be confirmed. PMID:24782230

  1. The histopathology of PRSS1 hereditary pancreatitis.

    PubMed

    Singhi, Aatur D; Pai, Reetesh K; Kant, Jeffrey A; Bartholow, Tanner L; Zeh, Herbert J; Lee, Kenneth K; Wijkstrom, Martin; Yadav, Dhiraj; Bottino, Rita; Brand, Randall E; Chennat, Jennifer S; Lowe, Mark E; Papachristou, Georgios I; Slivka, Adam; Whitcomb, David C; Humar, Abhinav

    2014-03-01

    Hereditary pancreatitis is an autosomal dominant disorder with 80% penetrance and variable expressivity. The vast majority of cases have been linked to mutations within the cationic trypsinogen gene, also referred to as serine protease 1 (PRSS1). Other than inheritance, PRSS1 pancreatitis has been considered clinically and pathologically indistinguishable from other etiologies of chronic pancreatitis. However, to date, the histologic findings of PRSS1 pancreatitis have not been well described. We, therefore, collected pancreatic specimens from 10 PRSS1 patients of various ages and examined their clinicopathologic features. Patients at the time of resection ranged in age from 9 to 66 years (median, 29 y), with a slight female predominance (60%). All patients reported a history of intermittent abdominal pain, with an age of onset ranging from infancy to 21 years of age. Examination of the gross and microscopic findings suggested a sequential pattern of changes with increasing patient age. In pediatric patients (n=4), although in most cases the pancreas was grossly normal, there was microscopic variation in lobular size and shape. Although the central portions of the pancreas displayed parenchymal loss accompanied by loose perilobular and interlobular fibrosis, the periphery was remarkable for replacement by mature adipose tissue. These changes were more developed in younger adults (n=2), in whom fatty replacement seemed to extend from the periphery to the central portions of the pancreas. With older patients (n=4), the pancreas showed marked atrophy and extensive replacement by mature adipose tissue with scattered islets of Langerhans and rare acinar epithelium concentrated near the main pancreatic duct. In summary, PRSS1 hereditary pancreatitis is characterized by progressive lipomatous atrophy of the pancreas. PMID:24525505

  2. PIK3CA-related overgrowth spectrum (PROS): diagnostic and testing eligibility criteria, differential diagnosis, and evaluation.

    PubMed

    Keppler-Noreuil, Kim M; Rios, Jonathan J; Parker, Victoria E R; Semple, Robert K; Lindhurst, Marjorie J; Sapp, Julie C; Alomari, Ahmad; Ezaki, Marybeth; Dobyns, William; Biesecker, Leslie G

    2015-02-01

    Somatic activating mutations in the phosphatidylinositol-3-kinase/AKT/mTOR pathway underlie heterogeneous segmental overgrowth phenotypes. Because of the extreme differences among patients, we sought to characterize the phenotypic spectrum associated with different genotypes and mutation burdens, including a better understanding of associated complications and natural history. Historically, the clinical diagnoses in patients with PIK3CA activating mutations have included Fibroadipose hyperplasia or Overgrowth (FAO), Hemihyperplasia Multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal (CLOVES) syndrome, macrodactyly, Fibroadipose Infiltrating Lipomatosis, and the related megalencephaly syndromes, Megalencephaly-Capillary Malformation (MCAP or M-CM) and Dysplastic Megalencephaly (DMEG). A workshop was convened at the National Institutes of Health (NIH) to discuss and develop a consensus document regarding diagnosis and treatment of patients with PIK3CA-associated somatic overgrowth disorders. Participants in the workshop included a group of researchers from several institutions who have been studying these disorders and have published their findings, as well as representatives from patient-advocacy and support groups. The umbrella term of "PIK3CA-Related Overgrowth Spectrum (PROS)" was agreed upon to encompass both the known and emerging clinical entities associated with somatic PIK3CA mutations including, macrodactyly, FAO, HHML, CLOVES, and related megalencephaly conditions. Key clinical diagnostic features and criteria for testing were proposed, and testing approaches summarized. Preliminary recommendations for a uniform approach to assessment of overgrowth and molecular diagnostic testing were determined. Future areas to address include the surgical management of overgrowth tissue and vascular anomalies, the optimal approach to thrombosis risk, and the testing of potential pharmacologic therapies. PMID:25557259

  3. The Histopathology of PRSS1 Hereditary Pancreatitis

    PubMed Central

    Singhi, Aatur D.; Pai, Reetesh K.; Kant, Jeffrey A.; Bartholow, Tanner L.; Zeh, Herbert J.; Lee, Kenneth K.; Wijkstrom, Martin; Yadav, Dhiraj; Bottino, Rita; Brand, Randall E.; Chennat, Jennifer S.; Lowe, Mark E.; Papachristou, Georgios I.; Slivka, Adam; Whitcomb, David C.; Humar, Abhinav

    2015-01-01

    Hereditary pancreatitis is an autosomal dominant disorder with 80% penetrance and variable expressivity. The vast majority of cases have been linked to mutations within the cationic trypsinogen gene, also referred to as serine protease 1 (PRSS1). Other than inheritance, PRSS1 pancreatitis has been considered clinically and pathologically indistinguishable from other etiologies of chronic pancreatitis. However, to date, the histologic findings of PRSS1 pancreatitis have not been well described. We, therefore, collected pancreatic specimens from 10 PRSS1 patients of various ages and examined their clinicopathologic features. Patients at the time of resection ranged in age from 9 to 66 years (median, 29 y), with a slight female predominance (60%). All patients reported a history of intermittent abdominal pain, with an age of onset ranging from infancy to 21 years of age. Examination of the gross and microscopic findings suggested a sequential pattern of changes with increasing patient age. In pediatric patients (n=4), although in most cases the pancreas was grossly normal, there was microscopic variation in lobular size and shape. Although the central portions of the pancreas displayed parenchymal loss accompanied by loose perilobular and interlobular fibrosis, the periphery was remarkable for replacement by mature adipose tissue. These changes were more developed in younger adults (n=2), in whom fatty replacement seemed to extend from the periphery to the central portions of the pancreas. With older patients (n=4), the pancreas showed marked atrophy and extensive replacement by mature adipose tissue with scattered islets of Langerhans and rare acinar epithelium concentrated near the main pancreatic duct. In summary, PRSS1 hereditary pancreatitis is characterized by progressive lipomatous atrophy of the pancreas. PMID:24525505

  4. Clinical Delineation and Natural History of the PIK3CA-Related Overgrowth Spectrum**

    PubMed Central

    Keppler-Noreuil, Kim M; Sapp, Julie C; Lindhurst, Marjorie J; Parker, Victoria ER; Blumhorst, Cathy; Darling, Thomas; Tosi, Laura L; Huson, Susan M; Whitehouse, Richard W; Jakkula, Eveliina; Grant, Ian; Balasubramanian, Meena; Chandler, Kate E; Fraser, Jamie L; Gucev, Zoran; Crow, Yanick J; Brennan, Leslie Manace; Clark, Robin; Sellars, Elizabeth A; Pena, Loren DM; Krishnamurty, Vidya; Shuen, Andrew; Braverman, Nancy; Cunningham, Michael L; Sutton, V Reid; Tasic, Velibor; Graham, John M; Geer, Joseph; Henderson, Alex; Semple, Robert K; Biesecker, Leslie G

    2014-01-01

    Somatic mutations in the phosphatidylinositol/AKT/mTOR pathway cause segmental overgrowth disorders. Diagnostic descriptors associated with PIK3CA mutations include fibroadipose overgrowth (FAO), Hemihyperplasia multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, Scoliosis/skeletal and spinal (CLOVES) syndrome, macrodactyly, and the megalencephaly syndrome, Megalencephaly-Capillary malformation (MCAP) syndrome. We set out to refine the understanding of the clinical spectrum and natural history of these phenotypes, and now describe 35 patients with segmental overgrowth and somatic PIK3CA mutations. The phenotypic data show that these previously described disease entities have considerable overlap, and represent a spectrum. While this spectrum overlaps with Proteus syndrome (sporadic, mosaic, and progressive) it can be distinguished by the absence of cerebriform connective tissue nevi and a distinct natural history. Vascular malformations were found in 15/35 (43%) and epidermal nevi in 4/35 (11%) patients, lower than in Proteus syndrome. Unlike Proteus syndrome, 31/35 (89%) patients with PIK3CA mutations had congenital overgrowth, and in 35/35 patients this was asymmetric and disproportionate. Overgrowth was mild with little postnatal progression in most, while in others it was severe and progressive requiring multiple surgeries. Novel findings include: adipose dysregulation present in all patients, unilateral overgrowth that is predominantly left-sided, overgrowth that affects the lower extremities more than the upper extremities and progresses in a distal to proximal pattern, and in the most severely affected patients is associated with marked paucity of adipose tissue in unaffected areas. While the current data are consistent with some genotype–phenotype correlation, this cannot yet be confirmed. © The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc. PMID:24782230

  5. Three dosimetry models of lipoma arborescens treated by {sup 90}Y synovectomy

    SciTech Connect

    O’Doherty, Jim, E-mail: jim.odoherty@kcl.ac.uk [Department of Medical Physics-Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX, United Kingdom and Division of Imaging Sciences, PET Imaging Centre at St. Thomas’ Hospital, King's College London, London SE1 7EH (United Kingdom)] [Department of Medical Physics-Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX, United Kingdom and Division of Imaging Sciences, PET Imaging Centre at St. Thomas’ Hospital, King's College London, London SE1 7EH (United Kingdom); Clauss, Ralf [Department of Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom)] [Department of Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom); Scuffham, James [Department of Medical Physics-Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom)] [Department of Medical Physics-Nuclear Medicine, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom); Khan, Aman [Department of Rheumatology, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom)] [Department of Rheumatology, Royal Surrey County Hospital, Guildford GU2 7XX (United Kingdom); Petitguillaume, Alice; Desbrée, Aurélie [Service de Dosimétrie Interne, Institut de Radioprotection et de Sûreté Nucléaire, 92260 Fontenay-aux-Roses (France)] [Service de Dosimétrie Interne, Institut de Radioprotection et de Sûreté Nucléaire, 92260 Fontenay-aux-Roses (France)

    2014-05-15

    Purpose: Lipoma arborescens (LA) is a benign intra-articular lipomatous proliferation of the synovial membrane. This extremely rare condition has previously been treated by intra-articular{sup 90}Y radiosynoviorthesis but dosimetry literature on this form of radionuclide therapy is nonexistent. The authors detail methodology for successful treatment of LA and provide for the first time estimates of radiation dosimetry. The authors also analyze the biodistribution of the radiopharmaceutical over the course of the patient's treatment through sequential imaging. Methods: A patient with bilateral LA underwent intracavity injection of{sup 90}Y citrate colloid to the right and left knee joint spaces (181 and 198 MBq, respectively). SPECT/CT datasets were acquired over 9 days to quantify the biodistribution and kinetics of the radiopharmaceutical. Radiation dosimetry was performed using the MIRD schema (through OLINDA software), a custom voxel-based method, and a direct Monte Carlo calculation (OEDIPE). Results: Follow-up MRI showed marked reduction in LA size in both knees. Mean absorbed doses to the LA were 21.2 ± 0.8 and 42.9 ± 2.3 Gy using OLINDA, 8.1 ± 0.3 and 16.7 ± 0.5 Gy using voxel based methodology, and 8.2 ± 0.3 and 15.7 ± 0.5 Gy for OEDIPE in the right and left LA, respectively. Distribution of the radiopharmaceutical within the joint space alters over the imaging period, with less than 1% of the remaining activity having moved posteriorly in the knee cavity. No uptake was detected outside of the joint space after assessment with whole-body scintigraphy. Conclusions: An activity of approximately 185 MBq successfully relieved clinical symptoms of LA. There was good correlation between direct Monte Carlo and voxel based techniques, but OLINDA was shown to overestimate the absorbed dose to the tumor. Accurate dosimetry may help select an activity more tailored to the specific size and location of the LA.

  6. Loss of retinoblastoma protein expression in spindle cell/pleomorphic lipomas and cytogenetically related tumors: an immunohistochemical study with diagnostic implications.

    PubMed

    Chen, Benjamin J; Mariño-Enríquez, Adrián; Fletcher, Christopher D M; Hornick, Jason L

    2012-08-01

    Consistent rearrangements of chromosomes 13q and 16q have been identified in spindle cell and pleomorphic lipomas by cytogenetics. Mammary-type myofibroblastoma and cellular angiofibroma show overlapping histologic features and similar chromosomal losses, suggesting a possible relationship among these tumor types. The tumor suppressor gene RB1, encoding the retinoblastoma (Rb) protein, is located at 13q14, within a minimally deleted region in spindle cell lipoma. The purpose of this study was to examine expression of Rb by immunohistochemistry in spindle cell lipoma, pleomorphic lipoma, mammary-type myofibroblastoma, and cellular angiofibroma, and in histologic mimics, to determine its potential diagnostic utility. Whole-tissue sections of 194 tumors were evaluated: 18 spindle cell lipomas, 20 pleomorphic lipomas, 19 mammary-type myofibroblastomas, 16 cellular angiofibromas, 22 conventional lipomas (8 intramuscular), 18 atypical lipomatous tumors (all positive for MDM2 and CDK4), 19 solitary fibrous tumors, 19 myxoid liposarcomas, 14 hibernomas, 11 deep (aggressive) angiomyxomas, 9 angiomyofibroblastomas, and 9 vulval fibroepithelial stromal polyps. Immunohistochemistry was performed after pressure cooker antigen retrieval using a mouse anti-Rb monoclonal antibody. Nuclear staining for Rb was scored as "intact" or "deficient." Rb expression was deficient in all spindle cell lipomas, pleomorphic lipomas, and cellular angiofibromas and in 17 (89%) mammary-type myofibroblastomas. Rb staining was sometimes difficult to interpret in cellular angiofibromas with reactive stromal changes. Rb was also deficient in 2 (9%) conventional lipomas. Rb expression was intact in all other tumor types evaluated. In summary, of the soft tissue tumors associated with 13q deletions, all spindle cell lipomas, pleomorphic lipomas, and cellular angiofibromas and most mammary-type myofibroblastomas show loss of Rb expression. Rb expression is intact in histologic mimics. These findings reinforce the pathogenetic relationship among this group of tumors and demonstrate the potential diagnostic utility of immunohistochemistry for Rb. PMID:22790852

  7. Vulvovaginal angiomyofibroblastomas: morphologic, immunohistochemical, and fluorescence in situ hybridization analysis for deletion of 13q14 region.

    PubMed

    Magro, Gaetano; Righi, Alberto; Caltabiano, Rosario; Casorzo, Laura; Michal, Michal

    2014-08-01

    Angiomyofibroblastoma (AMFB) is a benign tumor that belongs to the category of the "stromal tumors of the lower female genital tract," together with cellular angiofibroma and myofibroblastoma. Previous studies have shown overlapping morphologic and immunohistochemical features between these tumors and spindle cell lipoma, mammary-type myofibroblastoma, and vulvovaginal cellular angiofibroma and myofibroblastoma. In addition, typical loss of genetic material from the 13q14 region has been documented in all the above-mentioned tumors, suggesting that they are histogenetically related. We report the clinicopathologic features of 11 new cases of vulvovaginal AMFBs. Histologically, the basic common theme was a proliferation of bland-looking spindle to round-to-epithelioid cells set in an edematous to fibrous stroma, frequently arranged around thin-walled blood vessels. Two cases were composed of a prominent mature fatty component closely admixed with typical areas of AMFB, and thus, they were designated as "lipomatous AMFBs." Notably, 1 case was closely reminiscent of Sertoli cell tumor, sclerosing type, because of its predominant cord-like arrangement. Immunohistochemically, all tumors were diffusely positive for vimentin, whereas desmin and ?-smooth muscle actin were expressed in a minority of cases, suggesting a fibroblastic rather than myofibroblastic differentiation. Most cases of AMFBs coexpressed Bcl-2 protein and CD99. Interestingly, all 5 cases of AMFB with evaluable signals failed to show monoallelic loss of FOXO1 loci (13q14) by fluorescence in situ hybridization. These cytogenetic findings suggest that vulvovaginal AMFB is not genetically related to cellular angiofibroma and myofibroblastoma of the lower female genital tract. PMID:24880711

  8. Fluorescence In Situ Hybridization for MDM2 Amplification as a Routine Ancillary Diagnostic Tool for Suspected Well-Differentiated and Dedifferentiated Liposarcomas: Experience at a Tertiary Center

    PubMed Central

    Wang, Jayson; Swansbury, John; Min, Toon

    2015-01-01

    Background. The assessment of MDM2 gene amplification by fluorescence in situ hybridization (FISH) has become a routine ancillary tool for diagnosing atypical lipomatous tumor (ALT)/well-differentiated liposarcoma and dedifferentiated liposarcoma (WDL/DDL) in specialist sarcoma units. We describe our experience of its utility at our tertiary institute. Methods. All routine histology samples in which MDM2 amplification was assessed with FISH over a 2-year period were included, and FISH results were correlated with clinical and histologic findings. Results. 365 samples from 347 patients had FISH for MDM2 gene amplification. 170 were positive (i.e., showed MDM2 gene amplification), 192 were negative, and 3 were technically unsatisfactory. There were 122 histologically benign cases showing a histology:FISH concordance rate of 92.6%, 142 WDL/DDL (concordance 96.5%), and 34 cases histologically equivocal for WDL (concordance 50%). Of 64 spindle cell/pleomorphic neoplasms (in which DDL was a differential diagnosis), 21.9% showed MDM2 amplification. Of the cases with discrepant histology and FISH, all but 3 had diagnoses amended following FISH results. For discrepancies of benign histology but positive FISH, lesions were on average larger, more frequently in “classical” (intra-abdominal or inguinal) sites for WDL/DDL and more frequently core biopsies. Discrepancies of malignant histology but negative FISH were smaller, less frequently in “classical” sites but again more frequently core biopsies. Conclusions. FISH has a high correlation rate with histology for cases with firm histologic diagnoses of lipoma or WDL/DDL. It is a useful ancillary diagnostic tool in histologically equivocal cases, particularly in WDL lacking significant histologic atypia or DDL without corresponding WDL component, especially in larger tumors, those from intra-abdominal or inguinal sites or core biopsies. There is a significant group of well-differentiated adipocytic neoplasms which are difficult to diagnose on morphology alone, in which FISH for MDM2 amplification is diagnostically contributory.

  9. Hepatic angiomyolipoma: a series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy

    PubMed Central

    Agaimy, Abbas; Vassos, Nikolaos; Croner, Roland S; Strobel, Deike; Lell, Michael

    2012-01-01

    Hepatic angiomyolipoma is rare and may pose differential diagnostic difficulty, particularly if encountered in core needle biopsy. We studied 6 cases from 5 males and one female (median age, 48.6 yrs). All presented with non-specific symptoms or an incidentally discovered tumor mass. Two patients had a remote history of chemotherapy for hematological neoplasms (acute lymphoblastic leukemia and Hodgkin lymphoma respectively) and another had clear cell renal cell carcinoma and anaplastic pancreatic carcinoma diagnosed at autopsy without definable syndrome. None of the patients had evidence of the tuberous sclerosis complex or renal or other extra-renal angiomyolipoma. Three tumors were resected completely and three have been only biopsied and followed up. None of the resected cases recurred at a mean follow-up of 35 months. Histologically, tumors were classified as classical triphasic (1), lipomatous (2), epithelioid/oncocytoid (1), epithelioid trabecular (1) and myelolipoma-like (1). The adjacent liver parenchyma was normal in 3 cases, showed pigment cirrhosis in one case and mild fatty change in another case. One case had clinically diagnosed but histologically unverified cirrhosis. The initial diagnostic impression/frozen section was misleading in 5 of the cases and included vascular lesion, focal fatty change, myelolipoma, hepatocellular tumor and oncocytic neoplasm. All tumors expressed HMB45 and variably desmin. One epithelioid lesion expressed HMB45 and TFE3, but lacked desmin expression. In conclusion, hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms. PMID:22949933

  10. Islet vasculature in atrophic pancreas: evidence for coexisting parallel and serial (insuloacinar) angioarchitecture.

    PubMed

    Weaver, F C; Sorenson, R L

    1989-01-01

    Islet vasculature was studied in rats rendered dietarily copper-deficient, a regimen that effects progressive acinar atrophy while leaving islets and ducts intact. Scanning electron microscopy of corrosion casts produced from Mercox injected rats was used to analyze islet angioarchitecture in the atrophied gland. The results indicate that with the onset of acinar atrophy, the vasculature of islets remained intact despite loss of insuloacinar interconnectivity. With atrophy of the dense acinar vascular matrix, three populations of islets could be distinguished according to their size and vascular structures: (a) All small islets (40-150 microns in diameter) were found to have exclusively serial vasculature since no postcapillary collecting venules were found directly continuous with parallel efferent veins. (b) A second population of intermediate size islets (160-250 microns) was found to have serial vessels in addition to postcapillary collecting venules that were directly continuous with larger interlobular veins indicative of a parallel mode of microcirculation. (c) All larger islets (260-700 microns) were also found to have both serial as well as parallel microcirculatory patterns based upon the coexistence of both types of postcapillary venules within the same islet. In all cases, postcapillary venules, which normally link islet capillaries to peri-insular acinar plexuses, were continuous with veins that extended through lipomatous lobules devoid of acini to link with branches of interlobular veins. It is proposed that the functional integrity of islet blood flow remains in the atrophied pancreas because of preservation of both serial and parallel microcirculation that facilitate normal glucose-stimulated insulin secretion in the atrophied pancreas, as demonstrated in an earlier study. Furthermore, it is suggested that analysis of the atrophied state of the gland provides a valid comparative model with which to study islet angioarchitecture and microcirculation in the normal gland. PMID:2654925

  11. Ductal vasculature in the atrophic pancreas: evidence for autonomous ductal angioarchitecture.

    PubMed

    Weaver, F C

    1988-01-01

    Although it is known that ducts remain intact in the atrophied pancreata of rats rendered dietarily copper-deficient, no information is available that characterizes the ductal angioarchitecture of the experimentally altered gland. Furthermore, since the vascular relationship between pancreatic ducts and the parenchyma of the normal gland has not been well defined, this atrophy model was used to examine blood flow to ducts in the atrophied gland where acinar tissue undergoes selective necrosis. Scanning electron microscopy (SEM) of pancreatic corrosion casts produced from Mercox-injected rats was used. SEM was compared with light microscopic study of stain-injected cleared pancreata and correlated with histological and ultrastructural studies. The results indicate that duct cells and ductal vasculature remain unaffected by surrounding acinar cell necrosis and glandular lipomatous atrophy. Furthermore, the results support a largely autonomous blood supply for the ductal system by arterial blood that flows directly from major extralobular arteries of the pancreas or their interlobular branches. In the same manner, venous blood draining the ductal system returns to major extralobular veins or by way of their interlobular branches. No evidence could be established for manifest acinoductal or ductoacinar portal systems. There were, however, places in the route of both ductal and acinar vessels where the vessels were found to anastomose. These places were found near the source of major arteries or veins that supply and drain confluent acinar and ductal tissues. It is concluded that, while some acinoductal or ductoacinar exchange may take place, the role of these putative portal systems would appear less significant than the direct route of blood to and from pancreatic ducts. PMID:3222249

  12. Pancreatic pseudotumors: non-neoplastic solid lesions of the pancreas that clinically mimic pancreas cancer.

    PubMed

    Adsay, N Volkan; Basturk, Olca; Klimstra, David S; Klöppel, Günter

    2004-11-01

    In the pancreas, a variety of non-neoplastic conditions may form solid masses that may mimic cancer. Up to 5% of pancreatectomies performed with the preoperative clinical diagnosis of carcinoma will prove to be non-neoplastic by pathologic examination, although this figure is decreasing with improved diagnostic modalities. Chronic inflammatory lesions are the leading cause of this phenomenon ("pseudotumoral pancreatitis"), and among these, autoimmune and paraduodenal pancreatitides (discussed separately in this issue) are most important. In this article, we will focus on the noninflammatory lesions that may form tumor-like lesions of the pancreas. Adenomyomatous hyperplasia of ampulla of Vater is a subtle lesion that is difficult to define; larger examples (>5 mm) have been found to be the cause of obstructive jaundice. Accessory (heterotopic) spleen may form a well-defined nodule within the tail of the pancreas and is typically mistaken for endocrine neoplasm. Lipomatous hypertrophy is the replacement of pancreatic tissue with mature adipose tissue that occasionally leads to moderate to marked enlargement of the pancreas. Hamartomas are very rare if the entity is defined strictly. They are characterized by irregularly arranged mature pancreatic elements admixed with stromal tissue. A cellular, spindle-cell variant with c-kit (CD117) expression is recognized. Pseudolymphoma forms well-defined nodules composed of hyperplastic lymphoid tissue. Rarely, foreign-body deposits, granulomatous inflammations (such as sarcoidosis or tuberculosis), and congenital lesions may form tumoral lesions. In conclusion, it is important to recognize the types of conditions that form pseudotumors in the pancreas so that they can be distinguished from ductal adenocarcinomas, especially clinically, but also pathologically. Nonspecific terms such as "inflammatory pseudotumor" ought to be avoided, and every attempt should be made to classify a "pseudotumor" into a more specific diagnostic category discussed above. PMID:16273945

  13. Real-time polymerase chain reaction analysis of MDM2 and CDK4 expression using total RNA from core-needle biopsies is useful for diagnosing adipocytic tumors

    PubMed Central

    2014-01-01

    Background Diagnosing adipocytic tumors can be challenging because it is often difficult to morphologically distinguish between benign, intermediate and malignant adipocytic tumors, and other sarcomas that are histologically similar. Recently, a number of tumor-specific chromosome translocations and associated fusion genes have been identified in adipocytic tumors and atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDL), which have a supernumerary ring and/or giant chromosome marker with amplified sequences of the MDM2 and CDK4 genes. The purpose of this study was to investigate whether quantitative real-time polymerase chain reaction (PCR) could be used to amplify MDM2 and CDK4 from total RNA samples obtained from core-needle biopsy sections for the diagnosis of ALT/WDL. Methods A series of lipoma (n?=?124) and ALT/WDL (n?=?44) cases were analyzed for cytogenetic analysis and lipoma fusion genes, as well as for MDM2 and CDK4 expression by real-time PCR. Moreover, the expression of MDM2 and CDK4 in whole tissue sections was compared with that in core-needle biopsy sections of the same tumor in order to determine whether real-time PCR could be used to distinguish ALT/WDL from lipoma at the preoperative stage. Results In whole tissue sections, the medians for MDM2 and CDK4 expression in ALT/WDL were higher than those in the lipomas (P?

  14. Multiple endocrine neoplasia type 1 and 2: from morphology to molecular pathology 1997.

    PubMed

    Komminoth, P

    1997-01-01

    Multiple Endocrine Neoplasia (MEN) is an inherited syndrome which appears in two major forms referred to as type 1 (MEN-1) and type 2 (MEN-2). MEN-1 is characterized by the occurrence of neuroendocrine parathyroid, pancreas, duodenum and pituitary lesions. In addition to these tumors adrenocortical, lipomatous and neuroendocrine tumors in other locations may develop. The genetic defect of MEN-1 has recently been identified and involves a new form of tumor suppressor gene called mu on chromosome 11q13. It codes for a protein called menin which is expressed in a variety of human tissues and organs. In MEN-1 gene carriers inactivating germline frameshift, nonsense, missense and in-frame deletion mutations scattered throughout the 10 coding exons have been identified. The MEN-2 syndrome is divided into three clinical variants referred to as MEN-2A, MEN-2B and familial medullary thyroid carcinoma (FMTC) which share medullary thyroid carcinomas as part of the disease phenotype. In MEN-2A pheochromocytomas and parathyroid hyperplasia and in MEN-2B additional skeletal abnormalities and ganglioneuromatosis may also be encountered. All three MEN-phenotypes are associated with oncogenic point mutations of the RET protooncogene on chromosome 10q11.2 which encodes a receptor-type tyrosine kinase. Its ligand--the glial cell line derived neurotropic factor (GDNF)--forms a signaling complex with the alpha type of the GDNF receptor. All neuroendocrine tumors of the different MEN-phenotypes may also occur sporadically and there are only few clinical and pathomorphological features which are helpful to discriminate sporadic from MEN-associated neuroendocrine neoplasms. The recent achievements of molecular pathology now allow for the unambiguous identification of MEN gene carriers among patients with neuroendocrine neoplasms by DNA testing for mutations in the mu and RET gene. In this overview, distinct macroscopic and histopathological features of the two MEN phenotypes will be summarized and most recent findings on the molecular pathology of these syndromes will be outlined, together with molecular methods to identify disease-gene carriers. PMID:9474863

  15. Scope of FNAC in the diagnosis of soft tissue tumors-A study from a tertiary cancer referral center in India

    PubMed Central

    Rekhi, Bharat; Gorad, Biru D; Kakade, Anagha C; Chinoy, RF

    2007-01-01

    Background Fine needle aspiration cytology (FNAC) forms one of the first diagnostic tools in the evaluation of tumors. Its role in diagnosing soft tissue tumors (STT) has been fairly documented, as well as debated. Present study was aimed at evaluating its scope in diagnosing 127 cases of soft tissue tumors. Methods Conventional Pap and MGG staining was available in all the cases. Immunocytochemistry (ICC) was performed in 15 cases. Histopathological details were available in 115 cases. Results 50% cases were referred for a primary diagnosis, while 26.8% & 22.8% cases were evaluated for recurrent and metastatic lesions, respectively. Extremities were the commonest sites. On FNAC, 101 cases (79.5%) were labeled as malignant, whereas 10 cases (7.9%) were labeled as benign. The remaining 16 cases (11%) were not categorized and were labeled as 'unsure/not specified'. Histopathological confirmation in 115 cases, gave a diagnostic accuracy of 98%, with a positive predictive value of 98% in malignant cases and a negative predictive value of 100% in benign cases. Two cases were false positive. Among the various cytological categories, 60 cases (47.2%) were of spindle cell type, followed by 32 (25.2%) of round cell type and 14 cases (11%) of lipomatous type. Other 12 cases (9.4%) were of pleomorphic type; 7 (5.5%) cases of epithelioid type and remaining 2 cases were of myxoid type. All the round cell, pleomorphic and myxoid type of tumors were sarcomas, whereas 73.3% cases of spindle cell type were labeled as 'malignant'. Exact cytological sub typing was offered in 58 cases, with rhabdomyosarcoma (RMS) as the most frequently sub typed tumor. The two false positive malignant cases were of fibromatosis and a pigmented schwannoma, on biopsy. Out of 28 metastatic lesions, lymph nodes were the commonest site for metastasis, with epithelioid tumors that formed highest percentage of metastatic cases. Conclusion FNAC is fairly specific and sensitive in STT diagnoses for primary, recurrent and metastatic lesions. The cytological types, especially round cell and pleomorphic sarcomas, can be quickly identified. Clinicopathological correlation with ICC as an adjunct, are valuable in exact sub typing. PMID:17973999

  16. Morphologic characterization of spontaneous nervous system tumors in mice and rats.

    PubMed

    Krinke, G J; Kaufmann, W; Mahrous, A T; Schaetti, P

    2000-01-01

    Spontaneous rodent nervous system tumors, in comparison to those of man, are less well differentiated. Among the central nervous system (CNS) tumors, the "embryonic" forms (medulloblastoma, pineoblastoma) occur both in rodents and humans, whereas the human "adult" forms (gliomas, ependymomas, meningiomas) have fewer counterparts in rodents. In general, the incidence of spontaneous CNS tumors is higher in rats (>1%) than in mice (>0.001%). A characteristic rat CNS tumor is the granular cell tumor. Usually it is associated with the meninges, and most meningeal tumors in rats seem to be totally or at least partly composed of granular cells, which have eosinophilic granular cytoplasm, are periodic acid-Schiff reaction (PAS)-positive, and contain lysosomes. Such tumors are frequently found on the cerebellar surface or at the brain basis. Rat astrocytomas are diffuse, frequently multifocal, and they invade perivascular spaces and meninges. The neoplastic cells with round to oval nuclei and indistinct cytoplasm grow around preexisting neurons, producing satellitosis. In large tumors, there are necrotic areas surrounded by palisading cells. Extensive damage of brain tissue is associated with the presence of scavenger cells that react positively with histiocytic/macrophage markers. The neoplastic astrocytes do not stain positively for glial fibrillary acidic protein; they probably represent an immature phenotype. In contrast to neoplastic oligodendroglia, they bind the lectin RCA-1. Astrocytomas are frequently located in the brain stem, especially the basal ganglia. Rat oligodendroglial tumors are well circumscribed and frequently grow in the walls of brain ventricles. Their cells have water-clear cytoplasm and round, dark-staining nuclei. Atypical vascular endothelial proliferation occurs, especially at the tumor periphery. Occasionally in the oligodendrogliomas, primitive glial elements with large nuclei occur in the form of cell groups that form rows and circles. Primitive neuroectodermal tumors of rats, such as pineal tumors or medulloblastomas, appear to have features similar to those found in man. In mice, the meningeal tumors are mostly devoid of granular cells and the astrocytomas are similar to those occurring in rats, whereas spontaneous oligodendrogliomas are observed extremely rarely. Tumorlike lesions, such as lipomatous hamartomas or epidermoid cysts, are occasionally encountered in the mouse CNS. It is suggested that we classify rodent CNS lesions as "low grade" and "high grade" rather than as "benign" and "malignant." The size of CNS tumors is generally related to their malignancy. Tumors of the peripheral nervous system are schwannomas and neurofibromas or neurofibrosarcomas consisting of Schwann cells, fibroblasts, and perineural cells. Well-differentiated schwannomas are characterized by S-100 positivity and the presence of basement membrane. They show either Antoni A pattern with fusiform palisading cells or Antoni B pattern, which is sparsely cellular and has a clear matrix. The rat develops specific forms of schwannomas in the areas of the submandibular salivary gland, the external ear, the orbit, and the endocardium. Spontaneous ganglioneuromas occur in the rat adrenal medulla or thyroid gland. Compared to experimentally induced neoplasms, the spontaneous tumors of the rodent nervous system are poor and impractical models of human disease, although they may serve as general indicators of the carcinogenic potential of tested chemicals. PMID:10669006