Neuroendocrine tumours of the gynecological tract

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.