Ovarian clear cell carcinoma comprising dual components of differential alpha-fetoprotein productivity and drug sensitivity

Abstract

Epithelial ovarian tumor with elevated alpha-fetoprotein (AFP) is extremely rare and eventually needs differential diagnosis. This study investigated AFP-producing ovarian pure clear cell carcinoma without any components differentiating it to yolk sac tumor or hepatoid carcinoma. A 51-year-old Japanese woman presented with lower-abdominal distension continued for 6 months and was found to have a huge lower-abdominal mass on pelvic examination and MRI scan. Laboratory tests showed serum AFP 31533 ng/ml, CA125 456 U/ml, and LDH 485 IU/l. Bilateral salpingo-oophorectomy, total hysterectomy and omentectomy were performed with <1 cm residual macroscopic tumor. Microscopically, the tumor was characterized by typical clear cell carcinoma without any differentiating components. Pathologic stage was pT3cNxM0. Tumor cells were diffusely immunoreactive for AFP with more prominent staining on cell block cytology from ascites than surgical specimen. The patient was treated by 6 cycles of chemotherapy composed of dose-dense paclitaxel with intraperitoneal carboplatin, producing normalization of both AFP and CA125 without any evidence of the disease. After 11 months, she showed remarkable increase in CA125 to 1365 u/ml, with normal level of AFP. The recurrent tumor was resected and she received chemotherapy with gemcitabine and carboplatin, producing stabilization of the disease without any fluctuations in CA125 level. Eight months after, she showed slight increase of AFP to 29 ng/ml with uncontrollable massive ascites which was out of control resulting in her death.