| Summary: | <p>PURPOSE: Primary ovarian neuroendocrine tumours (NETs) develop in pure form or in association with other tumours, mainly teratomas. The available data regarding this rare condition are limited. Much of the literature focuses on single case reports, previously dismissed as a totally benign disorder, and thus long-term considerations and evidenced-based management guidelines are lacking. The objective of the current study was to describe the clinical and pathologic characteristics and the effect of various therapeutic modalities on patient morbidity and mortality from two major centres. A secondary objective was to highlight that carcinoid syndrome may manifest in the absence of metastatic disease.</p><p> METHODS: The authors retrospectively studied a cohort of 34 consecutive patients with primary ovarian NETs who attended either the Oxford University Hospitals or Beatson Oncology Centre, Glasgow, between 1984 -2014, and had their ovarian carcinoid data (e.g. tumour growth, chemotherapy regime) and mortality outcomes assessed. </p><p> RESULTS: All patients were women, average age was 53, range 23-87 years at diagnosis. Of the 34 patients, 8 patients (23.5%) presented with the carcinoid symptoms. Carcinoid heart disease was evident in 2 of the 34 patients (6%). All patients had a primary ovarian carcinoid tumour on histopathology and two patients had bilateral ovarian carcinoid. Teratoma associations could be assessed in 29 tumours, with 20/29 (69%) showing a pathological association. The mean tumour size was 48mm (range 4-120mm, SD 40mm). The mean follow up was 4.5 years, with up to 17 years. The disease has been quiescent with no evidence of relapse in 22 of 34 patients; in 12 of 34 patients it was metastatic. There were 5 patients who received treatment with somatostatin analogues or chemotherapy. 8 patients died of disease (23.5%). Metastatic disease was found up to 11 years from initial diagnosis. </p><p> CONCLUSIONS: Primary ovarian NET is rare and likely under-represented in the literature. It can no longer be simply dismissed as a generally benign entity. It requires careful evaluation and review.</p>
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