2021 American Thyroid Association Guidelines for Management of Patients with Anaplastic Thyroid Cancer

Abstract

Background: Anaplastic thyroid cancer (ATC) is a rare but highly lethal form of thyroid cancer. Since the guidelines for the management of ATC by the American Thyroid Association were first published in 2012, significant clinical and scientific advances have occurred in the field. The aim of these guidelines is to inform clinicians, patients, and researchers on published evidence relating to the diagnosis and management of ATC. Methods: The specific clinical questions and topics addressed in these guidelines were based on prior versions of the guidelines, stakeholder input, and input of the Task Force members (authors of the guideline). Relevant literature was reviewed, including serial PubMed searches supplemented with additional articles. The American College of Physicians Guideline Grading System was used for critical appraisal of evidence and grading strength of recommendations. Results: The guidelines include the diagnosis, initial evaluation, establishment of treatment goals, approaches to locoregional disease (surgery, radiotherapy, targeted/systemic therapy, supportive care during active therapy), approaches to advanced/metastatic disease, palliative care options, surveillance and long-term monitoring, and ethical issues, including end of life. The guidelines include 31 recommendations and 16 good practice statements. Conclusions: We have developed evidence-based recommendations to inform clinical decision-making in the management of ATC. While all care must be individualized, such recommendations provide, in our opinion, optimal care paradigms for patients with ATC.

Keywords: BRAF; IMRT (intensity-modulated radiotherapy); SBRT (stereotactic body radiation therapy); chemoradiation; chemotherapy; ethics; multimodality therapy; palliative care; radiation therapy; squamous cell cancer of the thyroid; surgery; targeted therapy; undifferentiated thyroid cancer.

FIG. 1.

Initial treatment of stages IVA and IVB ATC. ¹Additonal agents exist and are in development, listing not meant to be comprehensive; clinical trials preferred if available; see text. *Cytotoxic chemotherapy may be started as a “bridge” while awaiting genomic information or while awaiting targeted therapy (e.g., dabrafenib and trametinib). Dashed arrows depict circumstances where competing therapeutic options may be of consideration. ATC, anaplastic thyroid cancer.

FIG. 2.

Initial treatment of stage IVC ATC. ¹Additonal agents exist and are in development, listings not meant to be comprehensive; clinical trials preferred if available; see text. *Cytotoxic chemotherapy may be started as a “bridge” while awaiting genomic information or while awaiting targeted therapy (e.g., dabrafenib and trametinib). **Consolidate Rx refers to focal therapy intended to control residual macrometastatic disease among those electing aggressive therapy. Dashed arrows depict circumstances where competing therapeutic options may be of consideration. TMB, tumor mutational burden.