Pulmonary artery sarcoma masquerading as pulmonary embolism: an under-recognised entity

Abstract

Pulmonary artery sarcoma is a rare disease with only a handful of cases reported. It is histologically classified as leiomyosarcoma, spindle cell sarcoma, fibrous histiocytoma or undifferentiated sarcoma. The disease is mostly misdiagnosed as pulmonary thromboembolism and carries a grim prognosis with an average survival of only a few months. Misdiagnosis often results in patients being treated inappropriately and diagnosed in later stages of the disease. This delay in diagnosis can be associated with significant mortality in the setting of an already poor prognosis. Early aggressive surgery targeting complete surgical resection is the standard treatment. Chemotherapy and radiation therapy have been tried with variable outcomes. Given the aggressive nature of pulmonary artery sarcoma, regular post-surgery follow-up is indicated.