{"entity":{"id":"anaplastic-thyroid-cancer","kind":"cancer","name":"Anaplastic thyroid cancer","aka":["Anaplastic thyroid carcinoma","ATC","Undifferentiated thyroid carcinoma"],"tldr":"Anaplastic thyroid cancer is the rare, fast-growing form that presents as a rapidly enlarging neck mass threatening the airway. It was almost uniformly fatal within months; combining BRAF-targeted drugs, immunotherapy, surgery and radiotherapy has lifted survival for the first time.","summary":"Anaplastic thyroid cancer is an undifferentiated carcinoma that usually arises from a pre-existing papillary or follicular cancer through the accumulation of TP53 and TERT mutations; about 40 percent carry BRAF V600E. It presents in older adults as a hard, rapidly growing neck mass with hoarseness, breathing or swallowing difficulty, and it does not take up iodine. Every case is staged IV. Rapid molecular testing is now urged at diagnosis because BRAF V600E tumours respond to dabrafenib plus trametinib, approved in 2018 on the ROAR basket trial, and neoadjuvant use has made some inoperable tumours resectable; pembrolizumab added to targeted therapy and chemoradiation for resectable disease are part of the multimodal approach that raised one-year survival at MD Anderson from about a fifth to over half. Airway management and early palliative care remain central.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Anaplastic_thyroid_cancer","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Anaplastic_thyroid_cancer"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"endocrine","burden":"One to two percent of thyroid cancers but a large share of thyroid cancer deaths; median survival has historically been about six months, and every case is stage IV by definition.","subtypes":["BRAF V600E-mutant (about 40 percent)","RAS-mutant","Arising from differentiated thyroid cancer","Squamous and other rare patterns"],"biomarkers":["BRAF V600E (urgent testing at diagnosis)","TP53 and TERT promoter mutations","PD-L1 expression","NTRK, RET and ALK fusions (rare)"],"standardOfCare":[{"setting":"Diagnosis","approach":"Core biopsy with rapid BRAF testing, airway assessment, staging CT and PET; multidisciplinary planning within days.","refs":["braf"]},{"setting":"BRAF V600E-mutant","approach":"Dabrafenib plus trametinib, with pembrolizumab in many centres; neoadjuvant use to make tumours resectable, then surgery and radiotherapy.","refs":["dabrafenib","trametinib","pembrolizumab"]},{"setting":"BRAF wild-type, resectable","approach":"Surgery followed by chemoradiation (paclitaxel- or doxorubicin-based).","refs":["paclitaxel","doxorubicin","imrt-igrt"]},{"setting":"Unresectable BRAF wild-type","approach":"Chemoradiation or immunotherapy in trials; lenvatinib in some countries; palliative radiotherapy and airway support.","refs":["lenvatinib","palliative-radiotherapy","palliative-care"]}],"stateOfArt":["Dabrafenib-trametinib was the first drug approval ever for anaplastic thyroid cancer and turns some inoperable tumours into operable ones.","Immunotherapy with BRAF inhibition and multimodal treatment has more than doubled one-year survival in specialist centres.","Rapid molecular testing at diagnosis, within days, is now the standard of care because the window for treatment is short."],"history":[{"year":1990,"title":"Anaplastic cancer classified as stage IV in every case","refs":[]},{"year":2018,"title":"Dabrafenib plus trametinib approved (ROAR)","note":"The first drug approval for this cancer.","refs":["dabrafenib","trametinib"]},{"year":2020,"title":"MD Anderson reports survival gains with targeted therapy, immunotherapy and surgery","refs":[]}],"pipeline":["pembrolizumab","lenvatinib"],"openProblems":["Most patients still die within a year.","BRAF wild-type disease has no targeted option.","Trials are tiny because the disease is rare and fast."],"parent":"thyroid"},"route":"/cancers/anaplastic-thyroid-cancer/","neighbours":{"drug":[{"id":"dabrafenib","kind":"drug","name":"Dabrafenib","route":"/drugs/dabrafenib/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"lenvatinib","kind":"drug","name":"Lenvatinib","route":"/drugs/lenvatinib/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"},{"id":"trametinib","kind":"drug","name":"Trametinib","route":"/drugs/trametinib/"}],"target":[{"id":"braf","kind":"target","name":"BRAF","route":"/targets/braf/"}],"technology":[{"id":"palliative-care","kind":"technology","name":"Early integrated palliative care","route":"/technologies/palliative-care/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"palliative-radiotherapy","kind":"technology","name":"Palliative radiotherapy","route":"/technologies/palliative-radiotherapy/"}],"cancer":[{"id":"thyroid","kind":"cancer","name":"Thyroid cancer","route":"/cancers/thyroid/"}]}}