Pulmonary Artery Intimal Sarcoma: A Deadly Diagnosis in Disguise

Abstract Pulmonary Artery Intimal Sarcoma (PAIS) is a very rare tumour. The prevalence of PAIS is estimated between 0.001 and 0.003%, but this may be an underestimation because of potential misdiagnosis due to its similar presentation to pulmonary thromboembolism (PTE). The prognosis is very poor, with median overall survival between 11-18 months. We report a case of a 36-year-old gentleman who presents to our Cardiac Surgery Clinic reporting non-specific symptoms and was found to have PAIS requiring surgical resection and adjuvant chemotherapy. We outline the radiologic features, pathologic characteristics, surgical approach and chemotherapy treatment utilized.