MEN syndrome : Mnemonics

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

Mnemonic: MEN are Dominant.

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 X P’s

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

MEN IIA or MEN 2A (Sipple’s syndrome)

Mnemonic: 3 X C’s

1. Catecholamine releasing tumor: Pheochromocytoma
2. Calcitonin releasing tumor: Medullary carcinoma of thyroid
3. Calcium increasing: Parathyroid hyperplasia (hyperparathyroidism)

MEN IIB or 2B (MEN III)

Mnemonic: 2C 2M

1. Catecholamine releasing tumor: Pheochromocytoma
2. Calcitonin releasing tumor: Medullary carcinoma of thyroid
3. Mucosal neuromas
4. Marfanoid habitus

MEN IV or MEN 4

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