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Multiple endocrine neoplasia

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Multiple endocrine neoplasia
ICD-10 D448
ICD-9 258.0
OMIM [1]
DiseasesDB [2]
MedlinePlus [3]
eMedicine /
MeSH {{{MeshNumber}}}

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. [1]

TerminologyEdit

The older names "multiple endocrine adenomas", or "multiple endocrine adenomatosis" (MEA) have been replaced by the current terminology.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.[2]

Related conditionsEdit

Although not officially categorized as multiple endocrine neoplasia syndromes, Von Hippel-Lindau disease[3] and Carney complex[4] 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 syndrome characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.

ComparisonEdit

Feature MEN 1 MEN 2
MEN 2A MEN 2B FMTC
Eponym Wermer syndrome Sipple syndrome (see below) (none)
OMIM 131100 171400 162300 155240
Pancreatic tumors insulinoma, gastrinoma, vipoma - - -
Pituitary adenoma Yes - - -
Parathyroid hyperplasia Yes Yes - -
Medullary thyroid carcinoma - Yes 100% 100%
Pheochromocytoma - Yes 50% -
Marfanoid body habitus - - 80% -
Multiple Mucosal Neuromata - - >95% -
spontaneous mutation rate 50%
Gene(s) MEN1 (131100) RET (164761) RET (164761) RET (164761),
NTRK1 (191315)
Approx. prevalence 1 in 35,000 1 in 1,000,000
Initial description (year) 1954[5] 1961[6] 1965

(Blanks indicate that data are not yet available.)

MEN 2B is sometimes known as MEN 3 and the designation varies by institution (c.f. www.ClinicalReview.com). Although a variety of eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome), none ever gained sufficient traction to merit continued use and, indeed, are all but abandoned in the medical literature. Another early report was Schimke et al in 1968.[7]

OMIM also includes a fourth form of multiple endocrine neoplasia ("MEN4"), associated with CDKN1B.[8] The presentation is believed to overlap that of MEN1 and MEN2.[9]

ReferencesEdit

  1. Template:DorlandsDict
  2. Carney JA (Feb 2005). Familial multiple endocrine neoplasia: the first 100 years. Am. J. Surg. Pathol. 29 (2): 254–74.
  3. Carney JA (Jun 1998). Familial multiple endocrine neoplasia syndromes: components, classification, and nomenclature. J. Intern. Med. 243 (6): 425–32.
  4. Callender GG, Rich TA, Perrier ND (Aug 2008). Multiple endocrine neoplasia syndromes. Surg. Clin. North Am. 88 (4): 863–95.
  5. Wermer P (1954). Genetic aspects of adenomatosis of endocrine glands. Am. J. Med. 16 (3): 363–71.
  6. Sipple JH (1961). The association of pheochromocytoma with carcinoma of the thyroid gland. Am. J. Med. 31: 163-6.
  7. Schimke RN, Hartmann WH, Prout TE, Rimoin DL (1968). Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue. N. Engl. J. Med. 279 (1): 1–7.
  8. OMIM 610755
  9. Pellegata NS, Quintanilla-Martinez L, Siggelkow H, et al (Oct 2006). Germ-line mutations in p27Kip1 cause a multiple endocrine neoplasia syndrome in rats and humans. Proc. Natl. Acad. Sci. U.S.A. 103 (42): 15558–63.

External linksEdit

Template:Tumor morphology Template:Endocrine gland neoplasiaTemplate:ENwp

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