A Rare Case Presentation of Endometrial Clear-Cell Carcinoma

Background: Endometrial clear-cell carcinoma (ECCC) is a rare and particularly aggressive form of endometrial cancer that accounts for only 1%-6% of all cases. ECCC is more commonly found in older African American women. This report highlights the importance of tissue sampling in the atypical patient. Case: A 31-year-old Caucasian female presented with heavy menses and had an abnormal Papanicolaou smear. Cervical cytology and histology specimens were obtained. Results: A timely evaluation was performed. Endometrial sampling was found to be remarkable for ECCC. She had exploratory laparotomy, total abdominal hysterectomy and bilateral salpingooophorectomy, and pelvic and para-aortic lymph-node dissection Final staging was reported as Stage 1A grade 3 ECCC. She received 6 cycles of carboplatin/taxol. A follow-up computed tomography scan 6 months later of her chest, abdomen, and pelvis showed no evidence of residual or recurrent disease. Conclusions: ECCC is a rare, aggressive endometrial cancer. There are no existing screening tools for ECCC. Disease presentation is not always typical. Thorough evaluation of patients with menstrual irregularities associated with abnormal Papanicolaou smears may prevent delayed diagnosis of malignancy in the atypical patient.