Uterine Clear Cell Carcinoma

Uterine clear cell carcinoma (UCCC) is an uncommon variant of carcinoma of the uterus that represents no more than 5% of all endometrial carcinomas. The etiology of UCCC is not well understood but appears to be unique from endometrioid histology, and the suggestion by some recent studies that clear cell endometrial cancer may have a premalignant lesion deserves more studies. Comprehensive surgical staging is critical in order to plan appropriate postoperative management. Patients with UCCC often experience relapse in the pelvis, in para-aortic nodes, and at distant sites, whereas they do not seem to have a high propensity to fail in the abdomen. Pelvic radiotherapy might reduce the risk of pelvic recurrence. In stage III or IV UCCC and in women with recurrent disease, adjuvant chemotherapy with platinum-based chemotherapy, paclitaxel, and doxorubicin either in a doublet or triplet combination has demonstrated efficacy. The triplet combination is more toxic.