7111 Intrapericardial Paraganglioma, An Exceedingly Rare Finding

Disclosure: R. Habibi: None. M. Pendergrass: None. E. Neda: None. R. Cardenas Lara: None. Introduction: Hereditary paraganglioma/pheochromocytoma (PGL/PCC) syndromes are rare tumors occasionally resulting from germline pathogenic variant in succinate dehydrogenase subunit B and D (SDHB and SDHD) genes. Hereditary forms are usually present in the posterior mediastinum. Few cases of PGL/PCC syndromes are reported in intrapericardial space. Below, we report on such a patient, illustrating the importance of genetic testing and whole-body imaging surveillance in patients with tumors suggestive of paragangliomas. Presentation: We present a case of a 25-year-old female with an intracardiac mass suspicious for intrapericardial paraganglioma. She was initially diagnosed with a large jugular when she presented to the emergency department with nausea, vomiting, headaches, with recent diagnosis of cranial nerve VI palsy and vertigo. MRI brain with and without contrast showed a hypervascular glomus jugular tumor in the right skull base with extension into the carotid space of the neck, with leftward effacement of the posterior oropharynx and flow into the right sigmoid and transverse sinus during the arterial phase, suggestive of a paraganglioma. The paraganglioma was deemed non secretory with unremarkable initial endocrine work up. Her 24 hours urine 3-methoxythyramine and dopamine levels were elevated as expected with paraganglioma. She was referred to a geneticist and diagnosed with hereditary PGL/PCC syndrome. This was secondary to a germline pathogenic variant in SDHB gene (deletion exon 8). Cardiac MRI revealed an intensely enhancing intrapericardial lesion along the anterior/superior aspect of the left atrium. Imaging features were suggestive of an intrapericardial paraganglioma, given the genetic analysis and concurrent imaging findings of neck glomus tumor. She also had abdominal and pelvic CT scan with IV contrast which did not reveal any mass. Cardiothoracic surgeon decided on non-surgical intervention. The neurosurgery team recommended surgery since surgery in the jugular foramen seemed morbid with probable multiple cranial nerve injuries. The patient eventually received courses of radiation treatment to the glomus paraganglioma of the brain which improved the size of tumor. Discussion: Paragangliomas are chromaffin tumors that arise from either sympathetic or parasympathetic ganglia. The literature reveals about 2 % or less of paragangliomas affect the chest. Most of these tumors will be found in the posterior mediastinum. The very small proportion of them found in the heart are commonly seen arising from the roof of the left atrium in addition to affecting cardiac structures. Due to a high level of vascularization and the inherent risk of hemorrhage, tissue biopsy is not typically advised. In nonfunctional paragangliomas, surgery is often the ultimate treatment. Presentation: 6/2/2024