Gastrointestinal Stromal Tumor

Since the introduction of imatinib in the early 21st century, the management of metastatic GIST has shifted from solely surgical intervention to a systemic, chronic disease management model centered on tyrosine kinase inhibitors (TKIs)...These challenges underscore the necessity of this review, which discusses current standard drug treatment strategies for advanced GIST, including sequential TKIs therapy and investigations into mechanisms of drug resistance. Finally, the review explores precise and actionable future directions for GIST drug development and clinical management, including mutation-stratified therapeutic sequencing, rational TKI-based combination regimens, and circulating tumor DNA (ctDNA)-guided real-time treatment monitoring and resistance surveillance.

P2, N=40, Completed, Asan Medical Center | Recruiting --> Completed

P3, N=450, Recruiting, GlaxoSmithKline

Treatment with D1 LND is recommended for gastric neuroendocrine carcinoma (g-NEC), g-NET with lymphadenopathy, and grade 3 disease. Lymphadenectomy generally is unnecessary for g-GIST, except for those with persistent lymphadenopathy after neoadjuvant therapy or uncommon mutations.

P1/2, N=48, Recruiting, Asan Medical Center | Not yet recruiting --> Recruiting

P2, N=322, Recruiting, Merck Sharp & Dohme LLC | N=171 --> 322

Histologically, given its morphological diversity, it needs to be differentiated from spindle cell tumors such as pancreatic gastrointestinal stromal tumors, fibromatosis, inflammatory myofibroblastic tumors, and pancreatic sclerosing epithelioid fibrosarcoma. Nuclear expression of STAT6 and the presence of NAB2: : STAT6 fusion are important characteristics of SFT.

Histopathology consistent with low-grade combined type GIST with immunochemistry positive for CD117 (c-KIT). This case highlights an unusual presentation of small bowel bleeding originating from an aberrant artery in continuity with a hypervascular jejunal GIST.

The cumulative risk score based on m6A pathway variants showed a strong association with PFS. These findings provide preliminary, hypothesis-generating evidence that genetic variations may contribute to inter-patient variability in outcomes and warrant further investigation as potential biomarkers in IM-treated GIST.

This case expands the morphologic and immunophenotypic spectrum of PFM and indicates the possible diagnostic utility and biological significance of D2-40 expression. Although molecular confirmation of MALAT1::GLI1 fusion is definitive for the diagnosis of PFM, the findings of the present case may aid diagnosis in challenging cases that mimic GIST.

P1, N=18, Recruiting, University of California, San Diego | Not yet recruiting --> Recruiting | Initiation date: Dec 2025 --> Jun 2026