Multiple Endocrine Neoplasia

1) What is it?

- Subgroup of multiple endocrines hyper functions (adenoma or hyperplasia) from mechanisms other than a primary abnormality in the hypothalamic-pituitary axis.

2) Types of MEN

a) MEN 1 (Wermer Syndrome) -4P’

- Parathyroid hyperplasia

- Pituitary adenoma

- Pancreatic adenoma

- With/out PUD (H. Pylori associated Gastritis)

b) MEN 2A (Sipple syndrome)

- Medullary thyroid CA

- Pheochromocytoma

- Parathyroid hyperplasia

c) MEN 2B (mucosal neuroma syndrome)

- Medullary thyroid CA

- Pheochromocytoma

- Mucosal ganglioneuromatosis

- Marfanoid habitus

3) Pathophysiology

- MEN 2A , MEN 2B and familial MTC are autosomal dominant due to germ-line mutation in the RET proto-oncogene located in chromosomes 10 , band q11.2 which codes for a receptor-like tyrosine kinase.

- This mutation convert RET into a dominant transforming gene with oncogenic activity.

Abdominal CT Scan

Clinical impression:
1) Multiple calcified nodules involving both lobes of the liver.
2) Hypodense lesion at right adrenal gland 3 x 3 cm in diameter.


Image taken from the lecture note:

"A 21-year-old man with headache, flushing and palpitation following a thyroidectomy"
Dr Norlela Sukor, MD (UKM)
Dr Norazmi Kamaruddin, MRCP (UK)
Department of Medicine
Universiti Kebangsaan Malaysia