Mediastinal Paraganglioma: A retrospective analysis of 51 cases

• Published in: Respiratory medicine Vol. 216; p. 107296
• Main Authors: Kanj, Amjad N., Young, William F., Ryu, Jay H.
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.09.2023
• Subjects: Catecholamines; Mediastinum; Paraganglioma; Radiology; Succinate dehydrogenase; Paraganglioma; Radiology; Catecholamines; Succinate dehydrogenase; Mediastinum
• ISSN: 0954-6111; 1532-3064
• DOI: 10.1016/j.rmed.2023.107296

Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the presenting features and relevant aspects in optimizing the diagnosis and treatment of mediastinal paragangliomas. A computer-assisted search of electronic health records was performed to identify adult patients (≥18 years) who underwent evaluation for a primary mediastinal paraganglioma at Mayo Clinic between January 2000 and April 2022. Medical charts, laboratory tests and radiology images were reviewed to collect data. The study included 51 patients, each with a single mediastinal paraganglioma. The median age was 47 years (IQR: 39–67), 67% females. Symptoms of catecholamine excess were manifest in 39% of patients, and 14% presented with mass effect, while the remaining 47% had no paraganglioma-related symptoms. Genetic testing was performed in 35 patients; 66% harbored a pathogenic variant in the succinate dehydrogenase enzyme complex. Most paragangliomas (71%) were in the middle mediastinum and showed uptake of intravenous contrast on chest imaging. Biopsies were performed in 30 (59%) patients; 27% were inconclusive and 10% resulted in major complications. Surgical resection occurred in 75%, primarily for relief of symptoms (50%) followed by proximity to critical structures (45%). Perioperative complications were common (66%), but there were no cases of local tumor recurrence during the follow-up period (median 8 years; IQR: 4–13). Mediastinal paragangliomas are most located in the middle mediastinum and can often be diagnosed noninvasively using a combination of clinical, biochemical, and radiological features. •Most mediastinal paragangliomas are located in the middle mediastinal compartment.•Analysis of clinical, biochemical, and radiological features facilitate the diagnosis.•Many patients harbor a pathogenic variant in the SDH enzyme complex.•Surgical resection is often needed but is associated with high complication rates.