Making Sense of a Complex Disease

A Practical Approach to Managing Neuroendocrine Tumors

Janie Y. Zhang, MD; Pamela L. Kunz, MD


J Oncol Pract. 2022;18(4):258-264. 

In This Article


Several inherited genetic syndromes are associated with the development of NETs, including MEN1, MEN2, Von Hippel-Lindau syndrome, tuberous sclerosis complex, and neurofibromatosis type 1.[9] The National Comprehensive Cancer Network (NCCN) Guidelines for Neuroendocrine and Adrenal Tumors provides a framework for genetic risk assessment and counseling for patients with hereditary endocrine neoplasias. This includes evaluation in patients with gastrinomas, multifocal pNETs, a clinical suspicion of MEN1, and consideration in duodenal or pNET of any age.[9]

Certain somatic genetic alterations have been noted in NETs, although generally, mutation burden is low.[10] Somatic tumor profiling is not part of the NCCN NET treatment algorithm, although this may be considered in advanced disease to search for a targetable mutation.