The Intersection of a Rare Mass and Dual Primary Malignancies: A 73-Year-Old Male Patient's Cardiac Conundrum

Intimal sarcoma (IS) is a rare malignant tumor originating from the tunica intima of large blood vessels, mainly in the pulmonary circulation. Prevalence is extremely low (0.001%), with IS of the pulmonary vein even rarer than IS of the pulmonary artery (0.001%-0.003%).

A 73-year-old man with malignant peripheral nerve sheath tumor and high-grade urothelial carcinoma was diagnosed with grade 3 pulmonary vein IS during evaluation of probable acute decompensated heart failure. Echocardiography showed a hyperechoic mass extending from the pulmonary vein into the left atrium and protruding into the left ventricle. The patient underwent surgical resection.

Despite invasive interventions, aggressive IS often recurs, making regular imaging follow-up essential for early detection of metastasis and highlighting the need for further research into effective treatment strategies.

This case highlights the importance of including pulmonary vein IS in the differential diagnosis of left atrial masses and the value of multimodality imaging for timely detection and surgical planning.