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Epithelioid GIST differential diagnosis

Zehong Chen, Huijuan Shi, Jianjun Peng, Yujie Yuan, Jianhui Chen, Wu Song
Defined as a family of scarce mesenchymal neoplasm which distinctively co-express melanocytic markers and muscle markers, perivascular epithelioid cell tumors (PEComas) have been reported almost everybody site. Perivascular epithelioid cell tumors-not otherwise specified (PEComas-NOS) arising in the gastrointestinal (GI) tract are still restricted into sporadic case reports. Herein we present a case of GI PEComas-NOS which occurs in the duodenum of a 27-year-old male. Our initial diagnosis tended to gastrointestinal stromal tumor or smooth muscle tumor till the correct diagnosis of perivascular epithelioid cell tumor (PEComa) was established by postoperative pathological examination...
2015: International Journal of Clinical and Experimental Pathology
Aaron W James, Sarah M Dry
In this article, we focus on the histologic features, differential diagnosis, and potential pitfalls in the diagnosis of epithelioid sarcoma, alveolar soft part sarcoma, clear-cell sarcoma, ossifying fibromyxoid tumor, and malignant extrarenal rhabdoid tumor. Numerous other soft tissue tumors also may have epithelioid variants or epithelioid features. Examples include epithelioid angiosarcoma, epithelioid malignant peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, and perivascular epithelioid cell tumor, among others...
September 2015: Surgical Pathology Clinics
Markku Miettinen, Peter A McCue, Maarit Sarlomo-Rikala, Wojciech Biernat, Piotr Czapiewski, Janusz Kopczynski, Lester D Thompson, Jerzy Lasota, Zengfeng Wang, John F Fetsch
Sox10 transcription factor is expressed in schwannian and melanocytic lineages and is important in their development and can be used as a marker for corresponding tumors. In addition, it has been reported in subsets of myoepithelial/basal cell epithelial neoplasms, but its expression remains incompletely characterized. In this study, we examined Sox10 expression in 5134 human neoplasms spanning a wide spectrum of neuroectodermal, mesenchymal, lymphoid, and epithelial tumors. A new rabbit monoclonal antibody (clone EP268) and Leica Bond Max automation were used on multitumor block libraries containing 30 to 70 cases per slide...
June 2015: American Journal of Surgical Pathology
Abbey J Winant, Marc J Gollub, Jinru Shia, Christina Antonescu, Manjit S Bains, Marc S Levine
OBJECTIVE: The purpose of this article is to describe the imaging and clinicopathologic characteristics of esophageal gastrointestinal stromal tumors (GISTs) and to emphasize the features that differentiate esophageal GISTs from esophageal leiomyomas. MATERIALS AND METHODS: A pathology database search identified all surgically resected or biopsied esophageal GISTs, esophageal leiomyomas, and esophageal leiomyosarcomas from 1994 to 2012. Esophageal GISTs were included only if imaging studies (including CT, fluoroscopic, or (18)F-FDG PET/CT scans) and clinical data were available...
August 2014: AJR. American Journal of Roentgenology
M R Ambrosio, B J Rocca, M G Mastrogiulio, A Pesci, A De Martino, M A Mazzei, L Volterrani, F Arcuri, M Cintorino, S A Tripodi
Gastrointestinal stromal tumors (GISTs) represent a distinct subset of mesenchymal tumours of the gastrointestinal tract. They are more common in the stomach and small intestine, and are characterized by the proliferation of spindle or epithelioid cells and by the expression of CD117. Extra-gastrointestinal stromal tumors are rare and only 13 cases of pancreatic GISTs have been reported in the literature, only 1 of which presented as a cystic lesion. Mutational analysis of KIT and Platelet derived growth factor receptor-α genes was performed only in two out of the 13 cases...
December 2014: Histology and Histopathology
Markku Miettinen
Immunohistochemistry is an integral component in the proper analysis of soft tissue tumours, and a simple panel of six markers is useful in practical triage: CD34, desmin, epithelial membrane antigen (EMA), keratin cocktail AE1/AE3, S100 protein and alpha smooth muscle actin (SMA). These markers frequently assist in the differential diagnosis of fibroblastic, myoid, nerve sheath and perineurial cell tumours, synovial and epithelioid sarcoma and others. However, they all are multispecific, so that one has to be cognizant of their distribution in normal and neoplastic tissues...
January 2014: Histopathology
Kosuke Okuwaki, Mitsuhiro Kida, Hironori Masutani, Hiroshi Yamauchi, Hiroyuki Katagiri, Tetuo Mikami, Shiro Miyazawa, Tomohisa Iwai, Miyoko Takezawa, Hiroshi Imaizumi, Wasaburo Koizumi
Primary perivascular epithelioid cell tumors (PEComas) of the pancreas are extremely rare. We herein report our experience with a patient who had a primary PEComa of the pancreas that was diagnosed by the preoperative histopathological examination of a biopsy specimen obtained by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The patient was a 43-year-old woman whose chief complaint was abdominal pain. Imaging studies revealed a pancreatic tumor. Gastrointestinal stromal tumor (GIST), solid pseudopapillary tumor and neuroendocrine tumor were considered in the differential diagnosis...
2013: Internal Medicine
Guhyun Kang, Young Soo Park, Eun-Sun Jung, Mee Joo, Mi Seon Kang, Soomin Ahn, Gu Hyum Kang, Kyoung-Mee Kim
Studies on gastrointestinal stromal tumors (GISTs) in young patients are limited due to their rarity, and none have been conducted in Asian populations. GISTs from patients under the age of 30 were retrospectively reviewed and were analyzed for clinicopathologic features, immunohistochemistry for SDHB (succinate dehydrogenase subunit B), and mutations for exon 9, 11, 13, and 17 of KIT gene and exon 12, 14, and 18 of PDGFRA gene. We found two pediatric (<18 years old) and 20 young adult (18-30 years old) GIST cases...
October 2013: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Ondrej Daum, Tomas Vanecek, Zdenek Benes, Michal Michal
Gastrointestinal stromal tumor (GIST) is the most frequent mesenchymal tumor of the alimentary tract, presently being defined as a tumor composed of spindle and/or epithelioid cells presumably differentiating towards interstitial cells of Cajal. The most frequent location of gastrointestinal stromal tumor is the stomach, followed by other sites of gastrointestinal tract. Occasional sites of occurrence are mesenterium, omentum, retroperitoneum, gall bladder, urinary bladder, pancreas, prostate and the vagina...
November 2007: Expert Opinion on Medical Diagnostics
Leona A Doyle, Wei-Lien Wang, Paola Dal Cin, Dolores Lopez-Terrada, Fredrik Mertens, Alexander J F Lazar, Christopher D M Fletcher, Jason L Hornick
Sclerosing epithelioid fibrosarcoma (SEF) is a rare aggressive fibroblastic neoplasm composed of cords of epithelioid cells embedded in a dense collagenous stroma. The reported immunophenotype of SEF is nonspecific. Some SEF cases show morphologic and molecular overlap with low-grade fibromyxoid sarcoma (LGFMS), suggesting a relationship between these tumor types. MUC4 has recently been identified as a sensitive and specific marker for LGFMS; MUC4 expression was also observed in 2 tumors with hybrid features of SEF and LGFMS...
October 2012: American Journal of Surgical Pathology
Gong-wei Wang, Ying Wang, Yun-xin Chen, Qing Li, Dan-hua Shen
OBJECTIVE: To study the clinicopathologic features and differential diagnosis of perivascular epithelioid cell tumors (PEComas) occurring in the urinary system. METHODS: The clinicopathologic features of 21 cases of PEComa from September 2002 to September 2010 occurring in the urinary system were retrospectively reviewed. Immunohistochemical study for HMB 45, S-100 protein, smooth muscle actin, desmin, Melan A and Ki-67 was carried out. RESULTS: Amongst the 21 cases studied, there were 5 males and 16 females...
July 2012: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Loiy Alkhatib, Omar Albtoush, Nesreen Bataineh, Kamal Gharaibeh, Ismail Matalka, Yasuharu Tokuda
INTRODUCTION: Gastro Intestinal Stromal Tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract (GI). GIST that arises primarily outside the GI tract is termed Extragastrointestinal Stromal Tumor (EGIST). To the best of our knowledge, few cases of EGIST in the abdominal wall were reported. PRESENTATION OF CASE: We present a rare case of EGIST in the abdominal wall of a 57 year-old female patient. The asymptomatic tumor was located in the superior aspect of the left rectus abdominis muscle, measured 5...
2011: International Journal of Surgery Case Reports
Markku Miettinen, Jerzy Lasota
Gastrointestinal stromal tumor (GIST), generally driven by oncogenic KIT or PDGFRA mutations, is the most common mesenchymal tumor of the gastrointestinal (GI) tract. GIST is most common in the stomach (60%) and small intestine (30%), but can occur anywhere in the GI-tract and the intra-abdominal soft tissues. GIST can show spindle cell or epithelioid morphology, and mitotic count and tumor size are most important prognostic parameters. GISTs in NF1 patients and children are distinctive clinicopathologic groups...
December 2011: Journal of Surgical Oncology
Vickie Y Jo, Christopher D M Fletcher
p63 is a p53 homolog that is expressed in various normal epithelial tissues and epithelial malignancies. Its expression in mesenchymal lesions has not been examined in depth; therefore, we studied p63 expression by immunohistochemical analysis in 650 soft tissue tumors. We found that p63 expression is limited in soft tissue tumors. The majority of tumors studied were p63-, including all cases of angiosarcoma, lipomatous neoplasms, dermatofibrosarcoma protuberans, solitary fibrous tumor, schwannoma, neurofibroma, gastrointestinal stromal tumor, and leiomyosarcoma...
November 2011: American Journal of Clinical Pathology
Qi Sun, Hong-yan Wu, Xin-yan Chen, Jun Yang, Qing Ye, Xiang-shan Fan
No abstract text is available yet for this article.
June 2011: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Ozgur Mete, Theodorus H van der Kwast
This review examines the histopathologic, immunohistochemical, ultrastructural, and molecular biologic features of epithelioid angiomyolipoma (EAML), with an emphasis on the differential diagnosis of intra-abdominal EAML. Epithelioid angiomyolipoma is an uncommon mesenchymal tumor with malignant potential, frequently associated with tuberous sclerosis complex. Histologically, EAML is characterized by sheets or nests of large polygonal epithelioid cells with abundant eosinophilic or occasionally clear cytoplasm, often with prominent nucleoli, and EAML may include multinucleated and markedly pleomorphic forms...
May 2011: Archives of Pathology & Laboratory Medicine
Jun-na Cai, Min Shi, Jian Wang
No abstract text is available yet for this article.
January 2011: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Mukul Vij, Vinita Agrawal, Rakesh Pandey
CONTEXT: Gastrointestinal stromal tumors are CD117 (C-Kit) positive mesenchymal neoplasms considered to originate from the interstitial cells of Cajal. Gastrointestinal stromal tumors have been described outside the gastrointestinal tract in sites, such as the mesentery, omentum and retroperitoneum; however, pancreatic extra-gastrointestinal stromal tumors are extremely rare and there have only been seven previous reports in the literature. CASE REPORT: We describe a 38-year-old man with a malignant pancreatic gastrointestinal stromal tumor...
March 2011: JOP: Journal of the Pancreas
Runjan Chetty
Reticular/microcystic schwannoma is a rare variant of schwannoma with a predilection for viscera, especially the gastrointestinal tract. Of the 9 cases reported thus far, 7 have occurred in female patients. The average age of presentation is 67 years. They are usually small, asymptomatic lesions ranging in size from 0.85 to 2.2 cm with a mean size of 1.35 cm. They usually occur in the stomach, small bowel, and proximal large intestine. It differs from usual schwannomas of the gastrointestinal tract by lacking the peripheral cuff of lymphocytes...
June 2011: Annals of Diagnostic Pathology
Juan B Laforga Canales, Joan M Gasent Blesa
Epithelioid cell tumors presenting in the gastrointestinal tract are uncommon, but when they arise, arriving at a correct diagnosis is important. We report a case of anal malignant melanoma in an 82-year-old man who microscopically showed an epithelioid malignant tumor simulating a gastrointestinal stromal tumor. C-kit stain and Melan-A were diffusely and strongly positive, while HMB-45 was focally positive. This case illustrates the potential pitfall of relying on a single antibody or inadequate panel of immunohistochemical stains to confirm the diagnosis...
2009: Case Reports in Oncology
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