Anaplastic Thyroid Carcinoma

  • Anish Jacob Cherian
  • Deepak Abraham


Thyroid cancers are common world over. The majority are well-differentiated thyroid cancers which have a good prognosis. A minority of patients develop anaplastic thyroid cancer, an aggressive type of thyroid cancer with grave prognosis and limited treatment options. In order to improve outcomes for these patients the current area of research is in understanding the genetic and molecular pathogenesis of these cancers to facilitate targeted therapies. In this chapter, we review the epidemiology, clinicopathological features, investigations, treatment options and prognosis of anaplastic thyroid carcinomas.


Thyroid carcinoma Anaplastic Undifferentiated carcinoma 


  1. 1.
    Smallridge RC, Ain KB, Asa SL, Bible KC, Brierley JD, Burman KD, et al. American Thyroid Association Guidelines for management of patients with anaplastic thyroid cancer. Thyroid. 2012;22(11):1104–39.CrossRefGoogle Scholar
  2. 2.
    O’Neill JP, Shaha AR. Anaplastic thyroid cancer. Oral Oncol. 2013;49(7):702–6.CrossRefGoogle Scholar
  3. 3.
    Nagaiah G, Hossain A, Mooney CJ, Parmentier J, Remick SC, Nagaiah G, et al. Anaplastic thyroid cancer: a review of epidemiology, pathogenesis, and treatment. J Oncol. 2011;2011:e542358.CrossRefGoogle Scholar
  4. 4.
    Giuffrida D, Gharib H. Anaplastic thyroid carcinoma: current diagnosis and treatment. Ann Oncol. 2000;11(9):1083–9.CrossRefGoogle Scholar
  5. 5.
    Tan RK, Finley RK, Driscoll D, Bakamjian V, Hicks WL, Shedd DP. Anaplastic carcinoma of the thyroid: a 24-year experience. Head Neck. 1995;17(1):41–47-48.CrossRefGoogle Scholar
  6. 6.
    Ain KB. Anaplastic thyroid carcinoma: behavior, biology, and therapeutic approaches. Thyroid. 1998;8(8):715–26.CrossRefGoogle Scholar
  7. 7.
    Demeter JG, De Jong SA, Lawrence AM, Paloyan E. Anaplastic thyroid carcinoma: risk factors and outcome. Surgery. 1991;110(6):956–61. 961-3PubMedGoogle Scholar
  8. 8.
    Bakiri F, Djemli FK, Mokrane LA, Djidel FK. The relative roles of endemic goiter and socioeconomic development status in the prognosis of thyroid carcinoma. Cancer. 1998;82(6):1146–53.CrossRefGoogle Scholar
  9. 9.
    Keutgen XM, Sadowski SM, Kebebew E. Management of anaplastic thyroid cancer. Gland Surg. 2015;4(1):44–51.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Sun C, Li Q, Hu Z, He J, Li C, Li G, et al. Treatment and prognosis of anaplastic thyroid carcinoma: experience from a single institution in China. PLoS One. 2013;8(11):e80011.CrossRefGoogle Scholar
  11. 11.
    Parenti R, Salvatorelli L, Magro G. Anaplastic thyroid carcinoma: current treatments and potential new therapeutic options with emphasis on TfR1/CD71. Int J Endocrinol. 2014;2014:685396.CrossRefGoogle Scholar
  12. 12.
    Ragazzi M, Ciarrocchi A, Sancisi V, Gandolfi G, Bisagni A, Piana S. Update on anaplastic thyroid carcinoma: morphological, molecular, and genetic features of the most aggressive thyroid cancer. Int J Endocrinol. 2014;2014:790834.CrossRefGoogle Scholar
  13. 13.
    Patel KN, Shaha AR. Poorly differentiated and anaplastic thyroid cancer. Cancer Control. 2006;13(2):119–28.CrossRefGoogle Scholar
  14. 14.
    Papp S, Asa SL. When thyroid carcinoma goes bad: a morphological and molecular analysis. Head Neck Pathol. 2015;9(1):16–23.CrossRefGoogle Scholar
  15. 15.
    Smith N, Nucera C. Personalized therapy in patients with anaplastic thyroid cancer: targeting genetic and epigenetic alterations. J Clin Endocrinol Metab. 2015;100(1):35–42.CrossRefGoogle Scholar
  16. 16.
    Hsu K-T, Yu X-M, Audhya AW, Jaume JC, Lloyd RV, Miyamoto S, et al. Novel approaches in anaplastic thyroid cancer therapy. Oncologist. 2014;19(11):1148–55.CrossRefGoogle Scholar
  17. 17.
    Viola D, Valerio L, Molinaro E, Agate L, Bottici V, Biagini A, et al. Treatment of advanced thyroid cancer with targeted therapies: ten years of experience. Endocr Relat Cancer. 2016;23(4):R185–205.CrossRefGoogle Scholar
  18. 18.
    Are C, Shaha AR. Anaplastic thyroid carcinoma: biology, pathogenesis, prognostic factors, and treatment approaches. Ann Surg Oncol. 2006;13(4):453–64.CrossRefGoogle Scholar
  19. 19.
    Basolo F, Pollina L, Fontanini G, Fiore L, Pacini F, Baldanzi A. Apoptosis and proliferation in thyroid carcinoma: correlation with bcl-2 and p53 protein expression. Br J Cancer. 1997;75(4):537–41.CrossRefGoogle Scholar
  20. 20.
    Albores-Saavedra J, Sharma S. Poorly differentiated follicular thyroid carcinoma with rhabdoid phenotype: a clinicopathologic, immunohistochemical and electron microscopic study of two cases. Mod Pathol. 2001;14(2):98–104.CrossRefGoogle Scholar
  21. 21.
    Lai ML, Faa G, Serra S, Senes G, Daniele GM, Boi F, et al. Rhabdoid tumor of the thyroid gland: a variant of anaplastic carcinoma. Arch Pathol Lab Med. 2005;129(3):e55–7.PubMedGoogle Scholar
  22. 22.
    Canos JC, Serrano A, Matias-Guiu X. Paucicellular variant of anaplastic thyroid carcinoma: report of two cases. Endocr Pathol. 2001;12(2):157–61.CrossRefGoogle Scholar
  23. 23.
    Feng G, Laskin WB, Chou PM, Lin X. Anaplastic thyroid carcinoma with rhabdoid features. Diagn Cytopathol. 2015;43(5):416–20.CrossRefGoogle Scholar
  24. 24.
    Nel CJ, van Heerden JA, Goellner JR, Gharib H, McConahey WM, Taylor WF, et al. Anaplastic carcinoma of the thyroid: a clinicopathologic study of 82 cases. Mayo Clin Proc. 1985;60(1):51–8.CrossRefGoogle Scholar
  25. 25.
    Venkatesh YS, Ordonez NG, Schultz PN, Hickey RC, Goepfert H, Samaan NA. Anaplastic carcinoma of the thyroid. A clinicopathologic study of 121 cases. Cancer. 1990;66(2):321–30.CrossRefGoogle Scholar
  26. 26.
    Hadar T, Mor C, Shvero J, Levy R, Segal K. Anaplastic carcinoma of the thyroid. Eur J Surg Oncol. 1993;19(6):511–6.PubMedGoogle Scholar
  27. 27.
    Ain KB. Anaplastic thyroid carcinoma: a therapeutic challenge. Semin Surg Oncol. 1999;16(1):64–9.CrossRefGoogle Scholar
  28. 28.
    Aldinger KA, Samaan NA, Ibanez M, Hill CS. Anaplastic carcinoma of the thyroid: a review of 84 cases of spindle and giant cell carcinoma of the thyroid. Cancer. 1978;41(6):2267–75.CrossRefGoogle Scholar
  29. 29.
    Biganzoli L, Gebbia V, Maiorino L, Caraci P, Iirillo A. Thyroid cancer: different outcomes to chemotherapy according to tumour histology. Eur J Cancer. 1995;31A(13–14):2423–4.CrossRefGoogle Scholar
  30. 30.
    Lo CY, Lam KY, Wan KY. Anaplastic carcinoma of the thyroid. Am J Surg. 1999;177(4):337–9.CrossRefGoogle Scholar
  31. 31.
    Passler C, Scheuba C, Prager G, Kaserer K, Flores JA, Vierhapper H, et al. Anaplastic (undifferentiated) thyroid carcinoma (ATC). Langenbeck’s Arch Surg. 1999;384(3):284–93.CrossRefGoogle Scholar
  32. 32.
    Pierie J-PEN, Muzikansky A, Gaz RD, Faquin WC, Ott MJ. The effect of surgery and radiotherapy on outcome of anaplastic thyroid carcinoma. Ann Surg Oncol. 2002;9(1):57–64.CrossRefGoogle Scholar
  33. 33.
    Kebebew E, Greenspan FS, Clark OH, Woeber KA, McMillan A. Anaplastic thyroid carcinoma. Cancer. 2005;103(7):1330–5.CrossRefGoogle Scholar
  34. 34.
    Agrawal S, Rao RS, Parikh DM, Parikh HK, Borges AM, Sampat MB. Histologic trends in thyroid cancer 1969-1993: a clinico-pathologic analysis of the relative proportion of anaplastic carcinoma of the thyroid. J Surg Oncol. 1996;63(4):251–5.CrossRefGoogle Scholar
  35. 35.
    Besic N, Auersperg M, Us-Krasovec M, Golouh R, Frkovic-Grazio S, Vodnik A. Effect of primary treatment on survival in anaplastic thyroid carcinoma. Eur J Surg Oncol. 2001;27(3):260–4.CrossRefGoogle Scholar
  36. 36.
    McIver B, Hay ID, Giuffrida DF, Dvorak CE, Grant CS, Thompson GB, et al. Anaplastic thyroid carcinoma: a 50-year experience at a single institution. Surgery. 2001;130(6):1028–34.CrossRefGoogle Scholar
  37. 37.
    Lang BH-H, Lo C-Y. Surgical options in undifferentiated thyroid carcinoma. World J Surg. 2007;31(5):969–77.CrossRefGoogle Scholar
  38. 38.
    Poisson T, Deandreis D, Leboulleux S, Bidault F, Bonniaud G, Baillot S, et al. 18F-fluorodeoxyglucose positron emission tomography and computed tomography in anaplastic thyroid cancer. Eur J Nucl Med Mol Imaging. 2010;37(12):2277–85.CrossRefGoogle Scholar
  39. 39.
    Nguyen BD, Ram PC. PET/CT staging and posttherapeutic monitoring of anaplastic thyroid carcinoma. Clin Nucl Med. 2007;32(2):145–9.CrossRefGoogle Scholar
  40. 40.
    Samih N, Hovsepian S, Notel F, Prorok M, Zattara-Cannoni H, Mathieu S, et al. The impact of N- and O-glycosylation on the functions of Glut-1 transporter in human thyroid anaplastic cells. Biochim Biophys Acta. 2003;1621(1):92–101.CrossRefGoogle Scholar
  41. 41.
    Swaak-Kragten AT, de Wilt JHW, Schmitz PIM, Bontenbal M, Levendag PC. Multimodality treatment for anaplastic thyroid carcinoma—treatment outcome in 75 patients. Radiother Oncol. 2009;92(1):100–4.CrossRefGoogle Scholar
  42. 42.
    Haigh PI, Ituarte PH, Wu HS, Treseler PA, Posner MD, Quivey JM, et al. Completely resected anaplastic thyroid carcinoma combined with adjuvant chemotherapy and irradiation is associated with prolonged survival. Cancer. 2001;91(12):2335–42.CrossRefGoogle Scholar
  43. 43.
    Goutsouliak V, Hay JH. Anaplastic thyroid cancer in British Columbia 1985-1999: a population-based study. Clin Oncol (R Coll Radiol). 2005;17(2):75–8.CrossRefGoogle Scholar
  44. 44.
    Brignardello E, Gallo M, Baldi I, Palestini N, Piovesan A, Grossi E, et al. Anaplastic thyroid carcinoma: clinical outcome of 30 consecutive patients referred to a single institution in the past 5 years. Eur J Endocrinol. 2007;156(4):425–30.CrossRefGoogle Scholar
  45. 45.
    Kihara M, Miyauchi A, Yamauchi A, Yokomise H. Prognostic factors of anaplastic thyroid carcinoma. Surg Today. 2004;34(5):394–8.CrossRefGoogle Scholar
  46. 46.
    Veness MJ, Porter GS, Morgan GJ. Anaplastic thyroid carcinoma: dismal outcome despite current treatment approach. ANZ J Surg. 2004;74(7):559–62.CrossRefGoogle Scholar
  47. 47.
    Derbel O, Limem S, Ségura-Ferlay C, Lifante J-C, Carrie C, Peix J-L, et al. Results of combined treatment of anaplastic thyroid carcinoma (ATC). BMC Cancer. 2011;11(1):469.CrossRefGoogle Scholar
  48. 48.
    De Besi P, Busnardo B, Toso S, Girelli ME, Nacamulli D, Simioni N, et al. Combined chemotherapy with bleomycin, adriamycin, and platinum in advanced thyroid cancer. J Endocrinol Investig. 1991;14(6):475–80.CrossRefGoogle Scholar
  49. 49.
    Voigt W, Kegel T, Weiss M, Mueller T, Simon H, Schmoll HJ. Potential activity of paclitaxel, vinorelbine and gemcitabine in anaplastic thyroid carcinoma. J Cancer Res Clin Oncol. 2005;131(9):585–90.CrossRefGoogle Scholar
  50. 50.
    Shimaoka K, Schoenfeld DA, DeWys WD, Creech RH, DeConti R. A randomized trial of doxorubicin versus doxorubicin plus cisplatin in patients with advanced thyroid carcinoma. Cancer. 1985;56(9):2155–60.CrossRefGoogle Scholar
  51. 51.
    Segerhammar I, Larsson C, Nilsson I-L, Bäckdahl M, Höög A, Wallin G, et al. Anaplastic carcinoma of the thyroid gland: treatment and outcome over 13 years at one institution. J Surg Oncol. 2012;106(8):981–6.CrossRefGoogle Scholar
  52. 52.
    Tennvall J, Lundell G, Wahlberg P, Bergenfelz A, Grimelius L, Akerman M, et al. Anaplastic thyroid carcinoma: three protocols combining doxorubicin, hyperfractionated radiotherapy and surgery. Br J Cancer. 2002;86(12):1848–53.CrossRefGoogle Scholar
  53. 53.
    Tennvall J, Lundell G, Hallquist A, Wahlberg P, Wallin G, Tibblin S. Combined doxorubicin, hyperfractionated radiotherapy, and surgery in anaplastic thyroid carcinoma. Report on two protocols. The Swedish Anaplastic Thyroid Cancer Group. Cancer. 1994;74(4):1348–54.CrossRefGoogle Scholar
  54. 54.
    De Crevoisier R, Baudin E, Bachelot A, Leboulleux S, Travagli J-P, Caillou B, et al. Combined treatment of anaplastic thyroid carcinoma with surgery, chemotherapy, and hyperfractionated accelerated external radiotherapy. Int J Radiat Oncol Biol Phys. 2004;60(4):1137–43.CrossRefGoogle Scholar
  55. 55.
    Denaro N, Nigro CL, Russi EG, Merlano MC. The role of chemotherapy and latest emerging target therapies in anaplastic thyroid cancer. Onco Targets Ther. 2013;9:1231–41.CrossRefGoogle Scholar
  56. 56.
    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(3):564–73.CrossRefGoogle Scholar
  57. 57.
    Kim TY, Kim KW, Jung TS, Kim JM, Kim SW, Chung K-W, et al. Prognostic factors for Korean patients with anaplastic thyroid carcinoma. Head Neck. 2007;29(8):765–72.CrossRefGoogle Scholar
  58. 58.
    Sugitani I, Kasai N, Fujimoto Y, Yanagisawa A. Prognostic factors and therapeutic strategy for anaplastic carcinoma of the thyroid. World J Surg. 2001;25(5):617–22.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Singapore 2018

Authors and Affiliations

  • Anish Jacob Cherian
    • 1
  • Deepak Abraham
    • 1
  1. 1.Department of Endocrine SurgeryChristian Medical College and HospitalVelloreIndia

Personalised recommendations