Abstract
A liposarcoma of the perineal body was diagnosed in a 38-year-old woman. She presented with a hard lump in the perineal region of 2 months duration. Light microscopic examination revealed a poorly differentiated malignant neoplasm of undetermined type. Electron microscopy indicated that the tumor cells contained large irregular nuclei, prominent nucleoli, abundant rough endoplasmic reticulum, and variable amounts of lipid material, consistent with a pleomorphic liposarcoma. She was treated with posterior exenteration, including resection of the internal reproductive organs, anus, and rectum, perineum and vulva, and posterolateral vagina, leaving the anterior vagina in place. Eight months later, a nodule noted in the vagina was widely excised and found to represent recurrent liposarcoma. She was started on adjuvant chemotherapy at that time. Subsequently, there has been no evidence of additional tumor in the brief follow-up period to date. Liposarcomas generally occur in regions with large connective tissue spaces such as the retroperitoneal area, shoulder, and lower extremities. In reviewing the literature, we found only one case report of liposarcoma of the vulva and no previous cases of liposarcoma of the perineum.