Para-aortic nodal metastasis of mesenteric perivascular epithelioid cell tumor (PEComa)

Perivascular epithelioid cell tumors (PEComas) are a group of tumours of mesenchymal origin having a characteristic pathological presence of the epitheloid cell around blood vessels. They are uncommon tumours and hence their exact etiology and pathogenesis remain unclear. They can occur at any part of the body but the common sites of involvement are the gastrointestinal system and the genitourinary system. The isolated involvement of the intestinal mesentery is very rare, with only a few cases reported in the literature till date. The involvement of lymph nodes by these tumours is exceptionally rare. We report a hitherto undescribed case of mesenteric PEComa in a young female who developed para-aortic nodal metastasis.