Key result
Alveolar soft part sarcomas are gene fusion-driven sarcomas characterized by the ASPSCR1::TFE3 gene fusion, lacking a normal cellular counterpart.
Why the study?
To provide an updated review highlighting the clinical and pathologic features of alveolar soft part sarcoma following the consensus that it represents a gene fusion-driven sarcoma.
Provides an updated overview of the clinical and pathologic features of alveolar soft part sarcoma, a gene fusion-driven neoplasm.
Recognizing ASPSCR1::TFE3 fusion aids alveolar soft part sarcoma diagnosis; leaves open targeted therapy development for this rare sarcoma.
In 1952, Christopherson and colleagues described the distinctive clinical and pathologic features of alveolar soft part sarcoma (ASPS). For nearly half a century, controversy raged regarding the putative cell of origin of this peculiar neoplasm. Following the identification of the characteristic der(17)t(X;17)(p11;q25) translocation and discovery of the resulting ASPSCR1::TFE3 gene fusion in 2001, the current consensus is that alveolar soft part sarcomas represent one of several gene fusion-driven sarcomas which lack a normal cellular counterpart. This updated review highlights the clinical and pathologic features of this intriguing neoplasm.
No takes yet. Share an insight, caveat, or question.
Argani et al. (2025) conducted a review in Alveolar soft part sarcoma (ASPS). Alveolar soft part sarcomas are gene fusion-driven sarcomas characterized by the ASPSCR1::TFE3 gene fusion, lacking a normal cellular counterpart.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: