Primary large cell neuroendocrine carcinoma of the ovary: A rare entity.

BACKGROUND: Large-cell neuroendocrine carcinoma (LCNEC) of the ovary is rare, highly aggressive tumor and diagnosed at advanced stages. Immunohistochemistry is required for the diagnosis. The optimal treatment management is not codified because of its rarity. CASE PRESENTATION: We report the case of a 36-year-old woman with a locally advanced stage LCNEC of the ovary managed by surgery and adjuvant chemotherapy with etoposide and cisplatin. She remained disease free until now four years after the end of chemotherapy. CONCLUSION: This report suggests the necessity of immunohistochemical analysis in the diagnosis of LCNEC of the ovary. Due to the rarity of LCNC, the general consensus on treatment is not standardized. We used adjuvant chemotherapy regimen similar to large cell carcinoma of the lung.