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Pleural‐Based GLI1 ‐Altered Mesenchymal Tumor With ACTB‐GLI1 Fusion: A Rare Pulmonary Entity Mimicking Synovial Sarcoma

Year: 2026

Type: article

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.

Source: Thoracic Cancer

DOI: 10.1111/1759-7714.70271

Authors: Marco Lizwan, Li Xiao, Chun Yuen Chow, Hiu Yeung Lau, Boon‐Hean Ong

Institutions: National Heart Centre Singapore, Singapore General Hospital, KK Women's and Children's Hospital

Language: English

Topic: Sarcoma Diagnosis and Treatment

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,030