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Anaplastisches Schilddrüsenkarzinom (ATC)

Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.

IDDIS-THYROID-ANAPLASTIC
ArtErkrankung
Aliase
ATCAnaplastisches SchilddrüsenkarzinomUndifferentiated thyroid carcinomaАнапластична карцинома щитовидної залози
Statusreviewed 2026-04-27 | pending_clinical_signoff
ErkrankungenNone declared
QuellenSRC-NCCN-CNS-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Quelle: YAML

icd_o_3_morphology8021/3
icd_o_3_topography["C73"]
icd_10C73
who_classificationWHO Endokrine Tumore 5 ed (2022): Anaplastisches Schilddrüsenkarzinom

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Among the most lethal solid tumors historically — IVC by AJCC at diagnosis (any T any N M0/M1 = stage IVA/B/C). Median OS without targeted therapy 3-6 months; rapid airway compromise common. Time-critical workup: BRAF V600E status drives first-line systemic therapy and must be available within days, not weeks. ATA 2021 guideline mandates rapid IHC (VE1 antibody) + reflex sequencing. Therapy by genotype: - BRAF V600E mutant (~25–40%): dabrafenib + trametinib (ROAR basket trial — ORR ~56%, transformative); FDA approved 2018 for ATC. Often used as neoadjuvant induction → surgery → adjuvant chemoRT. - BRAF wild-type RAS / NTRK / RET targetable: selpercatinib / pralsetinib / larotrectinib / entrectinib per genotype. - BRAF/RAS/NTRK/RET wild-type: chemo-radiation (paclitaxel + RT or docetaxel + doxorubicin + RT); pembrolizumab if PD-L1+ or TMB-H. Tracheostomy decision is patient-/family-driven; some protocols defer to systemic-therapy response over upfront tracheostomy when BRAF-mutant.

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