Abstract
Osteosarcoma is the most common primary tumor of the bones affecting mainly young adults. Despite the advances in the field of systemic anticancer therapy, the prognosis of relapsed of metastatic osteosarcoma patients remain dismal with very short survival. However, the better understanding of the pathophysiology of this subtype of sarcoma has led to the identification of new targeted agents with significant activity. In fact, increased angiogenesis plays a major role in the tumor growth and survival of osteosarcoma patients. Several targeted agents have demonstrated a significant anti-tumor activity including multi-kinase inhibitors. In this review, we will discuss the pathophysiology, rationale, and role of targeting angiogenesis via the VEGF pathway in patients with osteosarcoma with emphasis on the published clinical trials and future directions.
Highlights
High-grade malignant osteosarcoma is a rare tumor with a worldwide incidence of3–4 cases per million [1]
This review summarizes the current clinical evidence and future directions in this setting
VEGFR2, the main vascular endothelial growth factor (VEGF)-A receptor involved in angiogenesis and vasculogenesis, and PD-L1, expressed in 64.5% and 35.5% of osteosarcoma cells, respectively, were associated with a pro-metastatic effect in the lungs, and tumor growth [30]
Summary
3–4 cases per million [1] It is the most common primary tumor of the bones, affecting mainly young adults with a peak incidence in the second decade of their life [1]. These tumors are characterized by the presence of malignant mesenchymal cells and increased osteoid production. Despite high PD-L1 expression levels (ranging from 14 to 75%) and promising results in preclinical models [19,20], immune checkpoint inhibitors in unselected patients with relapsed osteosarcoma have shown very limited clinical activity, with an overall response rate (ORR) of
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