The clinical presentation of MEN4 is similar to that of MEN1. Primarily occurring are parathyroid adenomas with PHPT and neuroendocrine tumors of adenohypophysis (PitNET), which may be somatotrophic, corticotrophic, or non-functional. In addition, renal angiomyolipomas, bronchial carcinoids, and gastroenteropancreatic neuroendocrine tumors have been described, along with tumors of the uterus, adrenal glands, and thyroid.
The earliest initial manifestation was a case of acromegaly in a 30-year-old patient.
Medullary thyroid carcinomas and pheochromocytomas, and tumors from the spectrum of MEN2 do not occur with MEN4.