Skip to main content
SpringerLink
Log in

Practical Management of Thyroid Cancer: A Multidisciplinary Approach-Medullary Thyroid Cancer

  • Chapter
  • First Online:
Practical Management of Thyroid Cancer

  • 1230 Accesses

Abstract

Medullary thyroid cancer (MTC) is one of the less common histological types of thyroid malignancy, only accounting for 1–2% of thyroid cancers [1]. It was first recognised as an entity in 1906 by Jaquet and subsequently described in more definitive detail by Hazard and Crile [2]. In practical terms its biology differs from the more common papillary cancer and as such, requires a different therapeutic approach. This chapter describes the current understanding of the biology of this thyroid cancer type and practical considerations useful for the multi-disciplinary care of these patients.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Wells SA Jr, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF, et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid. 2015;25(6):567–610.

    Article  Google Scholar 

  2. Hazard JB, Hawk WA, Crile G Jr. Medullary (solid) carcinoma of the thyroid; a clinicopathologic entity. J Clin Endocrinol Metab. 1959;19(1):152–61.

    Article  CAS  Google Scholar 

  3. Donis-Keller H, Dou S, Chi D, Carlson KM, Toshima K, Lairmore TC, et al. Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet. 1993;2(7):851–6.

    Article  CAS  Google Scholar 

  4. Mulligan LM, Kwok JB, Healey CS, Elsdon MJ, Eng C, Gardner E, et al. Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature. 1993;363(6428):458–60.

    Article  CAS  Google Scholar 

  5. Carlson KM, Dou S, Chi D, Scavarda N, Toshima K, Jackson CE, et al. Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci U S A. 1994;91(4):1579–83.

    Article  CAS  Google Scholar 

  6. Hofstra RM, Landsvater RM, Ceccherini I, Stulp RP, Stelwagen T, Luo Y, et al. A mutation in the RET proto-oncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma. Nature. 1994;367(6461):375–6.

    Article  CAS  Google Scholar 

  7. Eng C, Smith DP, Mulligan LM, Nagai MA, Healey CS, Ponder MA, et al. Point mutation within the tyrosine kinase domain of the RET proto-oncogene in multiple endocrine neoplasia type 2B and related sporadic tumours. Hum Mol Genet. 1994;3(2):237–41.

    Article  CAS  Google Scholar 

  8. Marsh DJ, McDowall D, Hyland VJ, Andrew SD, Schnitzler M, Gaskin EL, et al. The identification of false positive responses to the pentagastrin stimulation test in RET mutation negative members of MEN 2A families. Clin Endocrinol. 1996;44(2):213–20.

    Article  CAS  Google Scholar 

  9. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86(12):5658–71.

    Article  CAS  Google Scholar 

  10. Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, American Thyroid Association Guidelines Task Force, et al. Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid. 2009;19(6):565–612.

    Article  Google Scholar 

  11. Leenhardt L, Erdogan MF, Hegedus L, Mandel SJ, Paschke R, Rago T, et al. 2013 European thyroid association guidelines for cervical ultrasound scan and ultrasound-guided techniques in the postoperative management of patients with thyroid cancer. Eur Thyroid J. 2013;2(3):147–59.

    Article  CAS  Google Scholar 

  12. Schroder S, Bocker W, Baisch H, Burk CG, Arps H, Meiners I, et al. Prognostic factors in medullary thyroid carcinomas. Survival in relation to age, sex, stage, histology, immunocytochemistry, and DNA content. Cancer. 1988;61(4):806–16.

    Article  CAS  Google Scholar 

  13. Papaparaskeva K, Nagel H, Droese M. Cytologic diagnosis of medullary carcinoma of the thyroid gland. Diagn Cytopathol. 2000;22(6):351–8.

    Article  CAS  Google Scholar 

  14. Rios A, Rodriguez JM, Febrero B, Acosta Ortega JM, Torregrosa N, Balsalobre M, et al. Histological and immunohistochemical profile of sporadic and familial medullary thyroid carcinoma. Endocrinol Nutr. 2011;58(10):521–8.

    Article  Google Scholar 

  15. Algeciras-Schimnich A, Preissner CM, Theobald JP, Finseth MS, Grebe SK. Procalcitonin: a marker for the diagnosis and follow-up of patients with medullary thyroid carcinoma. J Clin Endocrinol Metab. 2009;94(3):861–8.

    Article  CAS  Google Scholar 

  16. Walter MA, Meier C, Radimerski T, Iten F, Kranzlin M, Muller-Brand J, et al. Procalcitonin levels predict clinical course and progression-free survival in patients with medullary thyroid cancer. Cancer. 2010;116(1):31–40.

    CAS  PubMed  Google Scholar 

  17. Weber T, Schilling T, Frank-Raue K, Colombo-Benkmann M, Hinz U, Ziegler R, et al. Impact of modified radical neck dissection on biochemical cure in medullary thyroid carcinomas. Surgery. 2001;130(6):1044–9.

    Article  CAS  Google Scholar 

  18. Machens A, Hinze R, Thomusch O, Dralle H. Pattern of nodal metastasis for primary and reoperative thyroid cancer. World J Surg. 2002;26(1):22–8.

    Article  Google Scholar 

  19. Moley JF, DeBenedetti MK. Patterns of nodal metastases in palpable medullary thyroid carcinoma: recommendations for extent of node dissection. Ann Surg. 1999;229(6):880–7. discussion 7–8

    Article  CAS  Google Scholar 

  20. McMullen C, Rocke D, Freeman J. Complications of bilateral neck dissection in thyroid cancer from a single high-volume center. JAMA Otolaryngol Head Neck Surg. 2017;143:376–81.

    Article  Google Scholar 

  21. Machens A, Dralle H. Biomarker-based risk stratification for previously untreated medullary thyroid cancer. J Clin Endocrinol Metab. 2010;95(6):2655–63.

    Article  CAS  Google Scholar 

  22. Brierley J, Tsang R, Simpson WJ, Gospodarowicz M, Sutcliffe S, Panzarella T. Medullary thyroid cancer: analyses of survival and prognostic factors and the role of radiation therapy in local control. Thyroid. 1996;6(4):305–10.

    Article  CAS  Google Scholar 

  23. Orlandi F, Caraci P, Berruti A, Puligheddu B, Pivano G, Dogliotti L, et al. Chemotherapy with dacarbazine and 5-fluorouracil in advanced medullary thyroid cancer. Ann Oncol. 1994;5(8):763–5.

    Article  CAS  Google Scholar 

  24. Petursson SR. Metastatic medullary thyroid carcinoma. Complete response to combination chemotherapy with dacarbazine and 5-fluorouracil. Cancer. 1988;62(9):1899–903.

    Article  CAS  Google Scholar 

  25. Nixon IJ, Shaha AR, Tuttle MR. Targeted therapy in thyroid cancer. Curr Opin Otolaryngol Head Neck Surg. 2013;21(2):130–4.

    Article  Google Scholar 

  26. Brauckhoff M, Gimm O, Weiss CL, Ukkat J, Sekulla C, Brauckhoff K, et al. Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease. World J Surg. 2004;28(12):1305–11.

    Article  Google Scholar 

  27. Frank-Raue K, Buhr H, Dralle H, Klar E, Senninger N, Weber T, et al. Long-term outcome in 46 gene carriers of hereditary medullary thyroid carcinoma after prophylactic thyroidectomy: impact of individual RET genotype. Eur J Endocrinol. 2006;155(2):229–36.

    Article  CAS  Google Scholar 

  28. Engelbach M, Gorges R, Forst T, Pfutzner A, Dawood R, Heerdt S, et al. Improved diagnostic methods in the follow-up of medullary thyroid carcinoma by highly specific calcitonin measurements. J Clin Endocrinol Metab. 2000;85(5):1890–4.

    CAS  PubMed  Google Scholar 

  29. Barbet J, Campion L, Kraeber-Bodere F, Chatal JF, Group GTES. Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma. J Clin Endocrinol Metab. 2005;90(11):6077–84.

    Article  CAS  Google Scholar 

  30. Boi F, Maurelli I, Pinna G, Atzeni F, Piga M, Lai ML, et al. Calcitonin measurement in wash-out fluid from fine needle aspiration of neck masses in patients with primary and metastatic medullary thyroid carcinoma. J Clin Endocrinol Metab. 2007;92(6):2115–8.

    Article  CAS  Google Scholar 

  31. Kudo T, Miyauchi A, Ito Y, Takamura Y, Amino N, Hirokawa M. Diagnosis of medullary thyroid carcinoma by calcitonin measurement in fine-needle aspiration biopsy specimens. Thyroid. 2007;17(7):635–8.

    Article  Google Scholar 

  32. Pellegriti G, Leboulleux S, Baudin E, Bellon N, Scollo C, Travagli JP, et al. Long-term outcome of medullary thyroid carcinoma in patients with normal postoperative medical imaging. Br J Cancer. 2003;88(10):1537–42.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Ipswich Hospital NHS Trust, Ipswich, UK

    Anna I. Kaleva

  2. Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Ashok R. Shaha

  3. NHS Lothian, Edinburgh, UK

    Iain J. Nixon

Authors
  1. Anna I. Kaleva
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ashok R. Shaha
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Iain J. Nixon
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Consultant (Hon), Northern Centre for Cancer Care, Freeman Hospital, Newcastle upon Tyne, UK

    Ujjal K. Mallick

  2. London, UK

    Clive Harmer

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Kaleva, A.I., Shaha, A.R., Nixon, I.J. (2018). Practical Management of Thyroid Cancer: A Multidisciplinary Approach-Medullary Thyroid Cancer. In: Mallick, U.K., Harmer, C. (eds) Practical Management of Thyroid Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-91725-2_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-91725-2_18

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-91724-5

  • Online ISBN: 978-3-319-91725-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation