Purpose: To report an uncommon case of orbital gastrointestinal stromal tumor (GIST) metastasis. Material and Methods: Observational case report. Results: A 65-year-old woman with metastatic GIST involving the left orbit with a history of two separate GIST nodules involving the stomach 6 years earlier. Computed tomography (CT) scan demonstrated a well-circumscribed enhancing lesion confined to the anterior orbit. Histopathology analysis of the tumor showed predominantly spindle cells with focal epithelioid forms. It also stained positive for c-KIT (CD117) on immunochemistry, confirming the diagnosis. Additional medical treatment was not required, and the patient was followed up regularly for disease recurrence. Conclusion: GISTs typically occur as sporadic solitary tumors. In malignant cases, it usually metastasizes to the liver or other intraabdominal sites. Orbital involvement is extremely rare. This is the first case of metastatic GIST involving the anterior orbit with histopathological and immunochemical confirmation.
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