Pulmonary Artery Sarcoma Overexpressing Platelet-derived Growth Factor Receptor α

Abstract

Pulmonary artery sarcoma is highly malignant and easily metastasizes to the systemic organs. Both the introduction of novel diagnostic procedures and the development of new treatment modalities are required to achieve long-term survival. Several studies have shown that platelet-derived growth factor receptor α (PDGFRα) gene amplification is frequently observed in pulmonary artery sarcoma. PDGFRα is known to be involved in cell proliferation in certain malignancies. PDGFRα may become a potential biological marker in pulmonary artery sarcoma. We report a case in which a diagnosis of pulmonary artery sarcoma overexpressing PDGFRα was made using endovascular catheter biopsy following positron emission tomography with integrated computed tomography (PET/CT) scans.

<p>Whole-exome sequencing insights into pulmonary artery sarcoma mimicking pulmonary embolism: a case report and review</p>

“…Genomic analysis showed that the amplification of MDM2, KIT , and PDFGRA in PAS and patients could benefit from therapies targeting PDGFRA or MDM2 20,21. Several investigators have reported that pulmonary artery sarcoma has MDM2 amplification and PDGFRα overexpression in PAS 16,22. The MDM2-p53 pathway is strongly associated with the tumorigenesis of pulmonary artery intimal sarcomas 23,24.…”

Primary pulmonary artery tumors easily misdiagnosed as pulmonary embolism: A review

“…[ 50 ] It may be an interesting method for patients with inoperable or suspected disease recurrence. [ 5 , 51 ] Due to the rarity of PPATs, there is no clear consensus on the diagnosis of PPATs. However, with the advancement of medical technology, various methods for obtaining tissues are constantly being tried, and there are still disputes on safety and reliability, which requires continuous technical training, and accurate estimation and processing ability for possible risks.…”

“…With the discovery of potentially targetable genetic alterations (MDM2, PDGFRA, and EGFR) [ 5 , 9 , 51 ] in patients with PPAS, it is considered that the MDM2-p53 pathway is closely related to the occurrence of PPAS, [ 52 ] which makes the targeted therapy very attractive in PPATs. In addition, in vitro immunoassays of primary sarcoma tumor cells have shown that both dasatinib and imatinib have strong inhibition effects on tumor cells, most likely due to the inhibition of downstream extracellular signal–regulated kinase 1/2 (ERK1/2) and serine/threonine kinase (AKT) signaling pathways.…”