GLI1-altered Epithelioid Soft Tissue Tumor: A Newly Described Entity with a Predilection for the Tongue

A novel subset of epithelioid soft tissue tumors with GLI1 gene fusions or amplifications has recently been characterized. Histologically, these tumors typically exhibit multinodular or plexiform growth of ovoid, round to epithelioid neoplastic cells, with a distinctive nested architecture separated by a rich delicate arborizing vascular network. The immunoprofile is variable and nonspecific, indicating no definitive line of differentiation. GLI1-altered epithelioid soft tissue tumor is considered to be a low-grade sarcoma; however, with a limited number of cases reported, the biologic behavior of this entity is unclear. Interestingly, these tumors frequently occur in the head and neck, with a clear predilection for the tongue. To date, 11 cases of lingual GLI1-altered epithelioid soft tissue tumors have been reported in the English literature, and none showed evidence of recurrence or metastasis. Herein, we report an additional case of lingual GLI1-altered epithelioid soft tissue tumor harboring GLI1 fusion in a 56-year-old man with regional nodal metastasis and distant metastasis to increase the awareness of this entity among oral pathologists and clinicians. An accumulation of similar cases is mandatory to clarify biological behaviors and also for the development of new therapeutic strategies.