Ovarian small cell carcinoma, hypercalcemic type exhibiting a response to high-dose chemotherapy.

A gravida 2, para 2 25-year-old woman three months post-partum presented to her primary physician with abdominal pain and bloating; a 20-cm complex cystic pelvic mass was identified by ultrasound. No ovarian masses were noted during ultrasound exam at the prior pregnancy, less than one year earlier. Her labs included hypercalcemia (11.8 mg/dL, normal less than 10.5) and an elevated CA 125 (160 U/mL, normal less than 35). An exploratory laparotomy revealed a 20-cm right ovarian mass. Frozen section was performed and a sex cord-stromal tumor was favored. Permanent sections of the specimen, however, revealed round, closely packed neoplastic cells with a high nuclear to cytoplasm ratio and high mitotic rate growing in a diffuse pattern with scattered follicle-like, ill-defined microcystic spaces. Immunohistochemical stains revealed the neoplasm to be focally positive for keratin and negative for inhibin. The final diagnosis rendered was small cell carcinoma of the ovary, hypercalcemic type. Further staging revealed para-aortic lymph node involvement (stage IIIC). Current literature suggests a very poor prognosis for these neoplasms despite aggressive therapy, with an overall survival rate of 10 percent. Rare response has been noted, however, with high-dose chemotherapy followed by autologous peripheral blood stem cell transplant. Our patient underwent a rigorous chemotherapeutic regimen followed by peripheral blood stem cell transplant, and as of August 2010, (17 months after initial diagnosis), the patient has had no recurrence.