Large cell neuroendocrine carcinoma of the ovary

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Abstract
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.

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