Types

1. Adrenal cancer
2. Carcinoid tumors
3. Merkel cell carcinoma
4. Pancreatic neuroendocrine tumors
5. Paraganglioma
6. Pheochromocytoma

Genetic syndromes
Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/neuroendocrine-tumors/symptoms-causes/syc-20354132
When should genetic testing be performed in patients with neuroendocrine tumours?
doi: 10.1007/s11154-017-9430-3
1. Multiple endocrine neoplasia, type 1 (MEN 1)
2. Multiple endocrine neoplasia, type 2 (MEN 2)
3. Von Hippel-Lindau disease
4. Tuberous sclerosis
5. Neurofibromatosis

Hereditary Syndromes and Abdominal Neuroendocrine Tumors - https://doi.org/10.1007/978-88-470-3955-

1_3
In recent decades, several hereditary diseases predisposing to abdominal neuroendocrine tumors (NETs)
have been identified, and clinically and genetically characterized:
1. multiple endocrine neoplasias type 1 (MEN1),
2. multiple endocrine neoplasias type 4 (MEN4),
3. von Hippel-Lindau syndrome (VHL),
4. neurofibromatosis 1 (NF1; von Recklinghausen’s disease),
5. tuberous sclerosis (TSC; Bourneville disease), and
6. Mahvash disease (MD).
Pheochromocytoma and Paraganglioma syndromes http://dx.doi.org/10.1016/j.hoc.2015.09.006
Functional neuroendocrine tumor syndromes
● https://doi.org/10.1080/14656566.2016.1236916- Treatment of symptomatic neuroendocrine tumor
syndromes: recent advances and controversies

Functional neuroendocrine tumor (F-NET) syndromes can be classified into those generally associated with
pNETs and those generally associated with GI-NETs (carcinoids).
There are 10 well-established pNET syndromes of which 9 are associated with a functional syndrome (F-
pNETs) and one is not associated with a specific clinical syndrome (nonfunctional-pNET, NF-pNET).
● NF-pNETs, in the strict sense, are not nonfunctional, in that they usually secrete a number of
different products (CgA, neuron-specific enolase, pancreatic polypeptide, ghrelin, neurotensin, etc.);
however, these do not result in a specific clinical syndrome [1,2,30].
● The nine F-pNETs include
○ gastrinomas-releasing gastrin to cause ZES,
○ insulinomas,
○ VIPomas secreting vasoactive intestine peptide,
○ glucagonomas,
○ SSomas,
○ GRFomas secreting growth hormone-releasing factor, ACTHomas,
○ pNETs secreting serotonin causing carcinoid syndrome, and
○ pNETs causing hypercalcemia by secreting PTH-RP [1,2].
● In addition to these well-established pNET syndromes, there are number of other very rare pNETs
with functional syndromes (1–5 cases) which include pNETs secreting luteinizing hormone, renin,
GLP-1, IGF-2, erythropoietin, enteroglucagon, and cholecystokinin [1,31], which will not be further
discussed because there are too few cases studied.