Familial Medullary Carcinoma Of The Thyroid

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Abstract
Familial medullary carcinoma of the thyroid occurring as a dominant auto-somal trait is recorded in 2 families. Familial carcinoma of the thyroid is usually, and possibly invariably, medullary with usually an amyloid stroma. It is considered as 1 manifestation of the medullary thyroid carcinoma-pheochromocytoma syndrome. Other categories of the syndrome include familial and non-familial coexistence of these 2 lesions, and familial pheochromocytomas. Other clinical features of the syndrome include: frequent bilateral occurrence of both the thyroid carcinoma and the pheochromocytoma; occasional coexistence of parathyroid tumors, neurofibromas, diabetes mellitus, and diarrhea. Although the medullary thyroid carcinoma-pheochromocytoma syndrome and the Zollinger-Ellison variety of multiple endocrine adenomatosis have certain similarities, including alterations in amine metabolism, each is considered to be a distinct genetic syndrome. The frequent bilateral involvement of the thyroid and metastases to cervical nodes justifies total thyroidectomy and lateral neck dissection for medullary carcinoma of the thyroid.