Primary ovarian malignant melanoma: A systematic review of cases and pooled analysis

Primary ovarian malignant melanoma is a rare malignancy arising from malignant transformation of a mature cystic teratoma of the ovary. Evidence is limited to case reports and case series. This systematic review and pooled analysis summarise clinical characteristics, management strategies and outcomes of this rare disease.

Systematic searches of Medline (PubMed), EMBASE, Scopus and Web of Science were conducted from inception to September 2025 identifying all case reports and case series reporting primary ovarian malignant melanoma. Data extraction and risk of bias assessments were performed in duplicate. Risk of bias was assessed using the Joanna Briggs Institute tool. Survival associations were analysed using Kaplan-Meier methods and Cox proportional hazards regression.

Seventy-six studies reporting 92 patients were included. Mean age at diagnosis was 50.6 years (range 11-86). Abdominal distension (34/73, 46.6%) and pain (32/73, 44.4%) were the commonest presenting symptoms. Almost half were diagnosed International Federation of Gynaecology and Obstetrics stage I (34/70, 48.6%); no cases of stage II; 30% (21/70) were stage III and 15/70 (21.4%) stage IV. Surgery was performed in 78/79 (98.7%) patients. Adjuvant therapies included chemotherapy (35/63, 55.6%), immunotherapy (17/63, 27.1%), radiotherapy (9/63, 14.3%), and targeted therapy (3/63, 4.8%), including imatinib, vemurafenib and aflibercept regimes. Median overall survival declined with advancing stage (p-value <0.01); 62% of patients died (37/60), with 57.8% doing so within 12 months of diagnosis (21/37). In an unadjusted analysis, immunotherapy appeared associated with improved overall survival (Hazard Ratio [HR] = 0.38, 95% CI: 0.17-0.86, p-value = 0.02). This was not significant when restricted to stage 1 disease.

Primary ovarian malignant melanoma is associated with poor prognosis across all disease stages. Our findings are descriptive, limited by the rarity of the disease and quality of existing evidence. International collaborative studies, rare cancer registries, and molecular profiling are essential to improve understanding and management.