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MEDULLARY CARCINOMA

Age: >50 ys. but may occur in younger age groups with other endocrinal tumours e.g. Sipple syndrome MEN type IIa (Multiple Endocrinal Neoplasms)
ؤ Medullary carcinoma
ؤ Pheochromocytoma: operated 1st as it is danger.
ؤ Parathyroid adenoma
It may run in families as autosomal dominant. If associated with multiple neuromas in the lips, tongue and inner aspect of eye lids with Marfanoid habitus then it is called MEN type IIb.
Pathology:
ؤ MAC: Hard nodular mass.
ؤ MIC: The tumour originates from the parafollicular C-cells in the form of sheets of pleomorphic cells containing amyloid
ؤ Spread: Early by blood and lymphatics.
ؤThe tumour produces:
Calcitonine: good tumour marker for follow up after resection.
5HT or prostaglandine which is responsible for diarrhea in 30% of cases.
Treatment:
It has no uptake of I131 and it is radio resistant
Total thyroidectomy with block dissection of the neck is the only line but the prognosis is still poor.
SOURCE: Prof. AYMAN SALEM'S BOOK
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Not to be reproduced without permission of Vascular Society of Egypt

[Inapawnvag]

Q. An 11-year-old girl presents to your office because of a family history of medullary carcinoma of the thyroid. Physical examination is normal. Which of the following tests would you perform?
a- Urine vanillylmandelic acid VMA level
b- Serum insulin level
c- Serum Gastrin level
d- Serum glucagon level
e- Serum somatostatin level