Abstract
Simple SummaryAnaplastic thyroid carcinoma (ATC) has a dismal prognostic. Chemotherapy and radiotherapy are the mainstem options for patients with ATC. In selected cases with actionable genomic alterations or with favorable immune tumor microenvironment, new therapeutic options as targeted therapies and immunotherapy have led to better outcome and raised some hope for treatment of this deadly disease.Anaplastic thyroid carcinoma (ATC) is a rare and undifferentiated form of thyroid cancer. Its prognosis is poor: the median overall survival (OS) of patients varies from 4 to 10 months after diagnosis. However, a doubling of the OS time may be possible owing to a more systematic use of molecular tests for targeted therapies and integration of fast-track dedicated care pathways for these patients in tertiary centers. The diagnostic confirmation, if needed, requires an urgent biopsy reread by an expert pathologist with additional immunohistochemical and molecular analyses. Therapeutic management, defined in multidisciplinary meetings, respecting the patient’s choice, must start within days following diagnosis. For localized disease diagnosed after primary surgical treatment, adjuvant chemo-radiotherapy is recommended. In the event of locally advanced or metastatic disease, the prognosis is very poor. Treatment should then involve chemotherapy or targeted therapy and decompressive cervical radiotherapy. Here we will review current knowledge on ATC and provide perspectives to improve the management of this deadly disease.
Highlights
Anaplastic thyroid cancer (ATC) is a rare malignancy with a poor prognosis
In a recent series of 126 samples of Anaplastic thyroid carcinoma (ATC) analyzed by Generation Sequencing (NGS), the most common molecular alterations were found in TERT promoter (75%), TP 53 (63%), BRAF (45%), RAS (22%), PIK3CA (18%), EIF1AX (14%) and PTEN (14%) with the first two being more frequent in ATC than the others, which can be seen in either differentiated thyroid cancer (DTC) or Poorly differentiated DTC (PDTC) [7,28]
BRAF mutations are found in 40–45% of cases in US studies [7,14] whereas they are found in 14–37% of cases in European studies [27,30,33]; data from south Korea reported a rate of 41% BRAF alterations in a series of 13 ATC cases [34]
Summary
Anaplastic thyroid cancer (ATC) is a rare malignancy with a poor prognosis. It is characterized by a rapid onset with local and distant metastases, local progression and distant evolution [1]. 2. Epidemiology and Clinical Presentation: A Rare Disease with a Rapid Onset and Poor Prognosis. Recent epidemiological studies have confirmed an age-adjusted incidence in the US of 0.12 per 100,000 person-years (95% CI: 0.8–1.6) in 2014 in the Surveillance, Epidemiology, and End Results (SEER) database [16] and 0.1 to 0.3 per 100,000 personyears in Europe according to data from Denmark [4], Wales [17] and the Netherlands [18] registries. The repercussions of local tumor spread on the general condition immediately indicate the gravity of the situation
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