Uterine papillary serous carcinoma: epidemiology, pathogenesis and management.

PURPOSE OF REVIEW: Uterine papillary serous carcinoma (UPSC) is a rare but aggressive subtype of endometrial cancer. Although it represents only 10% of all endometrial cancer cases, UPSC accounts for up to 40% of all endometrial cancer-related recurrences and subsequent deaths. The present article reviews the literature concerning the epidemiology, molecular pathogenesis and recent updates on management of UPSC. RECENT FINDINGS: Women most often present with postmenopausal vaginal bleeding but may also be diagnosed by vaginal cytology. In women diagnosed with metastatic disease, ascites, omental implants or a pelvic mass may be present. Pelvic and extrapelvic recurrences occur frequently, with extrapelvic relapses being observed most commonly. Although few prospective trials exist, several retrospective series have demonstrated that optimal cytoreduction and adjuvant platinum/taxane-based chemotherapy with or without radiotherapy appears to improve survival. In addition, another approach to UPSC management may lie in targeted therapy. SUMMARY: Women diagnosed with UPSC should undergo comprehensive surgical staging and an attempt at optimal cytoreduction. Platinum/taxane-based adjuvant chemotherapy should be considered in the treatment of both early and advanced-stage patients. Careful long-term surveillance is indicated as many of these women will recur. Prospective studies are needed to define the optimal treatment regimens and to study the role of targeted therapies in UPSC.