Primary intimal pulmonary artery sarcoma: A diagnostic challenge

Abstract

Primary pulmonary artery sarcoma (PAS), which was first described by Mandelstamm in 1923,1 is a rare entity that is often misdiagnosed as chronic pulmonary thromboembolism.2 The differentiation of these 2 diseases with similar clinical presentations is fundamental for the initiation of adequate therapy, which completely differs between the 2 conditions.3 PAS often has a fatal outcome because of insidious intravascular tumor growth with extension into the distal pulmonary artery system. Surgery and adjacent irradiation or chemotherapy are necessary to optimize the survival time that ranges between 14 and 18 months.