Clear cell carcinoma of the vagina and cervix

Clear cell carcinomas of the vagina and cervix are rare. The most well-established risk factor for these cancers is exposure to diethylstilbestrol (DES) in utero. However, after the first case series describing the association of DES with clear cell carcinomas was published in 1970, DES use significantly declined. Clear cell carcinomas of the vagina and cervix have also been associated with non-DES-related urinary tract abnormalities or Mullerian anomalies. For girls and women diagnosed with clear cell carcinomas of the vagina or cervix, the mean age at diagnosis is 22 years old, and most patients are diagnosed between the ages of 15 and 30. Five-year survival rates are slightly better for women with DES-related clear cell carcinomas of the vagina or cervix as compared with those who were not exposed to DES. The staging systems and diagnostic work-up mirrors cervical and vaginal cancers of other histologic subtypes. Early stage disease is often managed surgically, with or without adjuvant therapy. Locally advanced disease is often managed with chemoradiation, and metastatic disease frequently requires chemotherapy. Depending on the disease burden and location, recurrent disease may be managed surgically, or with chemotherapy and/or radiation therapy. Although there are few data evaluating the use of targeted therapies or immunotherapy agents in clear cell carcinomas of the vagina and cervix, these agents may offer some clinical efficacy and are worth considering in women with these cancer types.