Abstract: Background and purpose: Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare gynecological malignancy with poor prognosis, which mostly occurs in young women. This study analyzed clinical characteristics and treatment of 11 patients with SCCOHT, then clinical manifestations, treatment patterns and prognostic factors were summarized through literature review. Methods: A retrospective study was conducted on clinical data of 11 cases diagnosed with SCCOHT from Jan. 2000 to Dec. 2019 in Fudan University Shanghai Cancer Center. Results: The median age of 11 patients was 31 years (from 22 to 40 years). The main clinical presentations were abdominal pain (63.7%) and pelvic mass (36.4%). According to 2019 International Federation of Gynecology and Obstetrics (FIGO) staging system for ovarian cancer, stage Ⅰ included 4 cases, stage Ⅲ consisted of 1 case and stage Ⅳ had 6 cases. Four (36.4%) patients had elevated serum calcium. All patients were administered with surgery followed by adjuvant chemotherapy. Nine (81.8%) cases died within 1 year from initial diagnosis, and the median survival time was 6 months. In this cohort, 6-month survival rate was 45.5%, and 10-month survival rate was 13.6%. Conclusion: SCCOHT occurs in younger patients and is difficult to deal with due to its significantly aggressive behavior. Surgery combined with adjuvant chemotherapy is first-line treatment strategy. Pelvis and abdomen are the most common recurrence sites. Targeted therapy and immunotherapy are promising for SCCOHT.

Key words: Ovary small cell carcinoma, Hypercalcemic type, Clinical manifestations, Treatment, Prognosis