Alveolar Soft Part Sarcoma is one of the less commonly diagnosed soft tissue sarcoma subtypes, an infrequent subtype within the already rare category of human malignancy of sarcoma. In this article we will summarize the histopathological features, natural history and distinct molecular and biological features that have become increasingly appreciated with newer technologies and precision oncology. We will discuss the contemporary management of this disease as well as emerging treatment options.
Keywords: ASPL-TFE3 chimeric fusion protein; ASPS; Immune checkpoint inhibition; Immunotherapy; Tyrosine kinase inhibitors.
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