Cytoreductive Surgery in Patients With Metastatic Succinate Dehydrogenase-Deficient Gastrointestinal Stromal Tumors.
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BACKGROUND AND OBJECTIVES: Succinate dehydrogenase-deficient (SDH-deficient) gastrointestinal stromal tumors (GIST) are characterized by variable disease biology with poor responses to traditional tyrosine kinase inhibitors. The role of surgical intervention has been highly debated. METHODS: We performed a single-institution retrospective analysis of metastatic SDH-deficient GIST patients who underwent complete (CC-0) cytoreductive surgery (CRS) from 2017 to 2023. Pathologic-Peritoneal Cancer Index (P-PCI) and GIST Metastatectomy-Surgical Complexity Score (GM-SCS) were used to quantify complexity. Kaplan-Meier survival analysis compared time to progression on systemic therapy immediately before CRS (TTP-1L) and second to last line of systemic therapy before CRS (TTP-2L) versus time to recurrence (TTR) after CRS. RESULTS: Nine patients met inclusion criteria. The median age at CRS was 29 years (range: 17-43). Median P-PCI was 13 (IQR: 10-15) and median GM-SCS was 19 (IQR: 16-28). Clavien-Dindo grade ≥ 3 complication rate was 33.3% (3/9) within 90-days postoperatively. Median TTP-1L was unreached and median TTP-2L was 3.4 months (95% CI: 2.5-unreached), as compared to TTR at 26.1 months (95% CI: 15.2-unreached) post CRS (p ≤ 0.0001). CONCLUSIONS: Patients who underwent CC-0 CRS achieved prolonged disease control versus prior systemic therapies, suggesting that CRS may be a management option for highly selected SDH-deficient GIST patients.