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Multiple Endocrine Neoplasia (MEN) Syndromes

Anup Sabharwal, MD, MBA, FACE Reviewed 06/2019
 


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

1
Falchetti A. Genetics of multiple endocrine neoplasia type 1 syndrome: what’s new and what’s old. F1000Res.  2017;6:F1000 Faculty Rev-73. [View Abstract on OvidMedline]
2
Wells SAJr, As...

ADDITIONAL READING

  • Carney JA. Familial multiple endocrine neoplasia: the first 100 years. Am J Surg Pathol.  2005;29(2):254–274. [View Abstract on OvidMedline]

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

SEE ALSO

Gastric Cancer; Hyperparathyroidism; Insulinoma; Marfan Syndrome; Pheochromocytoma; Thyroid Malignant Neoplasia 

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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