Transient Response of Olaparib on Pulmonary Artery Sarcoma Harboring Multiple Homologous Recombinant Repair Gene Alterations

• Main Authors: Chiao-En Wu, Ca Tung Ng, Kien Thiam Tan
• Format: Article
• Language: English
• Published: MDPI AG 2021-04-01
• Series: Journal of Personalized Medicine
• Subjects: PARP inhibitor; olaparib; next-generation sequencing (NGS); comprehensive genetic profiling (CGP); primary pulmonary artery sarcoma (PPAS); homologous recombination repair (HRR)
• Online Access: https://www.mdpi.com/2075-4426/11/5/357

Primary pulmonary artery sarcoma (PPAS) is a rare malignancy arising from mesenchymal pulmonary artery cells and mimics pulmonary embolism. Palliative chemotherapy such as anthracycline- or ifosfamide-based regimens and targeted therapy are the only options. However, the evidence of clinically beneficial systemic treatment is scarce. Here, we report a case of disseminated PPAS achieving clinical tumor response to olaparib based on comprehensive genetic profiling (CGP) showing genetic alterations involving DNA repair pathway. This provides supportive evidence that olaparib could be a promising therapeutic agent for patients with disseminated PPAS harboring actionable haploinsufficiency of DNA damage repair (DDR).