Where surgery is not amenable as a first-line option, radiation and chemotherapy may downstage the disease, allowing for surgery to aid with locoregional control. This is then followed by radiation and chemotherapy. When extrathyroidal is encountered, en bloc resection should be considered if grossly negative margins can be achieved. The recommendation from the ATA is for a total thyroidectomy with therapeutic lymph node dissection in these patients. Ten per cent of patients with ATC present with intra-thyroidal disease alone. The role of surgery in this disease ultimately depends on the disease characteristics. To summarize, the ATA guidelines, patients with stages IVa and IVb (potentially resectable disease) should be treated with a multimodality approach, including surgery, external beam radiation and systemic therapy. This chapter nevertheless serves as a great resource for the clinician caring for patients with this rare tumour. This is the first time the ATA has put together guidelines for the management of ATC. Recently, guidelines for the management of ATC were published by the American Thyroid Association (ATA). Although to date treatment of ATC remains largely ineffective, the authors outline the potential benefit of surgery, radiation, chemotherapy, and the newer targeted molecular therapies that have shown promise as therapeutic options. This chapter covers presentation, the molecular and genetic characterization of these tumours, and the treatment option currently utilized. The current treatment regimes are multimodal and include surgery, radiation and systemic therapy.
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Galindo and Wiseman have provided strong evidence to suggest that an ATC develops from pre-existing well-differentiated thyroid cancer. All ATCs are considered stage IV tumours, according to the current American Joint Committee on Cancer (AJCC) staging system.
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Anaplastic thyroid cancer (ATC) is a rare but highly lethal form of thyroid cancer.