Anaplastic thyroid carcinoma: from clinicopathology to genetics and advanced therapies

Nat Rev Endocrinol. 2017 Nov;13(11):644-660. doi: 10.1038/nrendo.2017.76. Epub 2017 Jul 14.


Anaplastic thyroid carcinoma (ATC) is a rare malignancy, accounting for 1-2% of all thyroid cancers. Although rare, ATC accounts for the majority of deaths from thyroid carcinoma. ATC often originates in a pre-existing thyroid cancer lesion, as suggested by the simultaneous presence of areas of differentiated or poorly differentiated thyroid carcinoma. ATC is characterized by the accumulation of several oncogenic alterations, and studies have shown that an increased number of oncogenic alterations equates to an increased level of dedifferentiation and aggressiveness. The clinical management of ATC requires a multidisciplinary approach; according to recent American Thyroid Association guidelines, surgery, radiotherapy and/or chemotherapy should be considered. In addition to conventional therapies, novel molecular targeted therapies are the most promising emerging treatment modalities. These drugs are often multiple receptor tyrosine kinase inhibitors, several of which have been tested in clinical trials with encouraging results so far. Accordingly, clinical trials are ongoing to evaluate the safety, efficacy and effectiveness of these new agents. This Review describes the updated clinical and pathological features of ATC and provides insight into the molecular biology of this disease. The most recent literature regarding conventional, newly available and future therapies for ATC is also discussed.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Deglutition Disorders / etiology
  • Dyspnea / etiology
  • GTP Phosphohydrolases / genetics
  • Hoarseness / etiology
  • Humans
  • Membrane Proteins / genetics
  • Neck Pain / etiology
  • Neoplasm Staging
  • Protein Kinase Inhibitors / therapeutic use*
  • Proto-Oncogene Proteins B-raf / genetics
  • Proto-Oncogene Proteins p21(ras) / genetics
  • Radiation Exposure / statistics & numerical data
  • Radiotherapy*
  • Respiratory Sounds / etiology
  • Risk Factors
  • Stilbenes / therapeutic use
  • Telomerase / genetics
  • Thiazolidinediones / therapeutic use
  • Thyroid Carcinoma, Anaplastic / complications
  • Thyroid Carcinoma, Anaplastic / genetics
  • Thyroid Carcinoma, Anaplastic / pathology
  • Thyroid Carcinoma, Anaplastic / therapy*
  • Thyroid Neoplasms / complications
  • Thyroid Neoplasms / genetics
  • Thyroid Neoplasms / pathology
  • Thyroid Neoplasms / therapy*
  • Thyroidectomy*


  • Membrane Proteins
  • Protein Kinase Inhibitors
  • Stilbenes
  • Thiazolidinediones
  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf
  • TERT protein, human
  • Telomerase
  • GTP Phosphohydrolases
  • NRAS protein, human
  • HRAS protein, human
  • Proto-Oncogene Proteins p21(ras)
  • fosbretabulin
  • efatutazone