Clear cell carcinoma of the female genital tract (not everything is as clear as it seems)

Adv Anat Pathol. 2012 Sep;19(5):296-312. doi: 10.1097/PAP.0b013e31826663b1.


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.

Publication types

  • Review

MeSH terms

  • Adenocarcinoma, Clear Cell / diagnosis*
  • Adenocarcinoma, Clear Cell / genetics
  • Adenocarcinoma, Clear Cell / metabolism
  • Carcinoma, Endometrioid / diagnosis
  • Carcinoma, Renal Cell / diagnosis
  • Carcinoma, Renal Cell / secondary
  • Cystadenocarcinoma, Serous / diagnosis
  • DNA, Neoplasm / analysis
  • Diagnosis, Differential
  • Diagnostic Errors
  • Dysgerminoma / diagnosis
  • Endodermal Sinus Tumor / diagnosis
  • Female
  • Genital Neoplasms, Female / diagnosis*
  • Genital Neoplasms, Female / genetics
  • Genital Neoplasms, Female / metabolism
  • Granulosa Cell Tumor / diagnosis
  • Humans
  • Molecular Diagnostic Techniques
  • Mutation / genetics
  • Pathology, Molecular / methods
  • Sex Cord-Gonadal Stromal Tumors / diagnosis


  • DNA, Neoplasm

Supplementary concepts

  • Granulosa cell tumor of the ovary