Clear Cell Carcinoma

Introduction

• Description: A clear cell carcinoma is an ovarian epithelial tumor comprising cells containing large amounts of glycogen, giving them a clear or “hobnailed” appearance. These tumors may also arise in the endocervix, endometrium, and vagina. Cervical and vaginal tumors have been linked to in utero exposure to diethylstilbestrol (DES).

• Prevalence: 5%–11% of ovarian cancers.

• Predominant Age: 40–78 years.

• Genetics: No genetic pattern. The AT-rich interactive domain 1A (ARID1A) may play a role as a tumor suppressor gene in ovarian clear cell carcinoma.

Etiology and Pathogenesis

• Causes: Unknown. May arise from mesonephric or Müllerian elements.

• Risk Factors: None known. Endometriosis has been postulated.

Signs and Symptoms

• Asymptomatic

• Pelvic mass (up to 30 cm)—partially cystic with yellow, gray, and hemorrhagic areas

• Papillary projections generally present, giving the mass a velvety appearance; 40% of tumors are bilateral

Diagnostic Approach