A case of sclerosing epithelioid fibrosarcoma arising in the thigh of a 40-year-old man is reported. This recently described sarcoma has a distinctive histological appearance which may cause confusion with a variety of other sarcomas with an epithelioid pattern, and indeed with benign soft tissue tumours showing hyalinization. Despite its bland cytology, sclerosing epithelioid fibrosarcoma is capable of metastases, often several years after surgical removal. The patient described here developed lung metastases five years after excision and remains alive a further three years later. The differential diagnosis and possible histogenesis are discussed.

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.

No abstract