Further Inpatient Care
- Role of adjuvant therapy
- Thyroid hormone therapy and radiotherapy are not as effective as surgical treatment for medullary thyroid carcinoma (MTC).
- Positive surgical margins or mediastinal extension may be an indication for adjuvant radiotherapy.
- A palliative benefit in controlling symptoms from bony metastases may be derived from external beam radiotherapy.
Further Outpatient Care
- Measure calcitonin and carcinoembryonic antigen (CEA) levels after thyroidectomy. Patients with undetectable calcitonin — or, in patients with sporadic MTC who have undergone hemithyroidectomy, calcitonin levels within the normal reference range — should have follow-up testing every 6-12 months. Detectable levels after total thyroidectomy, or above-normal levels after hemithyroidectomy, mandate further assessment with imaging studies, as per American Thyroid Association guidelines.
- I f calcitonin becomes detectable after total thyroidectomy but imaging studies do not identify disease or if calcitonin levels rise after hemithyroidectomy, doubling time of calcitonin and CEA levels may be used to assess tumor progression.[1] In one study, 94% of patients with doubling times shorter than 25 months had progressive disease and 86% of patients with doubling times longer than 24 months had stable disease.[15]
- Perform reoperative cervical exploration for isolated recurrent cervical disease (without distant metastases) as demonstrated by ultrasonography or CT scanning.
- Identification of distant metastatic disease may depend on laparoscopy with probe ultrasonography to detect liver surface lesions and bone scanning to detect osseous disease.
- Selective hepatic venous sampling for liver metastases is an experimental procedure that is used to detect intrahepatic lesions with greater sensitivity.
- If metastatic workup findings are negative in the presence of elevated plasma calcitonin levels, elective cervical lymph node dissection or modified radial neck dissection may be performed.
Deterrence/Prevention
Management guidelines from the American Thyroid Association (ATA) recommend prophylactic thyroidectomy for individuals with documented RET mutation who are at risk for aggressive medullary thyroid carcinoma.[1] The ATA has proposed schedules for the recommended age of RET testing, first ultrasound, serum calcitonin level, and prophylactic surgery, depending on the level of risk; in those at highest risk, surgery is recommended within the first year of life.
Complications
- Permanent hypoparathyroidism and recurrent laryngeal nerve palsy reportedly occur in less than 2% of virgin neck dissections; however, reoperation is associated with a considerably higher risk of these injuries.
Prognosis
- Prognosis depends on patient age, histologic grade, and status of surgical resection.
- Patients with a worse prognosis tend to be older, have higher-grade lesions, and have undergone incomplete surgical resection of the lesion.
- A study by Rohmer et al concluded that disease-free survival (DFS) in younger patients (>21 y) with hereditary MTC was best predicted by TNM staging and preoperative basal CT level of less than 30 pg/mL.[16] Basal CT findings, class D genotype, and age were the key factors in deciding peroperatively timely surgery.
Patient Education
- For excellent patient education resources, visit eMedicine's Endocrine System Center. Also, see eMedicine's patient education articles Thyroid Problems and Anatomy of the Endocrine System.
[Guideline] Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, Gharib H, et al. Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid. Jun 2009;19(6):565-612. [Medline]. [Full Text].
Hundahl SA, Cady B, Cunningham MP, Mazzaferri E, McKee RF, Rosai J, et al. Initial results from a prospective cohort study of 5583 cases of thyroid carcinoma treated in the united states during 1996. U.S. and German Thyroid Cancer Study Group. An American College of Surgeons Commission on Cancer Patient Care Evaluation study. Cancer. Jul 1 2000;89(1):202-17. [Medline].
Kebebew E, Greenspan FS, Clark OH, et al. Extent of disease and practice patterns for medullary thyroid cancer. J Am Coll Surg. Jun 2005;200(6):890-6. [Medline].
Costante G, Meringolo D, Durante C, Bianchi D, Nocera M, Tumino S, et al. Predictive value of serum calcitonin levels for preoperative diagnosis of medullary thyroid carcinoma in a cohort of 5817 consecutive patients with thyroid nodules. J Clin Endocrinol Metab. Feb 2007;92(2):450-5. [Medline]. [Full Text].
Ahmed SR, Ball DW. Clinical review: Incidentally discovered medullary thyroid cancer: diagnostic strategies and treatment. J Clin Endocrinol Metab. May 2011;96(5):1237-45. [Medline]. [Full Text].
Machens A, Lorenz K, Dralle H. Individualization of lymph node dissection in RET (rearranged during transfection) carriers at risk for medullary thyroid cancer: value of pretherapeutic calcitonin levels. Ann Surg. Aug 2009;250(2):305-10. [Medline].
Chang TC, Wu SL, Hsiao YL. Medullary thyroid carcinoma: pitfalls in diagnosis by fine needle aspiration cytology and relationship of cytomorphology to RET proto-oncogene mutations. Acta Cytol. Sep-Oct 2005;49(5):477-82. [Medline].
Boostrom SY, Grant CS, Thompson GB, Farley DR, Richards ML, Hoskin TL, et al. Need for a revised staging consensus in medullary thyroid carcinoma. Arch Surg. Jul 2009;144(7):663-9. [Medline].
Ye L, Santarpia L, Gagel RF. Targeted Therapy for Endocrine Cancer: The Medullary Thyroid Carcinoma Paradigm. Endocr Pract. Jun 22 2009;1-24. [Medline].
Ducic Y, Oxford L. Transcervical elective superior mediastinal dissection for thyroid carcinoma. Am J Otolaryngol. Jul-Aug 2009;30(4):221-4. [Medline].
Ahmed SR, Ball DW. Incidentally Discovered Medullary Thyroid Cancer: Diagnostic Strategies and Treatment. J Clin Endocrinol Metab. Feb 23 2011;[Medline].
Sherman SI. Advances in chemotherapy of differentiated epithelial and medullary thyroid cancers. J Clin Endocrinol Metab. May 2009;94(5):1493-9. [Medline].
Schlumberger MJ, Elisei R, Bastholt L, Wirth LJ, Martins RG, Locati LD, et al. Phase II study of safety and efficacy of motesanib in patients with progressive or symptomatic, advanced or metastatic medullary thyroid cancer. J Clin Oncol. Aug 10 2009;27(23):3794-801. [Medline].
Wells SA, Robinson RF, Gagel H, Dralle JA, Fagin M, Santoro E, et al. Vandetanib (VAN) in locally advanced or metastatic medullary thyroid cancer (MTC): A randomized, double-bind phase III trial (ZETA). J Clin Oncol. 2010;28:(suppl: abstr 5503. [Full Text].
Laure Giraudet A, Al Ghulzan A, Aupérin A, Leboulleux S, Chehboun A, Troalen F, et al. Progression of medullary thyroid carcinoma: assessment with calcitonin and carcinoembryonic antigen doubling times. Eur J Endocrinol. Feb 2008;158(2):239-46. [Medline]. [Full Text].
Rohmer V, Vidal-Trecan G, Bourdelot A, et al. Prognostic factors of disease-free survival after thyroidectomy in 170 young patients with a RET germline mutation: a multicenter study of the Groupe Francais d'Etude des Tumeurs Endocrines. J Clin Endocrinol Metab. Mar 2011;96(3):E509-18. [Medline].
Carlomagno F, Santoro M. Identification of RET kinase inhibitors as potential new treatment for sporadic and inherited thyroid cancer. J Chemother. Nov 2004;16 Suppl 4:49-51. [Medline].
Chi DD, Moley JF. Medullary thyroid carcinoma: genetic advances, treatment recommendations, and the approach to the patient with persistent hypercalcitoninemia. Surg Oncol Clin N Am. Oct 1998;7(4):681-706. [Medline].
Evans DB, Fleming JB, Lee JE, et al. The surgical treatment of medullary thyroid carcinoma. Semin Surg Oncol. 1999;16:50-63. [Medline].
Fitze G. Management of patients with hereditary medullary thyroid carcinoma. Eur J Pediatr Surg. Dec 2004;14(6):375-83. [Medline].
Gibelin H, Essique D, Jones C, et al. Increased calcitonin level in thyroid nodules without medullary carcinoma. Br J Surg. May 2005;92(5):574-8. [Medline].
Hyer SL, Newbold K, Harmer C. Familial medullary thyroid cancer: clinical aspects and prognosis. Eur J Surg Oncol. May 2005;31(4):415-9. [Medline].
Quayle FJ, Moley JF. Medullary thyroid carcinoma: including MEN 2A and MEN 2B syndromes. J Surg Oncol. Mar 1 2005;89(3):122-9. [Medline].
Rosenthal MS, Pierce HH. Inherited medullary thyroid cancer and the duty to warn: revisiting Pate v. Threlkel in light of HIPAA. Thyroid. Feb 2005;15(2):140-5. [Medline].
Shaha AR. Management of the neck in thyroid cancer. Otolaryngol Clin North Am. 1998;31:823-31.
Udelsman R, Lakatos E, Ladenson P. Optimal surgery for papillary thyroid carcinoma. World J Surg. 1996;20:88-93. [Medline].
You YN, Lakhani V, Wells SA Jr, Moley JF. Medullary thyroid cancer. Surg Oncol Clin N Am. Jul 2006;15(3):639-60. [Medline].

