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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Table 3b.

Differential Diagnosis of Multiple Endocrine Neoplasia Type 4-Associated Clinical Features

Clinical FeatureComments
Pituitary tumorsIf multiple pituitary adenomas: see Table 3a
Zollinger-Ellison syndrome (ZES)Gastrinomas may also be present in MEN1, TSC, & NF1.
Nonfunctioning neuroendocrine tumorsMay also be present in MEN1 (20%-55%), VHL (10%-17%), & NF1
Carcinoid tumorsWhen not assoc w/MEN4: usually occur in derivatives of the midgut & hindgut & secrete serotonin (5-hydroxytryptamine)

MEN1 = multiple endocrine neoplasia type 1; MEN4 = multiple endocrine neoplasia type 4; NF1 = neurofibromatosis 1; TSC = tuberous sclerosis complex; VHL = von Hippel-Lindau syndrome

From: Multiple Endocrine Neoplasia Type 4

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