A Tale of Two Zebras: Cardiac Intimal Sarcoma Discovered in a Male Patient With SCAD and ADPKD
Jay A Patel1, Matthew G Kaye2, Aditya Kesari3
1Division of Cardiothoracic Surgery, Department of Surgery, Virginia Commonwealth University Medical Center, Richmond, Virginia, USA.
Background:
Cardiac intimal sarcoma is a very rare vascular-based sarcoma that arises from the left atrium and pulmonary artery.
Case Summary:
A 58-year-old male with a history of coronary artery disease, prior coronary artery bypass grafting, and spontaneous coronary artery dissection (SCAD) presented with myocardial infarction and was referred for heart transplantation. Further investigation revealed previously undiagnosed autosomal dominant polycystic kidney disease (ADPKD). During cardiac explantation, an incidental high-grade cardiac intimal sarcoma was discovered.
Discussion:
In male patients, SCAD and cardiac intimal sarcomas are both rare entities. Although ADPKD is associated with SCAD and solid tumor malignancies, its potential association with cardiac sarcoma is not well defined.
Take-Home Messages:
This case highlights the importance of investigating secondary causes of SCAD, particularly when it presents in a male patient. Additionally, this case suggests a potential connection between ADPKD and cardiac intimal sarcoma.


