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  • • Aggressive form of thyroid cancer that can be either sporadic or familial

    • Arises from parafollicular or C cells

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • Hard thyroid nodule

    • Enlarged or hard cervical lymph nodes

    • Pain in the thyroid or paralaryngeal area

    • Hoarseness

    • Dyspnea

    • Stridor

    • Dysphagia

    • Diarrhea

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Laboratory Findings

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  • • Normal thyroid-stimulating hormone (TSH) levels

    • Elevated calcitonin level (basal or calcium/pentagastrin stimulated)

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Imaging Findings

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  • US: Demonstrates extent of tumor and can detect enlarged lymph nodes

    • Plain films of neck may reveal irregular dense calcifications of the MTC lesion

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  • • 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)

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Rule Out

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  • • Multiple endocrine neoplasia type 2A or 2B

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  • • 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

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  • • 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

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Surgery

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Indications

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  • • All MTCs should be removed, with central neck dissection, as completely as possible

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Medications

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  • • Postoperative thyroid hormone replacement

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Treatment Monitoring

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  • • Calcitonin level and neck palpation every 3-6 months

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Complications

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  • • Neck hematoma

    • Superior laryngeal nerve injury

    • Recurrent laryngeal nerve injury

    • Transient or permanent hypoparathyroidism

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Prognosis

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  • • Outcome correlates directly with completeness of tumor resection and degree of extrathyroidal spread

    • 10-year survival about 30-40%

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References

Kebebew E et al: Medullary thyroid carcinoma: clinical characteristics, treatment, prognostic factors, and a comparison of staging systems. Cancer 2000;88:1139.  [PubMed: 10699905]
Moley JF, Fialkowski EA: Evidence-based approach to the management of sporadic medullary thyroid carcinoma. World J Surg 2007;31:946.  [PubMed: 17426901]

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Practice Guidelines

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  • • The National Comprehensive ...

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