Endocrinology Made Simple : Multiple Endocrine Neoplasia (MEN) Syndrome

Multiple Endocrine Neoplasia (MEN) Syndrome:

Multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. 

In some cases, the tumors are malignant, in others, benign.

MEN syndromes are inherited as autosomal dominant disorders.

Types Of MEN Syndromes:

MEN II Syndrome

Multiple Endocrine Neoplasia II Syndrome  consists of medullary thyroid carcinoma with pheochromocytoma.

It has two different types:

MEN II A consists of medullary thyroid cancer,pheochromocytoma and hyperparathyroidism.

MEN II B consists ofmedullary thyroid carcinoma,pheochromocytoma,marfinoid features and mucosal and gastrointestinal neuromas.

C cell hyperplasia precedes the development of Medullary thyroid carcinoma.

Pheochromocytoma occurs in 50 % of patients with MEN II and causes episodic or sustained hypertension with diaphoresis and heaadaches.

Management :

Pheochromocytomas are treated with alpha blockade for 10 – 14 days before surgical resection is undertaken.

Alpha blockade is helpful for intravascular fluid restoration and is carried out along with increasing fluid intake and liberal salt intake.

After alpha blockade has been completed,treatment with beta blockers is started to further control the symptoms.

Medullary thyroid cancer is treated by total thyroidectomy with central neck dissection.

CT scan canbe done to determine spread of cancer and its staging.

Biphosphonates are given to reduce serum calcium in the patients.