MEN syndrome Mnemonics

MEN syndrome is an autosomal dominant (AD) predisposition to developing multiple endocrine tumors.

Points to remember:

1. Parathyroid hyperplasia is common to Men I and Men IIa syndrome.
2. Medullary carcinoma of thyroid is common to MEN IIa and MEN IIb syndrome.
3. MEN I occurs due to mutation in Menin I gene located on chromosome 11q.
4. MEN II occurs due to mutation in Menin II gene located on choromsome 10q.
5. MEN IV (similar to MEN I) occurs due to mutation in CDKN1B located on chromosome 12p.

MEN I or MEN 1 (Wermer’s syndrome)

Mnemonic: 3 Ps

1. Parathyroid hyperplasia
2. Pancreatic islet cell tumor (Zollinger-Ellison syndrome in 50% and Insulinoma in 20%)
3. Pituitary tumors

MEN II or MEN 2

Mnemonic: 2 Cs

Associated mutation: RET proto-oncogene

1. Catecholamine releasing tumor: Pheochromocytoma
2. Calcitonin releasing tumor: Medullary carcinoma of thyroid

Component specific to MEN IIA and MEN IIB:

1. MEN IIA or 2A (Sipple’s syndrome): Parathyroid hyperplasia (hyperparathyroidism)
2. MEN IIB or 2B (also called MEN III): Mucosal neuromas and Marfanoid habitus

MEN IV or MEN 4

MEN IV occurs due to mutation in CDKN1B.

Men IV syndrome is clinically indistinguishable from MEN I syndrome. In addition, these may have:

1. Reproductive organ tumors (e.g. testicular tumors, neuroendocrine cervical carcinoma)
2. Adrenal + Renal tumors

Reference:

Endocrinology: Adult and Pediatric By J. Larry Jameson, Leslie J. De Groot