Onco Wiki / Disease

Gastrointestinal stromal tumor (GIST)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

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

Source YAML

Capabilities

Notes

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...

Used By

Actionability

• BMA-KIT-D816V-GIST - KIT D816V in GIST is rare as primary mutation (more typical of systemic mastocytosis) but...
• 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 - Secondary KIT mutations after imatinib failure cluster in exon 13/14 (ATP-binding pocket...
• 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 is rare and confined to KIT/PDGFRA wild-type GIST (which is itself ~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 mutations in GIST are very rare and are imatinib- sensitive. Treatment is...
• BMA-PDGFRA-EXON18-NON-D842-GIST - PDGFRA non-D842V exon 18 mutations in GIST are imatinib-SENSITIVE (distinct from D842V)....

Algorithms

• ALGO-GIST-1L - ALGO-GIST-1L
• ALGO-GIST-2L - ALGO-GIST-2L
• ALGO-STS-ADVANCED-1L - ALGO-STS-ADVANCED-1L

Disease

• DIS-PARAGANGLIOMA - Paraganglioma (PGL)

Indication

• IND-GIST-1L-AVAPRITINIB-PDGFRA-D842V - IND-GIST-1L-AVAPRITINIB-PDGFRA-D842V
• IND-GIST-1L-IMATINIB - IND-GIST-1L-IMATINIB
• IND-GIST-2L-SUNITINIB - IND-GIST-2L-SUNITINIB
• IND-GIST-4L-RIPRETINIB - IND-GIST-4L-RIPRETINIB

Questionnaires

• QUEST-GIST-1L-STUB - Gastrointestinal stromal tumor — first line

Red flag

• RF-GIST-FRAILTY-AGE - Age ≥75 with ECOG ≥2 or ≥2 comorbidities — high-dose imatinib 800 mg (KIT exon 9) and TKI...
• RF-GIST-HIGH-RISK-BIOLOGY - Adverse genotype or anatomic risk in GIST: PDGFRA D842V (imatinib-resistant — avapritinib...
• RF-GIST-INFECTION-SCREENING - Active or latent infection requiring resolution / prophylaxis before initiating imatinib...
• RF-GIST-ORGAN-DYSFUNCTION - Baseline organ dysfunction precluding standard imatinib / sunitinib / regorafenib / avapr...
• RF-GIST-PDGFRA-D842V - PDGFRA D842V substitution mutation in advanced or metastatic GIST. The PDGFRA D842V varia...
• RF-GIST-TRANSFORMATION-PROGRESSION - Radiographic progression on imatinib (RECIST progression or new lesion), focal nodule-wit...

Sources

• SRC-NCCN-GIST-2025 - NCCN Clinical Practice Guidelines — Gastrointestinal Stromal Tumors (GIST)