Complete resection of a pulmonary artery sarcoma involving the pulmonary valve and right ventricle outflow tract: a case report

BACKGROUND: Pulmonary artery sarcoma (PAS) is an extremely rare tumour, preferably treated by surgery. However, the surgical management remains largely debatable, as only less than half of patients with PAS can undergo thorough excision. CASE SUMMARY: A 32-year-old man with a tumour involving the right ventricle outflow tract, pulmonary trunk extending into the bifurcation, and right pulmonary arteries underwent complete resection using a homologous pulmonary valve and vascular grafts for reconstruction, combined with right pulmonary endarterectomy (PEA) for potential seeding metastasis. Histopathological examination demonstrated undifferentiated pleomorphic sarcoma with surgical margins free of disease. The patient remains asymptomatic, and follow-up computed tomography 5 months after surgery indicated no recurrence or metastasis. DISCUSSION: Radical resection of a PAS with reconstruction using pulmonary valve allograft and polytetrafluoroethylene vascular grafts is technically feasible and successful. Additionally, PEA may eliminate the potential intima implantation metastasis.