Multiple endocrine neoplasia
| Multiple endocrine neoplasia | |
|---|---|
 |
|
| Micrograph of a medullary thyroid carcinoma, as may be seen in MEN 2A and MEN 2B. H&E stain. | |
| Classification and external resources | |
| Specialty | endocrinology |
| ICD-10 | E31.20 |
| ICD-9-CM | 258.0 |
| ICD-O | 8360/1 |
| MedlinePlus | 000398 000399 |
| MeSH | D009377 |
The term multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
MEN syndromes are inherited as autosomal dominant disorders.
The older names, "multiple endocrine adenomas" and "multiple endocrine adenomatosis" (MEA), have been replaced by the current terminology.
The term multiple endocrine neoplasia is used when two or more endocrine tumor types, known to occur as a part of one of the defined MEN syndromes, occurs in a single patient and there is evidence for either a causative mutation or hereditary transmission. The presence of two or more tumor types in a single patient does not automatically designate that individual as having MEN because there is a small statistical chance that development of two "sporadic" tumors that occur in one of the MEN syndromes could occur by chance.
The term "multiple endocrine neoplasia" was introduced in 1968, but descriptions of the condition date back to 1903.
Although not officially categorized as multiple endocrine neoplasia syndromes, Von Hippel-Lindau disease and Carney complex are two other autosomal dominant endocrine tumor syndromes with features that overlap the clinical features of the MEN syndromes. Although not transmitted in the germline, McCune-Albright syndrome is a genetic disorder characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.
In 1903 Erdheim described the case of an acromegalic patient with a pituitary adenoma and three enlarged parathyroid glands.
In 1953 Underdahl et al. reported a case series of 8 patients with a syndrome of pituitary, parathyroid, and pancreatic islet adenomas.
In 1954 Wermer noted that this syndrome was transmitted as a dominant trait.
In 1959 Hazard et al. described medullary (solid) thyroid carcinoma.
...
Wikipedia