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Multiple Endocrine Neoplasia (MEN) Syndromes

Anup Sabharwal, MD MBA FACC FACE FASPC FNLA FEAA Reviewed 05/2023
 


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. 

ISSUES FOR REFERRAL

If diagnosis remains in question, a referral to an endocrine center of excellence ma...

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 Ov...

SEE ALSO

Gastric Cancer; Hyperparathyroidism; Insulinoma; Marfan Syndrome;  (8) 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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