Gastrointestinal-Struma-Tumor (GIST)
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | DIS-GIST |
|---|---|
| Art | Erkrankung |
| Aliase | GISTGastrointestinaler StromatumorICC-TumorГастроінтестинальна стромальна пухлина (GIST) |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Erkrankungen | None declared |
| Quellen | SRC-NCCN-MELANOMA-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8936/3 |
|---|---|
| icd_o_3_topography | ["C15", "C16", "C17", "C18", "C19", "C20", "C26"] |
| icd_10 | C49 |
| who_classification | WHO Weichgewebetumore 5 ed (2020): Gastrointestinal-Strom-Tumor |
Anmerkungen
Genotype-driven therapy is the defining feature of GIST. Decision tree: - KIT exon 11 mutant (~50%): imatinib 400 mg/day; best prognosis; after imatinib failure → sunitinib → regorafenib → ripretinib. - KIT exon 9 mutant (~10%, predominantly small-bowel): imatinib 800 mg/day from start (B2222 / EORTC-62005 dose effect); shorter PFS than exon 11. - PDGFRA D842V (~7%, gastric): imatinib RESISTANT — avapritinib 1L (NAVIGATOR / VOYAGER); ORR ~88% historical. - PDGFRA non-D842V exon 18 / exon 12: imatinib-sensitive. - KIT/PDGFRA wild-type SDH-deficient (~5–10%, gastric, young patients): refractory to imatinib; sunitinib / regorafenib partial; consider clinical trial. Carney triad and Carney-Stratakis fall here. - NF1-mutant (small bowel, multifocal): refractory to TKIs; surgery + MEK inhibitor (selumetinib) being studied. Risk stratification (Joensuu / NIH consensus / AFIP) on size, mitotic rate, primary site, rupture → 3 yr adjuvant imatinib (≥3 cm + mitoses ≥5/50 HPF, or rupture, or non-gastric primary) is standard; 6 yr extended in highest-risk per PERSIST-5 / SSG-XXII follow-up. Resistance: secondary KIT mutations in exons 13 / 14 (ATP-binding) or 17 / 18 (activation loop) emerge d...
Verwendet von
Therapeutische Relevanz
BMA-KIT-D816V-GIST- KIT D816V in GIST ist selten als primäre Mutation (typischer für systemische Mastozytose) aber...BMA-KIT-EXON11-GIST- KIT exon 11 mutation in GIST: imatinib 400 mg/day is standard 1L (B2222, Demetri NEJM 200...BMA-KIT-EXON13-17-GIST- Sekundäre KIT-Mutationen nach Imatinib-Ausfall-Cluster in exon 13/14 (ATP-bindende Tasche...BMA-KIT-EXON9-GIST- KIT exon 9 GIST has shorter PFS on imatinib 400 mg vs exon 11. Imatinib 800 mg/day (split...BMA-NTRK-FUSION-GIST- NTRK Fusion in GIST ist selten und beschränkt sich auf KIT/PDGFRA Wildtyp GIST (das ist selbst ~1...BMA-PDGFRA-D842V-GIST- PDGFRA D842V in GIST is imatinib-RESISTANT (distinct from imatinib-sensitive PDGFRA non-D...BMA-PDGFRA-EXON12-GIST- PDGFRA exon 12 mutations in GIST are imatinib-sensitive. Imatinib 400 mg/day is standard...BMA-PDGFRA-EXON14-GIST- PDGFRA exon 14 Mutationen in GIST sind sehr selten und sind Imatinib-sensibel. Behandlung ist...BMA-PDGFRA-EXON18-NON-D842-GIST- PDGFRA non-D842V exon 18 mutations in GIST are imatinib-SENSITIVE (distinct from D842V)....
Algorithmen
ALGO-GIST-1L- ALGO-GIST-1LALGO-GIST-2L- ALGO-GIST-2LALGO-STS-ADVANCED-1L- ALGO-STS-ADVANCED-1L
Erkrankung
DIS-PARAGANGLIOMA- Paraganglioma (PGL)
Anwendungsgebiet
IND-GIST-1L-AVAPRITINIB-PDGFRA-D842V- IND-GIST-1L-AVAPRITINIB-PDGFRA-D842VIND-GIST-1L-IMATINIB- IND-GIST-1L-IMATINIBIND-GIST-2L-SUNITINIB- IND-GIST-2L-SUNITINIBIND-GIST-4L-RIPRETINIB- IND-GIST-4L-RIPRETINIB
Fragebogen
QUEST-GIST-1L-STUB- Gastrointestinalstromatumor — erste Zeile
Warnhinweis
RF-GIST-FRAILTY-AGE- Alter ≥ 75 mit ECOG ≥ 2 oder ≥ 2 Komorbiditäten — hohe Dosis Imatinib 800 mg (KIT exon 9) und TKI...RF-GIST-HIGH-RISK-BIOLOGY- Unerwünschtes Genotyp- oder anatomisches Risiko bei GIST: PDGFRA D842V (imatinib-resistent — Avapritinib...RF-GIST-INFECTION-SCREENING- Aktive oder latente Infektion, die eine Auflösung / Prophylaxe vor Beginn der Imatinib...RF-GIST-ORGAN-DYSFUNCTION- Ausgangsorgan Dysfunktion, die Standard Imatinib / Sunitinib / Regorafenib / Avapr...RF-GIST-PDGFRA-D842V- PDGFRA D842V Substitutionsmutation in fortgeschrittenem oder metastasiertem GIST. Die PDGFRA D842V Varia...RF-GIST-TRANSFORMATION-PROGRESSION- Radiographische Progression bei Imatinib (RECIST Progression oder neue Läsion), fook...
Quellen
SRC-NCCN-GIST-2025- NCCN-Leitlinien für klinische Praxis — Gastrointestinale Stromatumore (GIST)