Diagnosis, Genetics, and Management of Twenty-four Patients with Cardiac Paragangliomas: Experience from a Single Center

Abstract Context Paragangliomas located within the pericardium represent a rare yet challenging clinical situation. Objective The current analysis aimed to describe characteristics of cardiac paragangliomas, with emphasis on diagnostic approach, genetic background, and multi-disciplinary management. Methods Twenty-four patients diagnosed paraganglioma in Peking Union Medical College Hospital, Beijing, China between 2003 2021 were identified. Clinical data was collected from medical record. Genetic screening SDHB immunohistochemistry performed 22 patients. Results median age at diagnosis 38 years (range, 11-51 years), 8 (33%) females, 4 (17%) had familial history. Hypertension and/or symptoms related catecholamine secretion present (92%) Excess levels catecholamines metanephrines detected (96%) 23 Cardiac PGLs localized 131I-metaiodobenzylguanidine scintigraphy 11/22(50%), 99mTc-hydrazinonicotinyl-tyr3-octreotide 24/24(100%) testing identified germline SDHx mutations 13/22 (59%) patients, while revealed SDH deficiency tumors 17/22 (77%) All managed by team through preparation, surgery, follow-up. Twenty-three received surgical treatment perioperative death occurred 2 cases. Overall, 21 alive follow-up (median 7.0 years, range 0.6-18 years). Local recurrence or metastasis developed 3 all whom SDH-deficient tumors. Conclusions paragangliomas can be based manifestations, biochemical tests, appropriate imaging studies. screening, long-term are crucial management this disease.