Pleomorphic rhabdomyosarcoma of the uterus in a postmenopausal woman with elevated sevum CA125

Background. Pure rhabdomyosarcoma of the uterus is an exceedingly rare tumo r. While there are no commonly associated tumor markers, a previously repor ted case confirmed CA125 production by a pure uterine rhabdomyosarcoma. Case. We describe a postmenopausal woman who presented with vaginal bleedin g and was diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Of par ticular interest was an elevated serum CA125 level at presentation. Immunoh istochemical staining with CA125 antibody, however, showed no activity with in the tumor. Despite aggressive therapy, the patient experienced distant n odal metastases and succumbed to the disease within 25 months of diagnosis. Conclusion. Our patient illustrates the typical presentation and aggressive behavior of this rare neoplasm. While serum CA125 may be a useful tumor ma rker in selected patients, elevated levels do not directly reflect tumor bu rden when associated with negative immunohistochemical staining. (C) 1999 A cademic Press.