Table 2.

Endocrine Tumor Types in MEN1 Syndrome

Tumor TypeTumor SubtypesHormone SecretingPrevalence in MEN1 Syndrome
ParathyroidNAYesPrimary hyperparathyroidism in 100% by age 50 yrs 1
Anterior pituitaryProlactinomaYesAnterior pituitary tumors in ~30%-40% 2Most commonly seen anterior pituitary tumor subtype, accounting for 60% of pituitary tumors 3
GH-secretingYesAccounts for 25% of anterior pituitary tumors 3
GH/PRL-secretingYes5% 3
TSH-secretingYesRare 4
ACTH-secretingYes<5% 3
Non-functioningNo<5% 3
Well-differentiated endocrineGastrinomaYesAccounts for 40% of well-differentiated endocrine tumors 5
InsulinomaYes10% 3
GlucagonomaYes<1% 3
VIPomaYes<1% 3
Non-functioning & PPomaNo20%-55% 3 of gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs)
CarcinoidBrochopulmonaryNo2% 3
ThymicNo2% 3
GastricNo10% 3
AdrenocorticalCortisol-secretingRarely40% 3
Aldosterone-secretingRarely
PheochromocytomaRarely<1% 3

NA = not applicable

GH = growth hormone

PRL = prolactin

TSH = thyroid-stimulating hormone

ACTH = adrenocorticotrophic hormone

VIPoma = vasoactive intestinal peptide-secreting tumor

PPOMA = pancreatic polypeptide-secreting tumor

1.

First clinical manifestation of MEN1 in 90% of individuals

2.

First clinical manifestation of MEN1 in 10% of familial cases and 25% of simplex cases

3.
4.
5.

Manifest as Zollinger-Ellison syndrome (ZES)

From: Multiple Endocrine Neoplasia Type 1

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