Multiple Endocrine Neoplasia (MEN) Syndromes

Anup K. Sabharwal, MD, MBA, FACE Reviewed 06/2018

BASICS

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant disorders that predispose individuals to the development of neoplasms, usually benign but sometimes malignant, in characteris...

DIAGNOSIS

MEN1: Clinical diagnosis of sporadic MEN1 is made in patients with tumors in two of the three main MEN1-associated endocrine glands; familial MEN1 is diagnosed in individuals who have one of...

TREATMENT

In general, treatment recommendations align with those of the specific isolated tumor.

SURGERY/OTHER PROCEDURES

Surgical management varies and depends on the specific tumor affected. For exam...

ONGOING CARE

DIET

Calcium and vitamin D should be limited in patients with hypercalcemia secondary to hyperparathyroidism until surgical intervention is complete. Patients with gastrinoma should avoid ...

REFERENCES

Falchetti A. Genetics of multiple endocrine neoplasia type 1 syndrome: what's new and what's old. F1000Res. 2017;6. [View Abstract on OvidMedline]

Marini F, Falchetti A, Del Monte F,...

ADDITIONAL READING

Carney JA. Familial multiple endocrine neoplasia: the first 100 years. Am J Surg Pathol. 2005;29(2):254–274.

CODES

ICD10

E31.20 Multiple endocrine neoplasia [MEN] syndrome, unspecified
E31.21 Multiple endocrine neoplasia [MEN] type I
E31.22 Multiple endocrine neoplasia [MEN] type IIA
E31.23 Multiple endocrine ne...

CLINICAL PEARLS

Young people (<30 years of age) with hypercalcemia: Think MEN1.
Multiglandular involvement in hyperparathyroidism: Think MEN1.
Adrenal mass and tachycardia: Think pheochromocytoma.
A p...

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