To evaluate the clinicopathologic characteristics of primary clear cell carcinoma of the ovary.

From February, 1993 to February, 2003, thirty patients with clear cell carcinoma of the ovary were identified, who were treated in the department of obstetrics and gynecology, Samsung Cheil Hospital and Women's Healthcare Center. The clinicopatholgic characteristics of the patients were retrospectively evaluated for age, chief complaint, preoperative CA-125 level, FIGO stage, maximal tumor diameter, association with endometriosis, management, and survival.

Clear cell ovarian carcinoma accounts for 5.2% of all cases of ovarian cancer at this center. Mean age was 44.6 years (26-62 years). Most patients presented with a large pelvic mass ranging from 7 to 30 cm in maximal diameter and seventeen (71%) of twenty-four patients had elevated serum level of preoperative CA-125. Associated pelvic endometriosis was 60% (18/30). Tumors were 73.3% (22/30) stage I, 10% (3/30) stage II, 13.3% (4/30) stage III and 3.3% (1/30) stage IV. Except for two patients who wanted to preserve fertility, all patients underwent total hysterectomy and bilateral salpingo-oophorectomy, omentectomy, peritoneal washing cytology and with or without pelvic and paraaortic lymph node dissection. All patient except one stage Ia received postoperative chemotherapy. With a median follow-up of 38.7 months (3-116 months), 96% (24/25) of stage I/II patients are alive, while 40% (2/5) of stage III/IV patients are alive.

Clear cell carcinoma of the ovary demonstrates a characteristic clinical behavior. Several factors may be associated with the survival rate in the ovarian clear cell carcinoma that are FIGO stage, associated pelvic endometriosis, spontaneous capsular rupture and presence of ascites.