Overview and Pathophysiology of Neuroendocrine Neoplasms

Neuroendocrine neoplasms are increasing in incidence and prevalence. Recent epidemiological data suggests an overall incidence of 7 per 100 000 population in USA. Symptoms can be diverse with a number of tumours causing distinct clinical syndromes, most commonly carcinoid syndrome. Investigations to aid diagnosis include biochemical, cross-sectional, and functional imaging. Histology remains the gold standard for diagnosis. Treatment options are dependent in part of the site of the primary tumour. Surgery remains the only chance of cure and should be offered to all patients when appropriate. There are a number of systemic palliative treatment therapies and a number of local regional therapies that can be employed for liver metastases. For patients with carcinoid syndrome or functional tumours somatostatin analogues can be very effective a reducing hormone secretion. Median overall survival has been improving over the last 30 years to 9.3 years.