A neoplastic disease, characterized by a triad of medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia or adenoma, in association with elevated calcitonin and catecholamine levels. Other neoplasms found in this syndrome may include glioblastomas and meningiomas. It is sometimes termed Multiple Endocrine Neoplasia syndrome (MEN 2A) to distinguish it from Wermer syndrome (MEN1) and MEN 2B. The latter is the same syndrome but without parathyroid involvement. It is inherited as an autosomal dominant trait with complete penetrance and variable expressivity,
- A. S. Eisenberg, H. Wallerstein:
Pheochromocytoma of the suprarenal medulla (paraganglioma): Clinicopathological study.
Archives of Pathology, Chicago, 1932, 14: 818-836.
- E. C. Beer, F. M. King, M. Prinzmetal:
Pheochromocytoma with demonstration of pressor (adrenalin) substances in the blood preoperatively during hypertensive crises.
Annals of Surgery, Philadelphia, 1937, 106: 85-91.
- J. H. Sipple:
The association of pheochromocytoma with carcinoma of the thyroid gland.
American Journal of Medicine, New York, 1961, 31: 163-166.
This was Sipple's only paper on an endocrinological topic.