Autosomal dominant transmission. Tumor or hyperplasia of at least two endocrine glands.

Types:

-        MEN I (Wermer syndrome) [MIM 131 100]: caused by the inactivation of the MEN1 gene (11q13); pituitary gland, pancreas and parathyroid. Sometimes bronchial or digestive carcinoid, angiofibromas. Very rarely symptomatic before adulthood. Sometimes diarrhea by pancreatic secretion of VIP (Vasoactive Intestinal Peptide), gastrin or calcitonin

-        MEN II: caused by a mutation of the RET gene; medullary carcinoma of the thyroid, pheochromocytoma, parathyroid glands;

       3 variants:

       1) MEN IIa: [MIM 171400] no physical abnormalities; increased risk of Hirschsprung disease, hyperparathyroidism or cutaneous amyloidosis (amyloid dermal deposits; pruriginous hyperkeratosic papulas, often pigmented on the trunk and the extremities, mostly on the tibias).

       2) MEN IIb [MIM 162300] (Sipple syndrome): mutation of the RET proto-oncogen (ch10), presence of small ganglioneuromas in the lips and mucosae,  thick bumpy lips, marfanoid habitus, diarrhea or constipation, sometimes pubertal delay. Early detection of thyroid cancer by measuring calcitonin blood levels.
3) MEN II familial [MIM 155 240]: familial thyroid medullary carcinoma

Anesthetic implications:

preoperative endocrine checkup.

References : 

-        Dubey RK, Verma N, Pandey CK. 
Anaesthetic management of a dopamine-secreting phaechromocytoma in multiple endocrine neoplasia 2B syndrome. 
Indian J Anaesth 2014; 58: 217-9. 

-        Andrade S, Sirchia F, Faleschini E, Barbi E.
A girl with delayed puberty and bumpy lips.
J Pediatr 2018; 203: 454-5.

-        Allahyar AE, Liang X, Vollers JM, de Armendi AJ.
Anesthesia management for pediatric patient with multiple endocrine neoplasia type 2B: a case report.
A&A Practice 2020 ;14 :e01215

Updated: May 2020