Key result
Higher PDGFR expression is linked to ~5-month longer progression-free survival on pazopanib in pulmonary intimal sarcoma.
Why the study?
The relationship between PDGFR expression and the response to pazopanib in intimal sarcoma of the pulmonary artery was unclear.
Does the level of PDGFR expression predict the efficacy of pazopanib in patients with intimal sarcoma of the pulmonary artery?
Case Report (n=2)
No
Does the level of PDGFR expression predict the efficacy of pazopanib in patients with intimal sarcoma of the pulmonary artery?
The level of PDGFR expression in tumor tissue may be predictive of pazopanib efficacy in patients with intimal sarcoma of the pulmonary artery.
Pazopanib use in PAIS remains hypothesis-generating; prospective trials needed before any practice consideration.
Intimal sarcoma of the pulmonary artery (PAIS) is a rare disease with a poor prognosis. Pazopanib, which has been indicated in metastatic non-adipocytic soft-tissue sarcomas and is expected to be active in PAIS, is a multi-kinase inhibitor that targets the tyrosine kinase activity of vascular endothelial growth factor receptor (VEGFR), platelet-derived growth factor receptor (PDGFR) and stem cell factor receptor. The present study reports findings related to two cases of PAIS with PDGF and VEGF expression following treatment with pazopanib. A case with a moderate to strong expression of PDGFR-α and -β presented a long-term stable disease when treated with pazopanib (progression-free survival, 5.8 months). In a second case with a weak expression of PDGFR-α and -β, the disease progressed rapidly on pazopanib (progression-free survival, 1.1 months). VEGFR-2 was not expressed in the tumors of both cases. The level of PDGFR expression in the tumor tissue may therefore be predictive of pazopanib efficacy.
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Sai et al. (2020) conducted a case report in Intimal sarcoma of the pulmonary artery (PAIS) (n=2). Pazopanib was evaluated on Progression-free survival. In two patients with intimal sarcoma of the pulmonary artery, moderate to strong PDGFR expression yielded a 5.8-month progression-free survival on pazopanib, versus 1.1 months with weak expression.
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