Anaplastic thyroid cancer
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.
Overview
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.
State of the art
- 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.
Anatomy and lymph node drainage
Site decides cause and behaviour: HPV drives oropharyngeal cancer, EBV drives nasopharyngeal cancer, tobacco drives oral and laryngeal cancer; all drain into the neck node levels that surgeons and radiotherapists map.
- Oral cavity and tongue
- Oropharynx: tonsil, base of tongue (HPV)
- Nasopharynx (EBV)
- Larynx and hypopharynx
- Parotid and other salivary glands
- ThyroidBRAF V600E-mutant (about 40 percent) · Arising from differentiated thyroid cancer
- level I (submandibular)
- level II (upper jugular)
- level III-IV (jugular)
- level V (posterior)
- level VI (central, thyroid)
- retropharyngeal (nasopharynx)
Same organ: Oropharyngeal cancer (tonsil and base of tongue), Laryngeal and hypopharyngeal cancer, Oral cavity cancer (mouth and tongue), Head and neck squamous cell carcinoma, Nasopharyngeal carcinoma, Salivary gland cancers, Papillary thyroid cancer, Follicular thyroid cancer, Medullary thyroid cancer, Thyroid cancer, Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), NUT carcinoma (midline carcinoma with NUTM1 rearrangement), Parathyroid carcinoma, Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- 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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Core biopsy with rapid BRAF testing, airway assessment, staging CT and PET; multidisciplinary planning within days.
Dabrafenib plus trametinib, with pembrolizumab in many centres; neoadjuvant use to make tumours resectable, then surgery and radiotherapy.
Surgery followed by chemoradiation (paclitaxel- or doxorubicin-based).
Chemoradiation or immunotherapy in trials; lenvatinib in some countries; palliative radiotherapy and airway support.
Subtypes & biomarkers
top- BRAF V600E (urgent testing at diagnosis)
- TP53 and TERT promoter mutations
- PD-L1 expression
- NTRK, RET and ALK fusions (rare)
How often this target appears
- 1990Anaplastic cancer classified as stage IV in every case
- 2018Dabrafenib plus trametinib approved (ROAR)
The first drug approval for this cancer.
- 2020MD Anderson reports survival gains with targeted therapy, immunotherapy and surgery
What is in development for Anaplastic thyroid cancer, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
Most patients still die within a year.
BRAF wild-type disease has no targeted option.
and how the field plans to fix it →What is being done about thisCancers without a drug targetAvailable now- AdagrasibApproved
- CRISPR functional genomicsEstablished
- DaraxonrasibApproved
- KRAS & RAS inhibitorsApproved
- PROTACs & molecular glues (targeted protein degradation)Approved
- RevumenibApproved
In trials- AI-driven drug & target discoveryPhase 2
- CodeBreaK 200Positive
- ELI-002 7PPhase 2
- ElironrasibPhase 2
- KRYSTAL-12Positive
- MRTX1133Phase 1
Ideas and roadmaps- A drug screen that only rewards killing sleeping cancer cells
- A guaranteed purchase prize for the first drug against a named hard target
- A precompetitive consortium for the twenty hardest cancer targets
- A synthetic lethality map for every cancer driver in every tissue context
- A target de-risking index that counts failures as well as successes
- AI-designed proteins that grip the floppy parts of cancer drivers
Background: p53 / RB / cell-cycle checkpoint, RAS / RAF / MEK / ERK (MAPK). Also on OnCo: Targets · KRAS roadmap.
Trials are tiny because the disease is rare and fast.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- Johns Hopkins Hospital / Sidney Kimmel Comprehensive Cancer CenterBaltimore, USNewsweek oncology #10NCI comprehensivevia Pembrolizumab
- Aarhus University HospitalAarhus, DKvia IMRT / IGRT (modern external beam)
- Alliance for Clinical Trials in OncologyChicago, IL, USvia Pembrolizumab
- American Society for Radiation OncologyArlington, VA, USvia IMRT / IGRT (modern external beam)
- Centre Antoine LacassagneNice, FRvia IMRT / IGRT (modern external beam)
- Centre Oscar LambretLille, FRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Christian Medical College, VelloreVellore, INvia Early integrated palliative care
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia IMRT / IGRT (modern external beam)
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- Geneva University Hospitals (HUG)Geneva, CHvia IMRT / IGRT (modern external beam)
- GOG FoundationPhiladelphia, PA, USvia Pembrolizumab
- Groote Schuur Hospital / University of Cape TownCape Town, ZAvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Hacettepe University Cancer InstituteAnkara, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ho Chi Minh City Oncology HospitalHo Chi Minh City, VNvia IMRT / IGRT (modern external beam)
- Hokkaido University HospitalSapporo, JPvia IMRT / IGRT (modern external beam)
- Hunan Cancer HospitalChangsha, CNvia IMRT / IGRT (modern external beam)
- Institut BergoniéBordeaux, FRvia IMRT / IGRT (modern external beam)
- Institut Jules BordetBrussels, BEvia Pembrolizumab
- Institut National d'Oncologie, RabatRabat, MAvia IMRT / IGRT (modern external beam)
- Institut Salah AzaïezTunis, TNvia IMRT / IGRT (modern external beam)
- Institute of Oncology LjubljanaLjubljana, SIvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia IMRT / IGRT (modern external beam)
- Istanbul University Institute of OncologyIstanbul, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia IMRT / IGRT (modern external beam)
- Kenyatta National HospitalNairobi, KEvia IMRT / IGRT (modern external beam)
- Korle Bu Teaching HospitalAccra, GHvia IMRT / IGRT (modern external beam)
- Lagos University Teaching HospitalLagos, NGvia IMRT / IGRT (modern external beam)
- via Pembrolizumab
- via IMRT / IGRT (modern external beam)
- Li Ka Shing FoundationHong Kong, HKvia Early integrated palliative care
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- National Institute of Oncology, HungaryBudapest, HUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ocean Road Cancer InstituteDar es Salaam, TZvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Rajiv Gandhi Cancer Institute and Research CentreNew Delhi, INvia IMRT / IGRT (modern external beam)
- Rambam Health Care CampusHaifa, ILvia IMRT / IGRT (modern external beam)
- Rigshospitalet, Copenhagen University HospitalCopenhagen, DKvia IMRT / IGRT (modern external beam)
- Royal Adelaide HospitalAdelaide, AUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- TROG Cancer ResearchNewcastle, NSW, AUvia IMRT / IGRT (modern external beam)
- UMC Utrecht Cancer CenterUtrecht, NLvia IMRT / IGRT (modern external beam)
- University of Malaya Medical CentreKuala Lumpur, MYvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Velindre Cancer CentreCardiff, GBvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Wellcome Sanger InstituteHinxton, GBvia BRAF
- Zhejiang Cancer HospitalHangzhou, CNvia IMRT / IGRT (modern external beam)
Questions to ask
topQuestions to ask your oncologist about Anaplastic thyroid cancer
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example BRAF V600E, TP53 and TERT promoter mutations, PD-L1 expression, NTRK, RET and ALK fusions), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include BRAF V600E-mutant, RAS-mutant, Arising from differentiated thyroid cancer.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Why: Guideline options include: Core biopsy with rapid BRAF testing, airway assessment, staging CT and PET; multidisciplinary planning within days.
BRAF V600E-mutant
- For my situation (braf v600e-mutant), which of the standard options do you recommend and why?Why: Guideline options include: Dabrafenib plus trametinib, with pembrolizumab in many centres; neoadjuvant use to make tumours resectable, then surgery and radiotherapy.
- Am I a candidate for Dabrafenib, Trametinib, Pembrolizumab, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
BRAF wild-type, resectable
- For my situation (braf wild-type, resectable), which of the standard options do you recommend and why?Why: Guideline options include: Surgery followed by chemoradiation (paclitaxel- or doxorubicin-based).
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Unresectable BRAF wild-type
- For my situation (unresectable braf wild-type), which of the standard options do you recommend and why?Why: Guideline options include: Chemoradiation or immunotherapy in trials; lenvatinib in some countries; palliative radiotherapy and airway support.
- Am I a candidate for Lenvatinib, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Most patients still die within a year”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “BRAF wild-type disease has no targeted option”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
7drugs
6companies
4Latest papers
topQuery for this cancer: (TITLE:"Anaplastic thyroid cancer" OR ABSTRACT:"Anaplastic thyroid cancer" OR TITLE:"Anaplastic thyroid carcinoma" OR ABSTRACT:"Anaplastic thyroid carcinoma" OR TITLE:"ATC" OR ABSTRACT:"ATC" OR TITLE:"Undifferentiated thyroid carcinoma" OR ABSTRACT:"Undifferentiated thyroid carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Anaplastic thyroid cancer, not a curated reading list.
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