| Summary: | GLI1-altered tumors were initially reported as pericytomas with t (7; 12) and ACTB-GLI1 fusion. These rare tumors stem from alterations in perivascular myoid cell differentiation and were first reported as a group of rare soft-tissue neoplasms of pericytic origin. Several series of GLI1-altered tumors have since been described. In 2024, the World Health Organization recognized these neoplasms as rare soft-tissue tumors of the head and neck in the tumor classification. They have been recently reported in multiple organs but rarely in the gastrointestinal tract. Most of these tumors are classified into low-grade malignancies; however, several malignant cases have been described. The present case report presents a gastric tumor with ACTB-GLI1 fusion in a 40-year-old male who completed a long-term follow-up. The tumor histology exhibited uniform polygonal cells with clear to slightly eosinophilic cytoplasm arranged in an organoid pattern. Immunohistochemical studies of the tumor cells revealed nonspecific finding and a definite diagnosis of ACTB-GLI1 fusion tumor was confirmed through fluorescence in situ hybridization. This report conducted a literature review and discussed the differential diagnosis, malignant potential, and clinical expression and treatment of these tumors.
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