Skip to Main Content

+

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

    • Arises from parafollicular or C cells

++

Epidemiology

+

  • • 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

++

Symptoms and Signs

+

  • • Hard thyroid nodule

    • Enlarged or hard cervical lymph nodes

    • Pain in the thyroid or paralaryngeal area

    • Hoarseness

    • Dyspnea

    • Stridor

    • Dysphagia

    • Diarrhea

++

Laboratory Findings

+

  • • Normal thyroid-stimulating hormone (TSH) levels

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

++

Imaging Findings

+

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

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

+

  • • 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)

++

Rule Out

+

  • • Multiple endocrine neoplasia type 2A or 2B

+

  • • 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

++

Surgery

++

Indications

+

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

++

Medications

+

  • • Postoperative thyroid hormone replacement

++

Treatment Monitoring

+

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

++

Complications

+

  • • Neck hematoma

    • Superior laryngeal nerve injury

    • Recurrent laryngeal nerve injury

    • Transient or permanent hypoparathyroidism

++

Prognosis

+

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

    • 10-year survival about 30-40%

++

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]

++

Practice Guidelines

+

  • • The National Comprehensive ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.