Large cell neuroendocrine carcinoma of the ovary

Large cell neuroendocrine carcinoma of the ovary is a recently described tumour entity that is now included in the WHO classification of primary ovarian neoplasms. Although mostly in stage I at diagnosis, this tumour shows an aggressive clinical behaviour with subsequent metastases and mean survival is less than one year. In addition to the neuroendocrine carcinoma, most cases also have a malignant surface epithelial tumour component. I here report a 64-year-old woman who was operated on for a right-sided ovarian large cell neuroendocrine carcinoma without a surface epithelial component, which constitutes only the second reported tumour of this "pure" kind. Histological and immunohistochemical findings are described and a review of the literature is presented. The patient was treated postoperatively with chemotherapy. She developed bleomycin-induced lung fibrosis that responded well to treatment with steroids. There have been no signs of local tumour recurrence or metastases at follow-up examinations during the first 9 months after the operation.