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Anaplastic thyroid cancer

Authors
R Michael Tuttle, MD
Eric J Sherman, MD
Section Editors
David S Cooper, MD
Douglas S Ross, MD
Deputy Editor
Jean E Mulder, MD

INTRODUCTION

Anaplastic thyroid cancers are undifferentiated tumors of the thyroid follicular epithelium. In marked contrast to differentiated thyroid cancers, anaplastic cancers are extremely aggressive, with a disease-specific mortality approaching 100 percent. Given the very rapid course of disease progression and the poor treatment outcomes, end-of-life issues and plans for comfort care measures are an integral part of initial disease management planning [1]. Early recognition of the disease is essential to allow prompt initiation of therapy.

The major clinical issues related to anaplastic thyroid cancer will be reviewed here. The molecular pathogenesis of this disorder is discussed separately. (See "Oncogenes and tumor suppressor genes in thyroid nodules and nonmedullary thyroid cancer".)

EPIDEMIOLOGY

The age-adjusted annual incidence of anaplastic cancer is approximately one to two per million persons [2-4] and accounts for 0.9 to 9.8 percent of all thyroid cancers globally [5,6]. Patients with anaplastic cancer are older than those with differentiated cancer; the mean age at diagnosis is 65 years and fewer than 10 percent are younger than 50 years. Sixty to 70 percent of tumors occur in women [7,8].

ANTECEDENT THYROID DISEASE

Approximately 20 percent of patients with anaplastic thyroid cancer have a history of differentiated thyroid cancer, and 20 to 30 percent have a coexisting differentiated cancer [9-13]; the percentage may be even higher with extensive sectioning of the thyroid gland [14]. The majority of synchronous thyroid tumors are papillary cancers, but coexisting follicular cancers have also been reported. Nearly 10 percent of patients with Hürthle cell cancers have foci of anaplastic cancer within the Hürthle cell cancer [15]. In addition, transformation from differentiated to anaplastic cancer has been described in a patient who was followed with serial biopsies [16].

These findings lend support to the hypothesis that anaplastic cancer develops from more differentiated tumors as a result of one or more dedifferentiating events [17]. Since activating mutations in BRAF and RAS are seen in both well-differentiated thyroid malignancies and anaplastic thyroid cancer, these are presumed to be early events in the progression pathway [18]. Late events that are seen more commonly in the anaplastic tumor rather than the precursor well-differentiated tumor include mutations in p53 tumor suppressor protein [19-22], 16p [23], catenin (cadherin-associated protein), beta 1, and PIK3CA [24].

                         

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Literature review current through: Nov 2016. | This topic last updated: Mon Nov 14 00:00:00 GMT+00:00 2016.
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