Clear-cell adenocarcinoma is a rare and aggressive form of cancer that typically arises in the female reproductive organs, particularly the ovaries and the endometrium as well as the kidneys and is characterized by the presence of clear,[1] glycogen-rich cells.

According to the WHO, these criteria include polygonal or hobnail or cells with clear or eosinophilic/oxyphilic cytoplasm and nuclear atypia, with different architectural patterns of growth, such as papillary, tubulocystic, or solid.

[3] Traditionally, clear cell carcinoma of the lower genital tract in females was related to diethylstilbestrol (DES) exposure in utero.

[5] Treatment of ovarian clear cell carcinoma typically begins with surgery to remove the ovaries and other affected organs.

A tumor debulking procedure is typically performed, consisting of a total hysterectomy and bilateral salpingo-oopherectomy.

Cytoreductive surgery, which involves removing all visible cancer cells during the operation, is the current standard of care.

[8] Clear cell adenocarcinoma of the vagina is a rare cancer that is related to DES exposure in utero.

Women who were exposed to DES in utero should be evaluated yearly with a pap smear and pelvic exam due to risk of developing clear cell adenocarcinoma.