Gastrointestinal Stromal Tumor

Despite overlapping pathological features, uterine and gastrointestinal stromal tumors differ clinically. Surgery is the primary treatment; comprehensive preoperative imaging and postoperative pathological examination are critical to prevent misdiagnosis and optimize management.

P3, N=482, Recruiting, Cogent Biosciences, Inc. | Active, not recruiting --> Recruiting

The patient recovered uneventfully with resolution of bleeding and normalization of hemoglobin. This case highlights the importance of considering small bowel GIST in unexplained melena and severe anemia and demonstrates the curative potential of timely surgical resection in localized low-risk disease.

P=N/A, N=750, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Jan 2026 --> Jan 2028 | Trial primary completion date: Jan 2026 --> Jan 2028

GALNT7-mediated O-GalNAc glycosylation stabilizes KIT and drives GIST progression. GALNT7 may serve as a prognostic biomarker and therapeutic target in GIST.

This case highlights an important diagnostic pitfall in which a giant exophytic gastric GIST may mimic ovarian cancer because of its pelvic location and cystic-solid appearance. Careful correlation of CECT, fused [18F]FDG PET/CT, and pathologic findings is essential for accurate assessment of the organ of origin in large abdominopelvic masses.

In a genetically engineered murine GIST model, PD-L1 blockade enhanced imatinib efficacy and restored tumor γδ T cell function. Thus, γδ T cells contribute to tumor immunity in GIST and are reprogrammed during imatinib resistance, making them an attractive immunotherapy target.

This review provides a practical, pathology-focused overview of SDH-deficient tumor syndromes, emphasizing the clinicopathologic spectrum of paraganglioma-pheochromocytoma syndromes (PGL1-5), SDH-deficient gastrointestinal stromal tumor, SDH-deficient renal cell carcinoma, Carney-Stratakis syndrome, and Carney triad. We highlight recurring morphologic cues across tumor types, outline a stepwise workflow integrating histology, SDHB and SDHA immunohistochemistry and targeted molecular testing, and discuss interpretive pitfalls.

Lastly, our biochemical data were validated using imatinib response data for first-line metastatic disease; patients with predicted exon 18 sensitive mutations had longer progression-free survival than patients with predicted imatinib-resistant mutations. These results provide key evidence that should be used to guide therapy selection for PDGFRA-mutant GIST.

Esophageal GISTs are exceptionally rare, and those driven by BRAF gene fusions are even more uncommon. This report describes a 69-year-old woman who underwent surgical resection of an esophageal GIST harboring an MKRN1-BRAF gene fusion, offering insight into future treatment and surveillance strategies.

P2/3, N=255, Recruiting, Ningbo Newbay Technology Development Co., Ltd | Not yet recruiting --> Recruiting

Adjuvant tyrosine kinase inhibitors, such as imatinib, are reserved for high-risk lesions or unresectable tumors. This case highlights the importance of considering GIST as a differential diagnosis in obscure upper GI bleeding. It also demonstrates that laparoscopic resection is a safe and effective treatment option for ileal GISTs, even in the context of acute bleeding.