PULMONARY ARTERY INTIMAL SARCOMA MIMICKING PULMONARY EMBOLISM: A CASE SERIES

Abstract

Learning Objectives: Pulmonary artery intimal sarcoma (PAS) is a rare lung disease with high mortality. Its clinical presentation is hardly distinguishable from pulmonary embolism(PE), which leads to a significant delay in diagnosis and treatment. It is important to make clinicians awareness of this disease since accurate diagnosis and promptly treatment can be curative. Methods: A 24- year–old woman presented to our institution with the chief complaint of progressive exertion dyspnea, and hemoptysis. Computed tomography of the pulmonary arteries (CTPA) demonstrated large irregular, lobulated filling defect within the main and left pulmonary arteries. PE was diagnosed. Patient was treated with heparin and changed to Coumadin later. Two weeks later, she came back with syncope. CTPA showed progressively of filling defects. Patient was treated with localized thrombolysis without improvement. Subsequently she developed cardiac arrest and passed away. Postmortem examination revealed lobulated mass arising from left side of main PA, with direct extension into left PA at hilum of lung and histology showed fusiform tumor cells in the lumen of the PA. Findings consistent with PAS. Results: PAS is very rare. PAS can be confused with PE due to the similarity of the signs and symptoms, as well as to image findings. Symptoms are commonly accompanied with dyspnea, chest pain, cough and hemoptysis which resemble PE. The diagnosis is often delayed and very rarely, that the correct diagnosis is made preoperatively. Most patients receive inappropriate therapy such as prolonged anticoagulation or thrombolysis. The CTPA of PAS tends to present as a unilateral, central, lobulated, pulmonary artery filling defect with an increase in the diameter of the PA but it is not always possible to differentiate therefore another modalities such as PET and MRI. Only surgical resection has shown benefit in term of survival times. In conclusion, although it is hardly distinguish between PAS and PE, a diagnosis of PAS should be suspected when CTPA characteristics of PAS or patients received anticoagulant therapy without improvement.