GLI1-Altered Mesenchymal Tumors.

Jeffrey M Cloutier, Darcy A Kerr

Journal: Surgical pathology clinics 2024;17(1):13-24

PMID: 38278602

Full Text on Elsevier Science View on PubMed

Abstract

GLI1-altered mesenchymal tumors comprise an emerging group of neoplasms characterized by fusions or amplifications involving GLI1, a gene that encodes a key regulator of the Hedgehog signaling pathway. In recent years, tumors with GLI1 alterations have been reported across a variety of anatomic sites and a broad age range. Although these tumors can exhibit a wide morphologic spectrum and a variable immunophenotype, they frequently present with monomorphic ovoid cells arranged in distinctive nests with a rich, arborizing vascular network. Recent evidence indicates that they have the potential to metastasize, which suggests that they may be best considered a sarcoma.

Address: Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756, USA; Dartmouth Geisel School of Medicine, 1 Rope Ferry Road, Hanover, NH 03755, USA. Electronic address: https://twitter.com/@JCloutierMD.; Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH 03756, USA; Dartmouth Geisel School of Medicine, 1 Rope Ferry Road, Hanover, NH 03755, USA. Electronic address: [email protected].

DOI: 10.1016/j.path.2023.06.004

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MeSH Terms: Humans, Zinc Finger Protein GLI1, Hedgehog Proteins, Sarcoma, Soft Tissue Neoplasms