Why the study?
Angiosarcomas are rare, heterogeneous malignant tumors with poor prognosis, where establishing consistent molecular characteristics and driver events has been difficult and immunological features require further elucidation.
This review provides an overview of critical biological pathways in angiosarcoma and their potential role in novel systemic treatments.
Highlights emerging therapeutic targets for a rare vascular malignancy; leaves open their clinical efficacy until validated in prospective trials.
Angiosarcomas are rare malignant tumors with a heterogeneous clinical presentation and generally poor prognosis. It has been difficult to establish consistent molecular characteristics and driver events in angiosarcoma development. Oncogenic and angiogenesis-related pathways have been investigated pre-clinically and clinically with varying results. A few promising responses to checkpoint inhibitors have been described, but immunological features require further elucidation. With this review we present an overview of the critical biological pathways and processes affected in angiosarcoma, and their potential role in novel, non-cytotoxic, systemic treatments.
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Weidema et al. (2019) studied this question.
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