Reed Nicholas S
Beatson Oncology Centre, Gartnavel General Hospital, Glasgow, Scotland, UK.
Curr Opin Oncol. 2016 Sep;28(5):412-8. doi: 10.1097/CCO.0000000000000321.
The purpose of this article will be to review neuroendocrine tumours (NETs) of the gynaecological tract. NETs are generally considered to be uncommon and primary tumours in the gynaecological tract on arrival rare or very rare. It is important to distinguish true primary tumours from metastatic as the management is very different. These tumours ranged from highly aggressive small cell carcinomas (SCCs) sometimes associated with secretion of neuropeptides that may be functional and cause distinctive syndromes. On the other hand, the well differentiated low-grade NETs generally behave in a very indolent manner with an excellent prognosis. Very recent new data have identified a molecular marker for small cell carcinomas of the ovary although as yet there is no associated candidate drug.
Recent developments include the identification of a molecular marker for small cell carcinomas of the ovary of hypercalcaemic type; this will help to identify these tumours more confidently. New therapeutic developments for well differentiated NETs include mammalian target of rapamycin pathway inhibitors and peptide receptor radionuclide therapy.
In summary, these are rare tumours with a low incidence although higher prevalence. They require a multidisciplinary approach to management and should be supervised by specialist teams familiar with NETs.
本文旨在综述生殖道神经内分泌肿瘤（NETs）。NETs通常被认为不常见，而原发于生殖道的肿瘤更是罕见或极其罕见。区分真正的原发性肿瘤和转移性肿瘤很重要，因为二者的治疗方法截然不同。这些肿瘤包括高度侵袭性的小细胞癌（SCCs），有时与神经肽分泌有关，可能具有功能性并导致独特的综合征。另一方面，分化良好的低级别NETs通常行为惰性，预后良好。最近有新数据确定了卵巢小细胞癌的一种分子标志物，不过目前尚无相关候选药物。
最近的进展包括确定了高钙血症型卵巢小细胞癌的一种分子标志物；这将有助于更准确地识别这些肿瘤。分化良好的NETs的新治疗进展包括雷帕霉素靶蛋白通路抑制剂和肽受体放射性核素治疗。
总之，这些是罕见肿瘤，发病率低但患病率较高。它们需要多学科管理方法，应由熟悉NETs的专业团队进行监管。
