PECOMA – A Histological Surprise

*, Ashwin Kumar C and Pai, Manohar V and Lobo, Flora Dorothy (2015) PECOMA – A Histological Surprise. International Journal of Science and Research (IJSR), 4 (2). pp. 1076-1079. ISSN 2319-7064

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Abstract

Introduction: Perivascular epithelioid cell tumor (PEComa) is rare neoplasm arising from mesenchymal cells with distinctive histologic, immunohistochemistry and clinical characteristics. It probably arises through activation of the mTOR signaling pathway. Malignant PEComa are extremely rare with very few cases being reported in literature. They occur at various anatomic sites in the body and have distinctive clinical presentation and variable prognosis. Presentation Of Case: We present a case of 39 yr old male patient who presented with anemia and palpable abdominal mass. He had previous history of two laprotomies for abdominal mass histologically suspected of GIST. He was evaluated with regular blood investigations, bone marrow examination and CECT Abdomen. Laprotomy was done and a tumor was found arising from the previous anastomotic site and transverse mesocolon. Clinically suspected as recurrent/malignant GIST, tumor was excised and sent for histopathology examination. At histopathology tumor sections showed perivascular pattern of spindle shaped epithelioid cells with atypia, high mitotic figures, and necrosis. IHC marking was positive for HMB45, SMA, Melan-A, Desmin, and immunonegative for S-100, ckit and DOG-1. Thus confirming the diagnosis of Malignant PEComa and patient kept on follow up. Discussion: The earliest description of PEComa date back to 1943 when they were first described as an of GIT are even Conclusion: Malignant have are from various case Keywords: PEComa, HMB45, SMA, DESMIN, Malignant PEComa, Colon, Recurrent GIST 1. Introduction Perivascular epithelioid cell tumor (PEComa) is an commonly at reported. In 1991 Bonnetti et mesenchymal term was used to tumor of lung. Zamboni of sugar tumor in tumors existing at many anatomical sites in body. To date PEComa have been described in many anatomic sites like liver, heart, rectum, breast, uterus, abdominal wall, and pancreas. PEComas are generally benign lesions with variable presentation and prognosis is usually good. Malignant PEComas are extremely rare and may have local and distal metastasis. To date there have been only fifty five cases of malignant PEComa reported in literature with very few being reported from gastrointestinal tract. PEComa of mesentery exceedingly rare with only 6 cases being reported in literature. Immunohistochemically, nearly all PEComa are immunoreactive for both smooth muscle (actin/desmin) and melanocytic (HMB45/melanA) markers. There are no to these cells. 2. Case We present the case of a thirty nine year old male patient he had two to in ICU setting. He of with platelet counts at 7 lakhs and CECT abdomen showed cavitatory mass lesion in transverse colon and mesentery overlying the pervious anastomotic site with lymph nodes. Initial working diagnosis was made as recurrent/malignant GIST with suspected myeloproilferative disorder. Bone marrow examination revealed normal study. JAK2 and BCR ABL mutation study was negative. In hospital course patient improved symptomatically but anemia failed to improve with persistent low hemoglobin. Patient was taken up laprotomy. Intraop large lesion size of 15x20cms was found in the previous anastomotic site involving adjacent transverse colon and mesentery. Tumor was freed of adhesions from previous surgery and excised in toto with ileo-transverse anastomosis. Specimen was sent for histopathology examination. Paper ID: SUB151405 1076 abnormal myoblast found in section specimen of renal angiomyolipoma. PEComa are rare tumors with variable presentation. There have been around 100 reported cases of PEComa with around 55 being malignant. Malignant PEComas rarer with few cases reported. They have an aggressive course with mean life time of around 30months. Conclusion PEComas are very rare tumors with aggressive course and no known effective treatment. The information we series and reports. The article is to sensitize the occurence of this tumor in case of histologically proven GIST, its clinical and histologic diagnostic difficulties and probable treatment options.

Item Type: Article
Uncontrolled Keywords: PEComa, HMB45, SMA, DESMIN, Malignant PEComa, Colon, Recurrent GIST
Subjects: Medicine > KMC Mangalore > Pathology
Medicine > KMC Mangalore > Surgery
Depositing User: KMCMLR User
Date Deposited: 24 Oct 2016 11:18
Last Modified: 24 Oct 2016 11:18
URI: http://eprints.manipal.edu/id/eprint/147392

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