Relationship between PDGFR expression and the response to pazopanib in intimal sarcoma of the pulmonary artery: A case report

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 endoth...

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Published inMolecular and Clinical Oncology Vol. 14; no. 1; p. 1
Main Authors Sai, Satoshi, Imamura, Yoshinori, Kiyota, Naomi, Jimbo, Naoe, Toyoda, Masanori, Funakoshi, Yohei, Chayahara, Naoko, Hyogo, Yasuko, Takenaka, Kei, Suto, Hirotaka, Minami, Hironobu
Format Journal Article
LanguageEnglish
Published Athens Spandidos Publications 01.01.2021
Spandidos Publications UK Ltd
D.A. Spandidos
Subjects
Angioplasty
Antibodies
Antimitotic agents
Antineoplastic agents
Case reports
Chemotherapy
Development and progression
Inhibitor drugs
Medical imaging
Medical prognosis
Metastasis
Oncology
Pathogenesis
Patients
Platelet-derived growth factor
Prognosis
Pulmonary arteries
Rare diseases
Sarcoma
Stem cells
Targeted cancer therapy
Tumors
Vascular endothelial growth factor
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ISSN2049-9450
2049-9469
DOI10.3892/mco.2020.2168

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Summary: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- a and -[beta] 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-[alpha] and -[beta], 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. Key words: case reports, intimal sarcoma, pazopanib, platelet-derived growth factor receptor, pulmonary artery, vascular endothelial growth factor receptor
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ISSN:2049-9450
2049-9469
DOI:10.3892/mco.2020.2168