Expressions of MEN1 with estimated penetrance (in parentheses) at age 40 years

Endocrine features	Nonendocrine features
Primary hyperparathyroidism (90%)	Lipomas (30%)
Entero-pancreatic tumor (30 to 70%)	Facial angiofibromas (85%)
Gastrinoma (30 to 40%)	Collagenomas (70%)
Insulinoma (10%)
NF* including pancreatic polypeptide (20 to 55%^¶)	Rare, maybe innate, endocrine or nonendocrine features
Other: glucagonoma, VIPoma, somatostatinoma, etc (2%)
Foregut carcinoid
Thymic carcinoid NF (2%)	Pheochromocytoma (<1%)
Bronchial carcinoid NF (2%)	Ependymoma (1%)
Gastric enterochromaffin-like tumor NF (10%)
Anterior pituitary tumor adenoma (30 to 40%)
Prolactinoma (20%)
Other: GH + PRL, GH, NF (each 5%)
ACTH (2%), TSH (rare)
Adrenal cortical tumor NF (40%)

MEN1: multiple endocrine neoplasia type 1; NF: nonfunctioning; GH: growth hormone; PRL: prolactin; ACTH: corticotropin; TSH: thyroid-stimulating hormone.
* May synthesize a peptide hormone or other factors (such as small amine) but does not usually oversecrete enough to produce a hormonal expression.
¶ Omits nearly 100% prevalence of NF and clinically silent tumors, some of which are detected incidental to pancreatico-duodenal surgery in MEN1.

Reproduced with permission from: Brandi ML, Gagel RF, Angeli A, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 2001; 86:5658. Copyright © 2001 The Endocrine Society.
Updated using:

Triponez F, Dosseh D, Goudet P, et al. Epidemiology data on 108 MEN 1 patients from the GTE with isolated nonfunctioning tumors of the pancreas. Ann Surg 2006; 243:265.
Kouvaraki MA, Shapiro SE, Cote GJ, et al. Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg 2006; 30:643.
Thakker RV, Newey PJ, Walls GV, et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 2012; 97:2990.

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Expressions of MEN1 with estimated penetrance (in parentheses) at age 40 years