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.
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