. Author manuscript; available in PMC: 2020 Nov 30.

Published in final edited form as: Surg Oncol Clin N Am. 2020 Apr;29(2):145–163. doi: 10.1016/j.soc.2019.11.002

Table 2.

Major neuroendocrine tumors of the GEP system and their associations with multiple endocrine neoplasias

Peptide/Amine	Syndrome/Symptom	Apudoma/NET	MEN1 or MEN2^a
Gastrin	Zollinger-Ellison diarrhea, atypical Gl ulcers	Gastrinoma	1
Insulinoma	Hypoglycemia, symptomatic	Insulinoma	1
VIP	Watery diarrhea syndrome	VIPoma	1 (rare)
Glucagon	Dermatosis, diabetes, thromboembolism	Glucagonoma	1 (rare)
Somatostatin	Diabetes, fat malabsorption, cholelithiasis	Somatostatinoma	1 (rare) 2 (very rare)
Pancreatic polypeptide	Asymptomatic	PP-oma	1
Calcitonin	Asymptomatic/diarrhea, flushing	Medullary thyroid carcinoma	2
Norepinephrine/epinephrine	Hypertension, tachycardia, pallor	Pheochromocytoma	2
Serotonin, substance P, neurotensin	Flushing, diarrhea, cool perspiration	Carcinoid	1 (lung carcinoid) 2 (ileal carcinoid)

MEN1, autosomal-dominant; gene resides on chromosome 11q13; 610 aa nuclear protein “MENIN” Tumor Suppressor. MEN2, autosomal-dominant, gene on chromosome 10q; a receptor tyrosine kinase protooncogene^6,59.

Modified from O’Dorisio TM, Vinik Al. Pancreatic polypeptide in missed hormone-producing tumors of the gastrointestinal tract. In Cohen S, Soloway RD (Eds) Contemporary issues in gastroenterology. Edinburgh: Churchill-Livingston 1984;5:117–128; with permission.