Primary Cardiac Paraganglioma Management and Resection With Rare Recurrent Metastasis.

BACKGROUND: Primary cardiac paragangliomas are rare tumors that present with nonspecific sympathomimetic symptoms. Early recognition and multidisciplinary planning are essential. CASE SUMMARY: A 55-year-old man presented with atrial fibrillation and pericarditis after weeks of dizziness, presyncope, and dyspnea. The patient underwent biopsy via thoracotomy, which was negative for malignancy. Workup revealed a 10 × 12 × 5 cm functional paraganglioma with left atrial involvement. He underwent cardiac autotransplantation with complete tumor resection and left atrial reconstruction. Postoperative recovery was complicated by extracorporeal membrane oxygenation requirement and stroke. One year later, surveillance identified a T9 vertebral metastasis, confirmed on biopsy, and later treated with Belzutifan, radiation, and kyphoplasty. DISCUSSION: Cardiac paragangliomas often require complex surgical approaches. This case highlights diagnostic difficulty, metastatic potential, and evolving systemic therapies of interest to cardiovascular and oncology specialists. TAKE-HOME MESSAGES: Cardiac paragangliomas demand multidisciplinary management and long-term surveillance. Newer targeted therapies expand options for metastatic disease.