ArticleThoracic cancer2026
Pleural-Based GLI1-Altered Mesenchymal Tumor With ACTB-GLI1 Fusion: A Rare Pulmonary Entity Mimicking Synovial Sarcoma.
Article in Thoracic cancer, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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5 authors.
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Abstract
GLI1-altered mesenchymal tumors are exceptionally rare mesenchymal tumors, with recent molecular studies implicating GLI1 gene fusion or amplification. We report a 39-year-old nonsmoking woman who presented with an incidentally detected right pleural-based mass. Cross-sectional imaging identified a paraspinal pleural lesion at the T5-T6 level without intraspinal extension. Video-assisted thoracoscopic resection revealed a biphasic tumor and next-generation sequencing confirmed an ACTB-GLI1 fusion. The diagnosis of GLI1-altered mesenchymal tumor was established. After multidisciplinary discussion, the patient opted for radiologic surveillance instead of adjuvant therapy. She remains disease-free at 1-year follow-up. This case expands the anatomic spectrum of GLI1-altered tumors and underscores the value of molecular testing in distinguishing them from other pleural neoplasms.
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