Alveolar Soft Part Sarcoma: An Updated Review

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.