Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically\ndistinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise\nfrom different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female\nwho presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass\nmeasuring 13.5 Ã?â?? 7.7 Ã?â?? 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into\nthe right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology\nresults were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to\nmake a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with\nreservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on\neverolimus, an m-TOR inhibitor, and has shown good response to this medication.
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