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

Primary tumors of the pulmonary artery are rare and often mimic thromboembolic disease, leading to delayed diagnosis with potentially serious consequences.

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.

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.

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.