Review ArticlesClear Cell Carcinoma of the Female Genital Tract (Not Everything Is as Clear as it Seems)Offman, Saul L. MD; Longacre, Teri A. MDAuthor Information Department of Pathology, Stanford University, Stanford, CA The authors have no funding or conflicts of interest to disclose. Reprints: Teri A. Longacre, MD, Department of Pathology, Room L235, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305 (e-mail: [email protected]). Advances In Anatomic Pathology: September 2012 - Volume 19 - Issue 5 - p 296-312 doi: 10.1097/PAP.0b013e31826663b1 Buy Metrics Abstract Clear cell carcinoma has a storied history in the female genital tract. From the initial designation of ovarian clear cell adenocarcinoma as “mesonephroma” to the linkage between vaginal clear cell carcinoma and diethylstilbestrol exposure in utero, gynecologic tract clear cell tumors have puzzled investigators, posed therapeutic dilemmas for oncologists, and otherwise presented major differential diagnostic challenges for pathologists. One of the most common errors in gynecologic pathology is misdiagnosis of clear cell carcinoma, on both frozen section and permanent section. Given the poor response to platinum-based chemotherapy for advanced-stage disease and increased risk of thromboembolism, accurate diagnosis of clear cell carcinoma is important in the female genital tract. This review (1) presents the clinical and pathologic features of female genital tract clear cell carcinomas; (2) highlights recent molecular developments; (3) identifies areas of potential diagnostic confusion; and (4) presents solutions for these diagnostic problems where they exist. © 2012 Lippincott Williams & Wilkins, Inc.