Skip to main content

Advertisement

SpringerLink
Log in

Medullary thyroid cancer

  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Patients with clinically evident medullary thyroid cancer should have a total extracapsular thyroidectomy with bilateral central neck dissection and an ipsilateral prophylactic or therapeutic modified (functional) radical neck dissection when the primary tumor is greater than 1 cm and when the central neck nodes are positive. A prophylactic contralateral neck dissection should be done when the primary tumor is bilateral and when there is extensive lymphadenopathy on the side of the primary tumor. Patients who have gross, unresectable residual medullary thyroid cancer should receive postoperative external radiotherapy.

Patients who are carriers of germ-line RET proto-oncogene point mutations or have an elevated (basal or stimulated) calcitonin levels on screening should have a prophylactic total thyroidectomy before age 6 years. In patients with an elevated basal or stimulated plasma calcitonin level and an intrathyroidal nodule on ultrasound, a total thyroidectomy and central neck lymph node dissection should be done.

Patients with persistent or recurrent medullary thyroid cancer should have a complete thyroidectomy (if not done initially) and bilateral central and modified radical neck dissection, including upper mediastinal lymphadenectomy.

Patients who are symptomatic from distant medullary thyroid cancer metastases (diarrhea, flushing, weight loss, or bone pain) should be treated with somatostatin analogs. Bone metastases should be resected if possible, and symptomatic lesions that are unresectable should be treated with external radiotherapy. Cytoreductive procedures such as radiofrequency ablation or cryoablation for liver metastases should be considered in symptomatic patients to reduce tumor burden. Localized pulmonary metastases should be resected. Chemotherapy or radioactive immunotherapy (iodine 131 labeled carcinoembryonic antigen monoclonal antibody) protocols should be considered in patients with nonoperative widely metastatic progressing medullary thyroid cancer.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Hazard JB, Hawk WA, Crile GJ: Medullary (solid) carcinoma of the thyroid—a clinicopathologic entity. J Clin Endocrinol Metab 1959, 19:152–161.

    PubMed  CAS  Google Scholar 

  2. Gilliland FD, Hunt WC, Morris DM, Key CR: Prognostic factors for thyroid carcinoma. A population-based study of 15,698 cases from the surveillance, epidemiology and end results (SEER) program 1973–1991. Cancer 1997, 79:564–573.

    Article  PubMed  CAS  Google Scholar 

  3. Hundahl SA, Fleming ID, Fremgen AM, Menck HR: A national cancer data base report on 53,856 cases of thyroid carcinoma treated in the U.S., 1985-1995. Cancer 1998, 83:2638–2648.

    Article  PubMed  CAS  Google Scholar 

  4. Kebebew E, Ituarte PHJ, Siperstein AE, et al.: Medullary thyroid cancer: clinical characteristics, treatment, prognostic factors and a comparison of staging systems. Cancer 2000, 88:1139–1148. A univariate and multivariate analysis study of prognostic factors for medullary thyroid cancer and treatment outcome of patients with medullary thyroid cancer.

    Article  PubMed  CAS  Google Scholar 

  5. Ponder BA, Ponder MA, Coffey R, et al.: Risk estimation and screening in families of patients with medullary thyroid carcinoma. Lancet 1988, 1:397–401.

    Article  PubMed  CAS  Google Scholar 

  6. Kebebew E, Treseler PA, Siperstein AE, et al.: Normal thyroid pathology in patients undergoing thyroidectomy for finding a RET gene germline mutation: a report of three cases and review of the literature. Thyroid 1999, 9:127–131.

    Article  PubMed  CAS  Google Scholar 

  7. Kebebew E, Kikuchi S, Duh Q-Y, Clark OH: Long-term results of reoperation and localizing studies in patients with persistent or recurrent medullary thyroid cancer. Arch Surg 2000, in press.

  8. Moley FJ, DeBenedetti MK: Patterns of nodal metastases in palpable medullary thyroid carcinoma. Recommendations for extent of node dissection. Ann Surg 1999, 229:880–888. This study emphasizes the rationale for at least an ipsilateral modified radical neck dissection and bilateral central neck node dissection in patients with clinically evident medullary thyroid cancer at the time of initial thyroidectomy.

    Article  PubMed  CAS  Google Scholar 

  9. Moley JF: Surgical management of persistent and recurrent medullary thyroid cancer. J Intern Med 1998, 243:251–256.

    Article  Google Scholar 

  10. Saad MF, Guido JJ, Samaan NA: Radioactive iodine in the treatment of medullary carcinoma of the thyroid. J Clin Endocrinol Metab 1983, 57:124–128.

    Article  PubMed  CAS  Google Scholar 

  11. Treseler PA, Clark OH: Prognostic factors in thyroid carcinoma. Surg Oncol Clin North Am 1997, 6:555–598.

    CAS  Google Scholar 

  12. Duh QY, Sancho JJ, Greenspan FS, et al.: Medullary thyroid carcinoma. The need for early diagnosis and total thyroidectomy. Arch Surg 1989, 124:1206–1210.

    PubMed  CAS  Google Scholar 

  13. Dralle H, Gimm O, Simon D, et al.: Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience. World J Surg 1998, 22:744–751. A German and Austrian cooperative retrospective study in 75 patients who underwent prophylactic surgical treatment for hereditary medullary thyroid cancer. Addresses the need for prophylactic regional node dissection at the time prophylactic thyroidectomy in patients with elevated calcitonin.

    Article  PubMed  CAS  Google Scholar 

  14. Brierley JD, Tsan RW: External radiation therapy in the treatment of thyroid malignancy. Endocrinol Metab Clin North Am 1996, 25:141–157.

    Article  PubMed  CAS  Google Scholar 

  15. Simpson WJ, Palmer JA, Rosen IB, Mustard RA: Management of medullary carcinoma of the thyroid. Am J Surg 1982, 144:420–422.

    Article  PubMed  CAS  Google Scholar 

  16. Samaan NA, Schultz PN, Hickey RC: Medullary thyroid carcinoma: prognosis of familial versus sporadic disease and the role of radiotherapy. J Clin Endocrinol Metab 1988, 67:801–805.

    PubMed  CAS  Google Scholar 

  17. Schlumberger M, Gardet P, de Vathaire F, et al.: External radiotherapy and chemotherapy in MTC patients. In Medullary Thyroid Carcinoma. Edited by Calmettes C, Guliana J. Paris: John Libbey, 1991:213–220.

    Google Scholar 

  18. Fife KM, Bower M, Harmer CL: Medullary thyroid cancer: the role of radiotherapy in local control. Eur J Surg Oncol 1996, 22:588–591.

    Article  PubMed  CAS  Google Scholar 

  19. Brierley J, Tsan R, Simpson WJ, et al.: Medullary thyroid cancer: analyses of survival and prognostic factors and the role of radiation therapy in local control. Thyroid 1996, 6:305–310.

    PubMed  CAS  Google Scholar 

  20. Nguyen TD, Chassard JL, Lagarde P, et al.: Results of postoperative radiation therapy in medullary carcinoma of the thyroid: a retrospective study by the French Federation of Cancer Institutes—The Radiotherapy Cooperative Group. Radiother Oncol 1992, 23:1–5.

    Article  PubMed  CAS  Google Scholar 

  21. Mak A, Morrison W, Garden A, et al.: The value of postoperative radiotherapy for regional medullary carcinoma of the thyroid. Int J Radiat Oncol Biol Phys 1994, 30:234.

    Google Scholar 

  22. Van Heerdan J, Grant C, Gharib H, et al.: Long-term course of patients with persistent hypercalcitoninemia after apparent curative primary surgery for medullary thyroid carcinoma. Ann Surg 1990, 212:395–400.

    Article  Google Scholar 

  23. Harmer C, Bidmead M, Shepherd S, et al.: Radiotherapy planning techniques for thyroid cancer. Br J Radiol 1998, 71:1069–1075.

    PubMed  CAS  Google Scholar 

  24. Petursson SR: Metastatic medullary thyroid carcinoma. Complete response to combination chemotherapy with dacarbazine and 5-fluorouracil. Cancer 1988, 62:1899–1903.

    Article  PubMed  CAS  Google Scholar 

  25. Orlandi F, Caraci P, Berruti A, et al.: Chemotherapy with dacarbazine and 5-fluorouracil in advanced medullary thyroid cancer. Ann Oncol 1994, 5:763–765.

    PubMed  CAS  Google Scholar 

  26. Wu LT, Averbuch SD, Ball DW, et al.: Treatment of advanced medullary thyroid carcinoma with a combination of cyclophosphamide, vincristine, and dacarbazine. Cancer 1994, 73:432–436.

    Article  PubMed  CAS  Google Scholar 

  27. Di Bartolomeo M, Bajetta E, Bohcicchio AM, et al.: A phase II trial of dacarbazine, fluorouracil, and epirubicin in patients with neuroendocrine tumours. A study by the Italian Trials in Medical Oncology (I.T.M.O.) Group. Ann Oncol 1995, 6:77–79.

    PubMed  Google Scholar 

  28. Schlumberger M, Abdelmoumene N, Delisle MJ, et al.: Treatment of advanced medullary thyroid cancer with an alternating combination of 5 FU-streptozocin and 5 FU-dacarbazine. Br J Cancer 1995, 71:363–365.

    PubMed  CAS  Google Scholar 

  29. Shimaoka K, Schoenfeld DA, DeWys D, et al.: A randomized trial of doxorubicin versus doxorubicin plus cisplatin in patients with advance thyroid carcinoma. Cancer 1985, 56:2155–2160.

    Article  PubMed  CAS  Google Scholar 

  30. Sridhar KS, Holland JF, Brown JC, et al.: Doxorubicin plus cisplatin in the treatment of apudomas. Cancer 1985, 55:2634–2637.

    Article  PubMed  CAS  Google Scholar 

  31. Gottlieb JA, Hill CS: Adriamycin (NSC-123127) therapy in thyroid carcinoma. Cancer Chemother Rep 1975, 6:283–296.

    Google Scholar 

  32. Schlumberger M, Gardet P, de Vathaire F, et al.: Chemotherapy in metastatic nonanaplastic thyroid cancer: experience at the Institut Gustave-Roussy. Tumori 1990, 76:480–483.

    PubMed  Google Scholar 

  33. Mahler C, Verhelst J, De Longueville M, Harris A: Longterm treatment of metastatic medullary thyroid carcinoma with the somatostatin analogue octreotide. Clin Endocrinol 1990, 33:261–269.

    CAS  Google Scholar 

  34. Modigliani E, Cohen R, Joannidis S, et al.: Results of long-term continuous subcutaneous octreotide administration in 14 patients with medullary thyroid carcinoma. Clin Endocrinol 1992, 36:183–186.

    CAS  Google Scholar 

  35. Juweid ME, Hajjar G, Stein GH, et al.: Initial experience with high-dose radioimmunotherapy of metastatic medullary thyroid cancer using 131I-MN-14 F(ab)2 anti-carcinoembryonic antigen Mab and AHSCR. J Nucl Med 2000, 41:93–103.

    PubMed  CAS  Google Scholar 

  36. Kraeber-Bodere F, Bardet S, Hoefnagel CA, et al.: Radio-immunotherapy in medullary thyroid cancer using bispecific antibody and iodine 131-labeled bivalent hapten: preliminary results of a phase I/II clinical trial. Clin Cancer Res 1999, 5(suppl):3190–3198.

    Google Scholar 

  37. Juweid M, Sharkey RM, Behr T, et al.: Targeting and initial radioimmunotherapy of medullary thyroid carcinoma with 131I-labeled monoclonal antibodies to carcinoembryonic antigen. Cancer Res 1995, 55(suppl):5946–5951.

    Google Scholar 

  38. Behr TM, Sharkey RM, Jueweid ME, et al.: Phase I/II clinical radioimmunotherapy with an iodine-131-labeled anti-carcinoembryonic antigen murine mono-clonal antibody IgG. J Nucl Med 1997, 38:858–870.

    PubMed  CAS  Google Scholar 

  39. Machens A, Gimm O, Ukkat J, et al.: Improved predic-tion of calcitonin normalization in medullary thyroid carcinoma patients by quantitative lymph node analysis. Cancer 2000, 88:1909–1915. A quantitative study of extent of medullary thyroid cancer lymph node involvement in patients undergoing cervical lymph node clearance. Patients who have more than two cervical lymph node involvement or 10 or more positive nodes are unlikely to have normalization of plasma calcitonin levels even after compartment-oriented lymph node microdissection.

    Article  PubMed  CAS  Google Scholar 

  40. Tissell L, Hansson G, Jansson S, Salander H: Re-operation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma. Surgery 1986, 99:60–66.

    Google Scholar 

  41. Moley JF, Dilley W, DeBenedetti M: Improved results of cervical re-operation for medullary thyroid carcinoma. Ann Surg 1997, 225:734–743.

    Article  PubMed  CAS  Google Scholar 

  42. Dralle H, Damm I, Scheumann GF, et al.: Compartment-oriented microdissection of regional lymph nodes in medullary thyroid carcinoma. Surg Today 1994, 24:112–121.

    Article  PubMed  CAS  Google Scholar 

  43. Chen H, Roberts JR, Ball DW, et al.: Effective long-term palliation of symptomatic, incurable metastatic medullary thyroid cancer by operative resection. Ann Surg 1998, 227:887–895.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, University of California, San Francisco, UCSF/Mt. Zion Medical Center, S343, Box 0470, 94143, San Francisco, CA, USA

    Electron Kebebew MD & Orlo H. Clark MD

Authors
  1. Electron Kebebew MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Orlo H. Clark MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kebebew, E., Clark, O.H. Medullary thyroid cancer. Curr. Treat. Options in Oncol. 1, 359–367 (2000). https://doi.org/10.1007/s11864-000-0052-7

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11864-000-0052-7

Keywords

Search

Navigation