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Open Access Case Report

Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature

Xinge Fu1, Ju-hong Jiang1*, Xia Gu1 and Zhi Li2

Author Affiliations

1 Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, 151, Yanjiang Road, Guangzhou, 510120, China

2 Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, 58, Zhongshan Road II, Guangzhou, 510080, China

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Diagnostic Pathology 2013, 8:60  doi:10.1186/1746-1596-8-60

Published: 15 April 2013

Abstract

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Perivascular epithelioid cell tumor (PEComa) is a rare but distinct mesenchymal neoplasm composed of histologically and immunohistochemically unique perivascular epithelioid cells. Due to its relative rarity, little is known about the histogenesis and prognostic factors of this tumor. We describe a case of unusual mesenteric PEComa in a 38-year-old female patient with regional lymph node involvement. Histologically, the tumor was composed of sheet of epithelioid cells with abundant clear or eosinophillic cytoplasms. Extensive coagulative necrosis and a few mitotic figures (2/50 high power field) could be found in tumor. The epithelioid tumor cells were diffusely positive for HMB-45, Melan-A, and focally positive for calponin. One of enlarged mesenteric lymph nodes was observed to be involved by tumor. A diagnosis of malignant mesenteric PEComa with lymph node involvement was made. The patient received chemotherapy after total resection of tumor and segmental resection of involved jejunum. There was no sign of recurrence of tumor found in period of 6-month regular follow-up after chemotherapy. To our knowledge, this is the first case of malignant PEComa in mesentery accompanied with regional lymph node involvement. The literature on this rare tumor is reviewed and diagnostic criteria of malignant PEComa are discussed.

Keywords:
Perivascular epithelioid cell tumor; Malignant PEComa; Mesentery; Lymph node involvement; Differential diagnosis