Ovarian Endometrioid Adenocarcinoma with Functioning Stroma Accompanied with Endometrial Endometrioid Adenocarcinoma: Immunohistochemical Study and Literature Review

Background: The ovarian tumors with functioning stroma are defined by the morphological presence of endocrine active cells in stroma, and the clinical, biochemical or pathological evidence of endocrine function. Case Report: The ovarian endometrioid adenocarcinoma with functioning stroma accompanied with endometrial endometrioid adenocarcinoma was found in 64-year-old post-menopausal woman complaining abnormal genital bleeding and mammary distention. Her preoperative serum 17ß-estradiol level was high (53.2 pg/ml) while human chorionic gonadotropin (hCG) level was within normal limit. Her right ovary with 8.8 × 5.3 cm in size and tan-yellow in color mostly consisted of solid tumor. Histologically, tumor was composed of estrogen receptor (ER)and progesterone receptor (PgR)-positive, and androgen receptor (AR)-negative cancerous endometrial cells with aggregates of vacuolated foamy stromal cells resembling luteinized cells. These stromal cells contained lipid droplets, and was immunopositive for α-inhibin and 17ß-estradiol. After surgery, serum 17ß-estradiol level decreased and became normal (14.2 pg/ml). These findings indicate the production of steroid hormone (17ß-estradiol) from the foamy stromal cells and may be correlated with the clinical symptoms. Furthermore, ERand PgR-positive endometrial endometrioid adenocarcinoma developed synchronously. However, ovary and uterus were totally immunonegative for human chorionic gonadotropin (hCG). Four other cases from the literature including ours are reviewed. Conclusion: Cancer cells were positive for ER and PgR in both ovary and uterus responded to steroid hormone produced by foamy stromal cells, which played a role in proliferation and progression of ovarian and endometrial endometrioid adenocarcinoma, respectively.