PARAGANGLIOMATOSIS ASSOCIATED WITH MULTIPLE ENDOCRINE ADENOMAS

  • 1 January 1976
    • journal article
    • research article
    • Vol. 100  (9) , 495-498
Abstract
A 19 yr old woman had multiple functioning extraadrenal 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 extraadrenal paragangliomas appears to be unique. Thyroid parafollicular cell proliferation could not be proved by immunohistochemical or EM 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 an endocrine polypeptide (APUD [amine precursor uptake and decarboxylation]) cell system may offer an explanation for the interrelation of these diverse growths.