Published online Dec 7, 2025. doi: 10.3748/wjg.v31.i45.113690
Revised: September 27, 2025
Accepted: October 22, 2025
Published online: December 7, 2025
Processing time: 93 Days and 20.4 Hours
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, predominantly arising in the stomach (approximately 60%) and small intestine (approximately 30%), and accounting for 1%-3% of all gastrointestinal malignancies. In most cases, GISTs originate within the gastrointestinal tract; However, in rare instances, they may develop in extra-GISTs (EGISTs). Among these, gallbladder-derived EGISTs are exceedingly uncommon, with only nine cases reported to date.
We present the case of a 66-year-old woman who presented with recurrent right upper quadrant abdominal pain and subsequently underwent cholecystectomy. Histopathological examination with immunohistochemistry revealed CD117(+), DOG-1(+), and CD34(+), with no evidence of a primary gastrointestinal lesion, thereby confirming the diagnosis of primary gallbladder EGIST. According to the 2017 Chinese consensus on GISTs, based on the modified NIH 2008 criteria, the tumor was classified as very low risk. Consequently, the patient did not receive adjuvant targeted therapy such as imatinib, and the patient remained disease-free during a 6-month follow up.
Primary gallbladder EGISTs are exceedingly rare, with insidious onset and nonspecific clinical manifestations. Histopathological examination combined with immunohistochemistry remains the cornerstone of definitive diagnosis.
Core Tip: Primary extra-gastrointestinal stromal tumor (EGIST) of the gallbladder is exceptionally rare, with only a handful of cases reported worldwide. We describe a 66-year-old female patient diagnosed with gallbladder EGIST, confirmed by immunohistochemical staining for CD117(+), DOG-1(+), and CD34(+). The tumor was classified as very low risk based on the modified NIH criteria and Chinese consensus guidelines, and the patient remained recurrence-free at follow-up. This case underscores the diagnostic challenges and clinical significance of gallbladder EGISTs, highlighting the importance of integrating imaging, pathology, and risk stratification for optimal management.