Endovascular Diagnosis of Pulmonary Artery Intimal Sarcoma Using Intracardiac Echocardiography–Guided Biopsy

BACKGROUND: Primary tumors of the pulmonary artery are rare and often mimic thromboembolic disease, leading to delayed diagnosis with potentially serious consequences. CASE SUMMARY: A middle-aged woman with reduced exercise tolerance was found to have a progressive left hilar mass causing near-complete obstruction of the left pulmonary artery. Given high procedural risk, an intracardiac echocardiography (ICE)-guided endovascular biopsy was performed. Tissue sampling was successful and complication free. Histopathology and molecular analysis demonstrated a malignant pleomorphic neoplasm with MDM2 amplification, consistent with pulmonary artery intimal sarcoma. DISCUSSION: ICE-guided biopsy enables safe, real-time tissue acquisition from central pulmonary artery lesions. This technique enables timely histological and molecular diagnosis, especially when conventional biopsy approaches are deemed too risky. TAKE-HOME MESSAGES: Pulmonary artery intimal sarcoma should be differentiated from thromboembolic disease using imaging and clinical progression. ICE-guided biopsy represents a safe diagnostic option in high-risk cases and supports multidisciplinary decision-making.