Paragangliomatosis associated with multiple endocrine adenomas

A 19-year-old woman had multiple functioning extra-adrenal paragangliomas, a pituitary adenoma associated with acromegaly, parathyroid hyperplasia, and pigmentary abnormalities. This case differs from previously described instances of multiple endocrine adenomatosis (MEA) and has features that bridge the classic MEA type 1 and 2 syndromes and possibly Von Recklinghausen disease. The coexistence of pheochromocytoma with acromegaly is extremely rare, and the association with extra-adrenal paragangiliomas appears to be unique. Thyroid parafollicular cell proliferation could not be proved by immunohistochemical or electron microscopical studies. The large number and extensive distribution of paragangliomas, ranging from neck to pelvis, is another unique feature of this case. The concept of neurocrestopathy or of an endocrine polypeptide (APUD) cell system may offer an explanation for the interrelation of these diverse growths.