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Anaplastic Thyroid Cancer Treatment by Multidisciplinary Palliative, Radiation, Oncology, and Surgical Teams

by Kareem Al-Mulki, MD, and Richard V. Smith, MD •

TRIO Best PracticeTRIO Best Practice articles are brief, structured reviews designed to provide the busy clinician with a handy outline and reference for day-to-day clinical decision making. The ENTtoday summary below includes the Background and Best Practice sections of the original article. To view the complete Laryngoscope articles free of charge, visit Laryngoscope.com.

BACKGROUND

With a historical median survival of about five months and a one-year survival of 20%, anaplastic thyroid cancer (ATC) is recognized as the most fatal of all thyroid tumors, but it only accounts for 1.7% of all thyroid cancers in the United States (Thyroid. 2021;31:337-386). Poor survival, coupled with the rarity of the disease, has made conducting adequate prospective treatment studies difficult. Nevertheless, such a poor prognosis necessitates quickly establishing the diagnosis, outlining goals of care, and creating a treatment plan. Numerous treatment modalities have been advocated for ATC, including various combinations of surgery, radiotherapy, chemotherapy, and, most recently, biotherapy; however, the role of aggressive multimodal therapy, in particular surgery with curative intent followed by chemoradiation, remains unclear.

BEST PRACTICE

Given its poor prognosis and rapidly fatal course, a high suspicion for ATC should be maintained in any patient presenting with a rapidly expanding central neck mass. Initial workup should begin with contrast-enhanced CT of the neck, chest, and abdomen/pelvis, along with a core biopsy that is urgently sent for BRAFV600E mutation testing. Resectable stage IVA/IVB tumors should be treated with surgery followed by chemoradiation, with consideration for dabrafenib/trametinib biotherapy for BRAFV600E-mutated tumors. Unresectable stage IVA/IVB and stage IVC tumors that require aggressive therapy should be urgently assessed for targeted biotherapies. If not eligible for this therapy, patients who desire aggressive treatment should be offered chemoradiation. Overall, ATC treatment should be handled by multidisciplinary palliative, radiation, oncology, and surgical teams.