Transient Response of Olaparib on Pulmonary Artery Sarcoma Harboring Multiple Homologous Recombinant Repair Gene Alterations.
Chiao-En WuCa Tung NgKien Thiam TanPublished in: Journal of personalized medicine (2021)
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).
Keyphrases
- pulmonary artery
- dna repair
- dna damage
- pulmonary embolism
- coronary artery
- pulmonary hypertension
- genome wide
- pulmonary arterial hypertension
- copy number
- induced apoptosis
- inferior vena cava
- oxidative stress
- dna damage response
- stem cells
- dna methylation
- cell cycle arrest
- bone marrow
- palliative care
- single cell
- endoplasmic reticulum stress
- locally advanced
- cell death
- combination therapy
- genome wide analysis