Historical Considerations

Most endocrine tumors are solitary, occurring in single organs without concomitant disease in other endocrine tissues. The first known report of neoplasia arising in multiple endocrine organs in the same individual appeared in 1903, when Erdheim described an acromegalic patient noted on autopsy to have tumors of both the pituitary and parathyroid glands.1 Thereafter, only similar sporadic reports of multiple endocrine tumors appeared in the literature until 1954, when Wermer first described a familial clustering of anterior pituitary, parathyroid, and pancreatic islet cell neoplasia, which he termed endocrine adenomatosis? This report, which described the pattern of endocrine tumors now known as MEN 1, established the existence of heritable endocrine tumor syndromes.

The presence of other related multiple endocrine adenomatoses became apparent over the next several years. In 1959, medullary thyroid carcinoma was defined as a histopathologically distinct tumor (characterized, unlike other thyroid tumors, by the presence of stromal amyloid) by Hazard and associates.3 Subsequent studies in the 1960s demonstrated the cell of origin of this tumor to be the thyroid parafollicular C cell, the source of calcitonin. In 1961, Sipple4 described an association between thyroid carcinoma and pheochromocytoma, a concordance further defined 4 years later when Williams5 and Schimke and colleagues6 noted that the involved thyroid tumor was medullary carcinoma. In 1968, Steiner and coworkers7 coined the term MEN 2, to describe the familial association of medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia. The authors also suggested that the syndrome described by Wermer be termed MEN 1.

In 1966, Williams and Pollack reported two patients with concurrent medullary thyroid carcinoma, pheochromocytomas, and mucosal neuromas of the mouth and eyes, describing an apparent variant of von Recklinghausen's disease.8 In 1968, both Gorlin,9 Schimke,10 and their colleagues subsequently defined this pattern as a distinct clinicopathologic syndrome of endocrine disease, and in a 1975 review of medullary carcinoma of the thyroid, Chong and coworkers11 applied the term MEN 2B to the syndrome, which emphasized both the similarities and differences between this and the other more common MEN syndromes.

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