• 5% of malignant tumors of the thyroid
• 35% of medullary thyroid cancer (MTC) is familial
• 65% of MTC is sporadic
• Peak incidence of familial MTC is second to third decade of life
• Peak incidence of sporadic MTC is fifth to sixth decade of life
• Tumors contain amyloid
• Not radioiodine sensitive (not follicular cell-derived)
• Growth not stimulated by TSH
• Spread is lymphatic
• Familial tumors are often multifocal and bilateral (vs unilateral in sporadic disease)
• Complete history and physical exam, with special consideration to family history and exam of the neck and cervical lymph nodes
• Fine-needle aspiration biopsy
• Serum calcitonin
• Screen for Ret mutations (10% of patients without a family history have de novo mutations)
• Cervical ultrasound to assess thyroid and regional lymph nodes
• Prophylactic total thyroidectomy before age 5 recommended for patients without symptoms and if detected by family genetic screening
• Surgical treatment represents the only chance for cure
• Nonresectable recurrence of MTC may respond to external beam radiation or multidrug chemotherapy regimen
• Neck hematoma
• Superior laryngeal nerve injury
• Recurrent laryngeal nerve injury
• Transient or permanent hypoparathyroidism
Kebebew E et al: Medullary thyroid carcinoma: clinical characteristics, treatment, prognostic factors, and a comparison of staging systems. Cancer
Moley JF, Fialkowski EA: Evidence-based approach to the management of sporadic medullary thyroid carcinoma. World J Surg
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