Table 2.

Endocrine Tumor Types in Multiple Endocrine Neoplasia Type 1

Tumor TypeTumor SubtypesHormone SecretingPrevalence in MEN1
ParathyroidNAYesPHPT in 100% by age 50 yrs
Anterior pituitaryProlactinomaYesAnterior pituitary tumors in ~30%-40%60%
GH-secretingYes25%
GH/PRL-secretingYes10% 1
TSH-secretingYesRare 2
ACTH-secretingYes<5% 1
NonfunctioningNo<10% 1
Well-differentiated endocrine of the GEP tractGastrinomaYes40%
InsulinomaYes10% 1
GlucagonomaYes<3% 1
VIPomaYes3%
SomatostatinomasYes<1%
NonfunctioningNo55% 1
CarcinoidThymicNo3%-8% 1
BronchialNo4.7%-6.6% 1
Type II gastric ECL carcinoidsNo10% 1
AdrenocorticalCortisol-secretingRarely, hypersecreting40% 1
Aldosterone-secretingRarely, hypersecreting
PheochromocytomaRarely<1% 1

ACTH = adrenocorticotrophic hormone; ECL = enterochromaffin-like; GEP = gastroenteropancreatic; GH = growth hormone; NA = not applicable; PHPT = primary hyperparathyroidism; PRL = prolactin; TSH = thyroid-stimulating hormone; VIPoma = vasoactive intestinal peptide-secreting tumor

1.
2.

From: Multiple Endocrine Neoplasia Type 1

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