title: Malignant mesenteric perivascular epithelioid cell neoplasm presenting as an intra-abdominal fistula in a 49-year-old female.
abstract: Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring 13.5 × 7.7 × 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication.
authors.affiliation: Department of Internal Medicine, Overlook Medical Center, 99 Beauvoir Avenue, Summit, NJ 07902, USA.
authors.forename: Sakshi
authors.identifier: 0000-0003-1310-0505
authors.initials: S
authors.lastname: Kapur
authors.affiliation: Department of Internal Medicine, Overlook Medical Center, 99 Beauvoir Avenue, Summit, NJ 07902, USA.
authors.forename: Napoleon K
authors.initials: NK
authors.lastname: Patel
authors.affiliation: Division of Pathology, Overlook Medical Center, 99 Beauvoir Avenue, Summit, NJ 07902, USA.
authors.forename: Miles B
authors.initials: MB
authors.lastname: Levin
authors.affiliation: MS III, St. George's University School of Medicine, True Blue, Grenada.
authors.forename: Richard
authors.identifier: 0000-0001-5235-3690
authors.initials: R
authors.lastname: Huang
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