Primary ovarian small-cell carcinoma of pulmonary type: a single-center case series and structured review of published cases

Abstract

Abstract Background Small-cell carcinoma of the ovary, pulmonary type (SCCOPT), is a rare, high-grade neuroendocrine carcinoma with no established standard treatment. We described seven patients treated at our centre and systematically reviewed published patient-level evidence. Methods We retrospectively reviewed seven patients treated between August 2015 and January 2025. PubMed, China National Knowledge Infrastructure, SpringerLink, and Wiley Online Library were searched from inception to 2 August 2026. Eligible reports provided patient-level diagnostic, treatment, follow-up, or outcome data. Published and institutional cases were combined for descriptive, Kaplan–Meier, and exploratory Cox analyses. Results Forty-two studies contributed 76 non-duplicated published patients. With seven institutional patients, the pooled cohort comprised 83 patients. The median age was 55 years (range, 14–85), and 57 of 78 patients with reported stage (73.1%) had FIGO stage III–IV disease. Taxane–platinum and etoposide–platinum exposure were reported in 32 (38.6%) and 24 (28.9%) patients, respectively. CD56, synaptophysin, and chromogranin A were positive in 46/47 (97.9%), 53/59 (89.8%), and 47/66 (71.2%) tested patients. Overall survival data were available for 66 patients; 42 deaths occurred, and the median overall survival was 14.0 months. The estimated 12-, 24-, and 36-month overall survival rates were 61.9%, 33.6%, and 30.2%. Conclusions SCCOPT has an aggressive clinical course and a variable immunophenotype. Surgery and platinum-based chemotherapy remain the main treatment approaches, but current evidence does not establish a preferred regimen. Multicentre registration, central pathological review, and standardised molecular and outcome reporting are needed.