Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.

Angiosarcoma is a rare vascular soft tissue tumor of endothelial origin most commonly seen in the elderly as a primary cutaneous head and neck malignancy. Furthermore, a peritoneal angiosarcoma is an exceedingly rare entity. This is the second case of primary peritoneal angiosarcoma reported in literature that is not associated with prior radiotherapy. Herein, we describe a case of primary peritoneal angiosarcoma metastatic to both the liver and bone in a male patient with metachronous renal cell carcinoma and parathyroid adenoma.

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