Pulmonary artery intimal sarcoma: A case series

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare condition with a historically poor prognosis. Diagnosis is often delayed due to clinical and radiological features which are easily mistaken for pulmonary embolism. Despite the commonality of clinical and macroscopic features across cases, microscopic features and immunophenotype are highly variable. Similarly, while cases of PAIS have been shown to carry genetic alterations including MDM2, CDK4, PDGFRA or EGFR amplification, their presence is not necessary for diagnosis.