Key result
Multimodal treatment resolves cardiac paraganglioma and normalizes biomarkers at 6 months in a single patient.
Why the study?
Cardiac paragangliomas are rare neuroendocrine tumors that remain difficult to diagnose due to nonspecific clinical manifestations and overlapping radiographic features with other cardiovascular diseases.
Case Report (n=1)
No
This case highlights the successful multidisciplinary management of a rare multifocal cardiac paraganglioma associated with an SDHC mutation using combined medical, radiotherapeutic, and surgical approaches.
Supports multidisciplinary management of rare SDHC-associated cardiac paragangliomas; hypothesis-generating pending larger studies.
Cardiac paragangliomas are rare neuroendocrine tumors, accounting for ≤1% of primary cardiac tumors. They remain difficult to diagnose, partly because of their nonspecific clinical manifestations and overlapping radiographic features with those of other cardiovascular diseases. This report describes a case of multifocal paragangliomas with pathogenic succinate dehydrogenase C (SDHC) gene mutations, highlighting the important role of a combined biochemical, radiographic, and genetic work-up in a multimodal approach. The patient was a 55-year-old man with progressively worsening left-sided tinnitus and hearing loss. Computed tomography scan and magnetic resonance imaging revealed the tumor in the left jugular bulb. In contrast, positron emission tomography with DOTATATE showed highly avid tracer accumulation in both jugular foramina and subcarinal mediastinum, with the former causing minimal indentation of the left atrium. His elevated plasma catecholamine levels (dopamine: 5.11 nmol/L; normetanephrine: 0.72 nmol/L; 3-methoxytyramine: 1.25 nmol/L) were confirmed. After adequate pre- and post-blockade with alpha- and beta-blockers, the patient underwent fractionated stereotactic irradiation of the jugular tumor, in addition to trans-arterial embolization and posterolateral thoracotomy with cardiopulmonary bypass and bovine pericardial reconstruction of the acutely injured left atrium. Histopathological verification demonstrated tumor cells reactive with chromogranin and synaptophysin, with focal necrosis in the heart. Germline genetic testing confirmed pathogenic mutations in the SDHC gene. Consequently, he was diagnosed with hereditary paraganglioma.
No takes yet. Share an insight, caveat, or question.
Ahmed Sameer I. Alshaer (2026) conducted a case report in 55-year-old man with multifocal hereditary paragangliomas including cardiac involvement and pathogenic SDHC gene mutation (n=1). Fractionated stereotactic radiotherapy (FSRT) for jugular tumor; preoperative bronchial artery embolization; surgical resection with cardiopulmonary bypass and bovine pericardial reconstruction for subcarinal/left atrial tumor was evaluated on Tumor control assessed by imaging and biochemical normalization at 6 months.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: