Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature

BACKGROUND: Neoplasms of the perivascular epithelioid cell, first described in 1992, comprise a family of tumors known as "PEComas." To the best of our knowledge, this is the third case of uterine PEComa reported in the literature to behave in a malignant fashion. The purpose of this report is to provide information that may be used to predict the future behavior of this tumor. CASE: The patient was a 79-year-old woman with a large uterine mass which recurred 2 years following resection. The patient died within months after resection of the recurrent tumor. Retrospective immunohistochemical staining with newly commercially available antibodies including MART-1 was done on both the original and the recurrent tumor, confirming the diagnosis of uterine PEComa. CONCLUSION: Uterine PEComas should be regarded as tumors with uncertain malignant potential.