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NCCN Flash Updates: NCCN Guidelines Updated for Gastrointestinal Stromal Tumors
NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), the NCCN Drugs & Biologics Compendium (NCCN Compendium®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™) for Gastrointestinal Stromal Tumors. These NCCN Guidelines® are currently available as Version 1.2025.
Link directly to the Updates section of the NCCN Guidelines: Gastrointestinal Stromal Tumors
GIST-1
Workup at Primary Presentation
- Deleted: Consider chest imaging from lower pathway.
- Column 2, modified: No high-risk EUS or biopsy features.
Footnotes:
- Deleted, h: Some patients may rapidly become unresectable; close monitoring is essential. (Also for GIST-2).
- New: Consider baseline chest imaging.
- Modified, m: General Principles of Surgery (GIST-C)
and Principles of Interventional Oncology (GIST-D). (Also for GIST-2, GIST-4, GIST-5). - Deleted: Neoadjuvant therapy for genotype-sensitive disease may prohibit accurate assessment of recurrence risk following resection (GIST-A). Testing tumor for mutation is recommended prior to starting preoperative therapy to ensure tumor has a genotype that is likely to respond to treatment (GIST-2). Consider neoadjuvant therapy only if surgical morbidity could be reduced by downsizing the tumor preoperatively (GIST-E). Maximal response may require treatment for 6 months or more to achieve. Once maximal response is achieved, consider surgical resection.
GIST-2
Neoadjuvant Therapy
- Column 3, last sentence, modified: Forgo neoadjuvant therapy if other mutations(other than listed above)
Footnotes:
- p, modified: Maximal response may require treatment for 6 months or more to achieve.
Once maximal response is achieved, consider surgical resection. - q, modified to include: FDG-PET/CT may give indication of TKI
imatinibefficacy after 2–4 weeks of therapy when rapid readout of activity is necessary. (Also for GIST-4). - t, deleted last sentence:
Maximal response may require treatment for 6 months or more to achieve.
GIST-3
Postoperative Outcomes
- Column 1, row 3, modified: Completely resected after neoadjuvant therapy with agent other than imatinib
avapritinib, larotrectinib, entrectinib, sunitinib, or dabrafenib trametinib - Row 4, modified: Gross residual disease (R2 resection) or
andPreoperative/intraoperative tumor rupture
Adjuvant Treatment
- Completely resected (no neoadjuvant therapy), modified: Adjuvant imatinib (category 1) preferred for patients with significant risk of recurrence (
intermediatemoderate or high risk if patient has an imatinib-sensitive mutation). (Also for the completely resected after neoadjuvant imatinib arm).
Follow-Up
- Modified to include the following: after 10 y, individualize surveillance
Footnote:
- w, 2nd sentence modified: Available data support the use of adjuvant imatinib for high-risk disease for at least 3 years (based on overall survival benefitand 6 years based on disease-free survival benefit). Last sentence added: Other references include Joensuu H, et al. JAMA Oncol 2020;6:1241-6, and Blay J-Y, et al. Ann Oncol 2024;35:1157-1168).
GIST-4
Footnotes:
- Deleted: Consider resection or ablation/liver-directed therapy for hepatic metastatic disease.
- Deleted: Resection of metastatic disease, especially if complete resection can be achieved, may be beneficial in patients on imatinib or sunitinib who have evidence of radiographic response, or limited disease progression.
GIST-5
Treatment for Progressive Disease
Limited
- Bullet 1, sub-bullet 2 modified: Ablation procedures or embolization, radioembolization, or chemoembolization.
- Bullet 2, sub-bullet 1 modified: Switch to alternate TKI
sunitinib (category 1)
Generalized (widespread, systemic)
- If disease
For performance status (PS) 0–2 and
GIST-A (1 of 3)
Principles of Biopsy and Risk Stratification for GIST
Risk stratification
- Sub-bullet 1, 2nd sentence, modified:
Neoadjuvant therapy that has evidence of pathologic treatment effect will not yield accurate mitotic information.Mitotic rate information might be inaccurate following neoadjuvant therapy, if there is other evidence of pathologic treatment effect (necrosis, reduced cellularity, etc). If risk stratification is performed on a small biopsy or on a resection specimen following neoadjuvant therapy, there is potential for inaccuracies in the mitotic rate.In this situation, risk stratification may need to be based on clinical parameters, size, and anatomic location instead.in the absence of mitotic rate.
GIST A (2 of 3)
- Risk stratification is determined without
anyprior exposure to TKI therapy.
GIST-B
Principles of Mutation Testing
- Bullet 4, 2nd sentence, modified: Most PDGFRA mutations are associated with a response to imatinib, with the exception of D842V, which responds to avapritinib and
whichis unlikely to respond to imatinib and most other approved TKIs for GIST.except for avapritinib - Bullet 10, new: Germline KIT and PDGFRA testing should be considered for the following: patients with a family history of GIST and/or melanoma, patients with multifocal GIST, and/or patients with NF1 or SDH-deficient GIST.
GIST-C
General Principles of Surgery
Considerations Prior to Surgery
- Bullet 2, modified: Patients with
SDH-deficient tumors or known SDH mutationsSDH germline mutations are at risk of paraganglioma;
GIST-E (1 of 4)
Systemic Therapy Agents and Regimens for GIST
- Neoadjuvant therapy, bullet 1, modified to include such as
includingD842V. Also for GIST-E 2 of 4. - Adjuvant therapy, bullet 1, modified: ...significant risk of recurrence,
intermediatemoderate or high risk....
GIST-F
Principles of Imaging
Workup
- Bullet 1, modified: CT abdomen/pelvis with contrast and/or MRI abdomen/pelvis with and without contrast. Also for bullet 2, sub-bullet 1; Response Assessment: bullet 3, sub-bullet 1, bullet 5 sub-bullet 1, bullet 9, sub-bullet 1.
- Bullet 2, sub-bullet 2, modified: Consider baseline chest imaging (x-ray or CT) for unresectable or metastatic disease.
Follow-up
- Bullet 1, modified: For completely resected primary disease, perform CT abdomen/pelvis with contrast and/or MRI abdomen/pelvis with and without contrast every 3–6 months for
3–5 years, then annually. After 10 years, surveillance should be individualized.- Less frequent imaging surveillance may be acceptable for low-risk
or very small gastrictumors(<2 cm). For low-risk tumors, see (GIST-A).
- Less frequent imaging surveillance may be acceptable for low-risk
For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium®), the NCCN Biomarkers Compendium®, the NCCN Chemotherapy Order Templates (NCCN Templates®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.
To view the NCCN Guidelines for Patients®, please visit NCCN.org/patientguidelines.
Free NCCN Guidelines apps for iPhone, iPad, and Android devices are now available! Visit NCCN.org/apps.
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