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Perivascular epithelioid cell tumor (PEComa) and Gastrointestinal

Yuka Kiriyama, Tetsuya Tsukamoto, Yoshikazu Mizoguchi, Shin Ishihara, Akihiko Horiguchi, Takamasa Tokoro, Yutaro Kato, Atsushi Sugioka, Makoto Kuroda
BACKGROUND: Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. CASE PRESENTATION: We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas...
2016: Diagnostic Pathology
Zehong Chen, Siqi Han, Jialin Wu, Minmin Xiong, Yanqiao Huang, Jianhui Chen, Yujie Yuan, Jianjun Peng, Wu Song
Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas-NOS) is still unclear. The aim of conducting this systematic review is to sum up what is known so far of the epidemiology, natural history, management and prognosis of GI PEComas-NOS...
July 2016: Medicine (Baltimore)
Rosa Virginia Acosta Materán, María Isabel Martín Arribas, Antonio Velasco Guardado, Cristina González Velasco, Ana María Mora Soler, Cristina Revilla Morato, Antonio Rodríguez Pérez
Perivascular epithelioid cell tumors (PEComa) are tumors of perivascular epithelioid cells with immunohistochemical features of smooth muscle and melanocytic tumors. The PEComa of the gastrointestinal tract is rare. The treatment is surgical, although there are data that suggest a good response to rapamycin.
February 22, 2016: Revista Española de Enfermedades Digestivas
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
Biyan Lu, Chenliang Wang, Junxiao Zhang, Roland P Kuiper, Minmin Song, Xiaoli Zhang, Shunxin Song, Ad Geurts van Kessel, Aikichi Iwamoto, Jianping Wang, Huanliang Liu
Perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas) are exceedingly rare, with only a limited number of published reports worldwide. Given the scarcity of GI PEComas and their relatively short follow-up periods, our current knowledge of their biologic behavior, molecular genetic alterations, diagnostic criteria, and prognostic factors continues to be very limited.We present 2 cases of GI PEComas, one of which showed an aggressive histologic behavior that underwent multiple combined chemotherapies...
January 2015: Medicine (Baltimore)
Huijuan Shi, Qinghua Cao, Hui Li, Tiantian Zhen, Yingrong Lai, Anjia Han
AIMS: To report one case of malignant perivascular epithelioid cell tumor (PEComa) of the kidney with rare pulmonary and ileum metastases and analyze its clinicopathological features. METHODS: We analyzed the clinicopathological features of one case of malignant PEComa of the kidney with pulmonary and ileum metastases. Immunohistochemistry staining was performed. RESULTS: The patient was a 48-year-old man with a renal mass approximately 14 cm × 11 cm × 8 cm in size...
2014: International Journal of Clinical and Experimental Pathology
Marco Pizzi, Ida di Lorenzo, Emanuele S d'Amore, Paolo D'Angelo, Rita Alaggio
Perivascular epithelioid cell tumors (PEComas) in the pediatric population are very rare and frequently arise in the gastrointestinal tract. These tumors are characterized by variable morphological and immunohistochemical features, which may pose significant problems in differential diagnosis with other gastrointestinal mesenchymal tumors in pediatric patients. In presenting an unusual pediatric case of gastrointestinal PEComa, we also discuss the possible differential diagnosis of this rare entity.
September 2014: Pediatric and Developmental Pathology
Daniele Crocetti, Giuseppe Pedullà, Maria Rita Tarallo, Antonietta De Gori, Annalisa Paliotta, Giuseppe Cavallaro, Giorgio De Toma
BACKGROUND: Perivascular epithelioid cell tumors (PEComas), make up a family of extremely rare mesenchymal neoplasms, with characteristic morphological, immunohistochemical and molecular findings. Malignant PEComas and gastrointestinal epithelioid angiomyolipoma (E-AML) are especially rare. To the best of our knowledge E-AML have not been found in the breast. The difficulty in determining what constitutes optimal therapy for PEComas, owing to the sparse literature available, led us to report this rare case...
March 2014: Annali Italiani di Chirurgia
Leona A Doyle, Jason L Hornick, Christopher D M Fletcher
Perivascular epithelioid cell tumors (PEComas) are distinctive mesenchymal neoplasms that most often arise in the retroperitoneum, visceral organs, and abdominopelvic sites and usually show reactivity for melanocytic and smooth muscle markers. Fewer than 20 PEComas of the gastrointestinal (GI) tract have been reported, and behavior and criteria for malignancy are incompletely defined. The purpose of this study was to examine the clinicopathologic features of a series of GI PEComas and to evaluate prognostic parameters...
December 2013: American Journal of Surgical Pathology
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
Young Whan Cho, Kyung Jo Kim, Byong Duk Ye, Jeong Sik Byeon, Seung Jae Myung, Suk Kyun Yang, Jin Ho Kim
Perivascular epithelioid cell tumor (PEComa) is extremely rare, which originated from mesenchymal cells in the intestine, and composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. We report here on a case of PEComa in the sigmoid colon. A 62-year-old woman presented with hematochzia 10 days ago. Her abdominal computed tomography scan showed a 5 cm sized intraluminal fungating heterogeneously enhanced, high density mass, which infiltrated pericolic tissue surrounding the sigmoid colon...
December 2012: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
Matteo Fassan, Mauro Cassaro, Massimo Vecchiato, Roberto Clemente, Gianmaria Pennelli, Stefano Merigliano, Giuseppe Altavilla
Malignant perivascular epithelioid cell tumor (PEComa) is a rare tumor composed of hybrid tumor cells characterized by immunoreactivity for both melanocytic and smooth muscle markers. This paper describes the uncommon esophageal location of an 8 cm PEComa in a 75-year-old Caucasian man who was presented with ingravescent dysphagia. Although PEComas arising within the gastrointestinal tract are exceptional findings, clinicians should not exclude this class of tumors in the diagnostic investigation of a bulky lesion of the esophageal wall...
2012: Case Reports in Pathology
Saime Unluoglu, Umit Bayol, Nilay Korkmaz, Bekir Ozenen, Fuat Ipekci, Emel Ebru Pala
Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed...
2012: Case Reports in Pathology
Constantine Gennatas, Vasiliki Michalaki, Paraskevi Vasilatou Kairi, Agathi Kondi-Paphiti, Dionysios Voros
Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm that appears to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites. Malignant PEComas exist but are very rare. These tumors represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. Metastatic PEComa is a rare form of sarcoma for which no effective therapy has been described previously and that has a uniformly fatal outcome...
2012: World Journal of Surgical Oncology
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
Newton A C S Wong, Zsombor Melegh
AIMS: Gastrointestinal stromal tumour (GIST) may share morphological and/or immunohistochemical features with various intra-abdominal neoplasms, including endometrial stromal sarcoma, perivascular epithelioid cell tumour (PEComa), melanoma and synovial sarcoma. Each of these various neoplasms has characteristic immunohistochemical markers, including epithelial membrane antigen (EMA), CD10, oestrogen receptor alpha (ERa) and/or HMB45, and therefore the primary aim of this study was to determine whether these markers are also expressed by GISTs...
October 2011: Histopathology
Carmen Ardeleanu, Gianni Bussolati
We advance the hypothesis that the telocyte might be the cell of origin of both PEComas (perivascular epithelioid cell tumours) and GISTs (gastro-intestinal and extra-gastrointestinal stromal tumours). The hypothesis is supported by data from the literature reporting that both PEComas and GISTs, as well as telocytes, share the expression of several markers. These data were supplemented by original immunohistochemical tests on selected series. Specifically: (1) Melanoma markers (Melan A, MiTF) typical of PEComas are expressed by a substantial fraction of GISTs...
December 2011: Journal of Cellular and Molecular Medicine
Jun-Na Cai, Min Shi, Jian Wang
OBJECTIVE: To study the clinicopathologic characteristics of perivascular epithelioid cell tumor (PEComa), not otherwise specified (NOS) and to evaluate the diagnostic criteria for malignancy. METHODS: The clinical and pathologic features of 31 cases of PEComa-NOS were reviewed. The follow-up data available were analyzed. RESULTS: There were a total of 24 females and 7 males. The age of the patients ranged from 13 to 66 years (mean = 40 years)...
April 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
Pedram Argani, Sebastian Aulmann, Peter B Illei, George J Netto, Jae Ro, Hyun-yee Cho, Snjezana Dogan, Marc Ladanyi, Guido Martignoni, John R Goldblum, Sharon W Weiss
Perivascular epithelioid cell neoplasms (PEComas) include the common renal angiomyolipoma, pulmonary clear cell sugar tumor, lymphangioleiomyomatosis, and less common neoplasms of soft tissue, gynecologic, and gastrointestinal tracts. Recently, aberrant immunoreactivity for TFE3 protein (a sensitive and specific marker of neoplasms harboring TFE3 gene fusions) has been reported in as many as 100% of PEComas; however, TFE3 gene status in these neoplasms has not been systematically investigated. We used a fluorescence in situ hybridization (FISH) break-apart assay to evaluate for evidence of TFE3 gene fusions in archival material from 29 PEComas...
October 2010: American Journal of Surgical Pathology
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