CTICasesIntimal Sarcoma of the Pulmonary Artery
Imaging findings
Chest radiograph shows a unilateral right-sided Westermarck sign with subtle decreased attenuation of the right lung from reduced pulmonary blood flow. CT demonstrates a large mass expanding the lumen of the right main pulmonary artery extending into the central PA and involving the pulmonary valve, with variable attenuation within the mass and scattered pulmonary nodules. The mass expands the vessel lumen beyond what a simple thrombus would produce. Following pneumonectomy, the resected specimen shows the intimal sarcoma being extracted from the pulmonary artery, with the tumor involving and expanding the intima of the main pulmonary artery.
Key takeaways
Intimal sarcoma of the pulmonary artery is a rare primary vascular malignancy that presents as a filling defect within the pulmonary artery lumen, closely mimicking pulmonary embolism or chronic thromboembolic disease. Distinguishing features that suggest sarcoma rather than thrombus include: expansion of the vessel lumen beyond its normal caliber, extension into the pulmonary valve, very heterogeneous attenuation, and the absence of other sites of DVT. Prognosis is poor with median survival measured in months, though surgical resection including pneumonectomy with PA reconstruction offers the best available treatment. UCSD has specialized expertise with both CTEPH endarterectomy and pulmonary artery sarcoma resection.
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