GLI1-altered mesenchymal tumors are a relatively rare type of mesenchymal tumor that is common in the head and neck. It is a newly added tumor type in the World Health Organization Tumor Classification of Head and Neck (5th edition). This article reports a case ofGLI1alteredmesenchymal tumor occurring at the left tongue and reviews the relevant literature to summarize the pathological morphological characteristics, immunophenotype, molecular changes, clinical manifestations, and prognosis of the disease.
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| http://dx.doi.org/10.7518/hxkq.2024.2024098 | DOI Listing |
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11669921 | PMC |
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Glioma-associated oncogene (-altered mesenchymal tumors are a newly described entity of neoplasms with very few case reports published in the literature. -altered neoplasms have a moderate degree of variability as they are seen in a broad range of anatomic sites and amongst people of all ages. A common feature that most -altered tumors share is the histologic makeup of monomorphic ovoid cells organized in distinct nests and an arborizing vascular blood supply.
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Pathogenic alterations, namely, fusions and amplifications, of the GLI1 gene have been identified in various mesenchymal tumors, including pericytoma with t(7;12), plexiform fibromyxoma, gastroblastoma, and other malignant mesenchymal neoplasms arising in the soft tissues, as well as in various visceral organs. However, only three cases of GLI1-rearranged renal tumors have been reported to date, comprising two low-grade spindle cell tumors with GLI1::FOXO4 fusion along with one GLI1-rearranged case with an unknown fusion partner. In this study, we analyzed three cases with GLI1::FOXO4 fusion and overlapping morphology.
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GLI1-altered mesenchymal tumors are a relatively rare type of mesenchymal tumor that is common in the head and neck. It is a newly added tumor type in the World Health Organization Tumor Classification of Head and Neck (5th edition). This article reports a case ofGLI1alteredmesenchymal tumor occurring at the left tongue and reviews the relevant literature to summarize the pathological morphological characteristics, immunophenotype, molecular changes, clinical manifestations, and prognosis of the disease.
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GLI1-altered tumors form a diverse group occurring in various anatomic locations. In the alimentary tract, the most established are gastroblastoma, a biphasic epithelial-mesenchymal neoplasm of the stomach, and plexiform fibromyxoma, a pure spindle cell neoplasm. The spectrum of GLI1-rearranged gastrointestinal tumors has recently expanded with reports of cases in other parts of the GI tract, some exhibiting gastroblastoma-like features and others being pure mesenchymal neoplasms.
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GLI1-altered mesenchymal tumors have recently emerged as a distinctive group of neoplasms characterized by GLI1 fusions or amplifications. Although there is clearly metastatic potential, the clinicopathologic features predicting for metastasis are currently unknown. Herein, we present 6 cases of GLI1-altered mesenchymal tumors with multiomics analysis.
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