Abstract
Background: Mediastinal paragangliomas (PGLs) are rare neuroendocrine tumors originating from extra-adrenal chromaffin tissue, accounting for less than 0.3% of mediastinal masses. They are often non-secretory but have the potential to grow and cause secondary mass effects. They also carry a potential risk of metastasis if left untreated. Advancements in imaging, genetics, and cardiopulmonary support have enhanced long-term outcomes. This study provides an updated follow-up of three surgically treated mediastinal PGLs and examines the existing literature on recurrence, metastasis, and outcomes.
Methods: A retrospective case series from a single institution involving three patients who underwent resection of middle mediastinal PGLs via cardiopulmonary bypass (CPB) was examined. Long-term follow-up data (7–12 years) were obtained from clinical records. A systematic literature review (PubMed, Embase, Scopus; from inception–July 2025) identified studies reporting mediastinal PGL resection with over six months of follow-up. The extracted data encompassed surgical approach, recurrence, metastasis, and complications.
Results: All three patients were female, aged 48 to 75 years, and presented with non-secretory middle mediastinal PGLs. Using CPB through a median sternotomy, all cases achieved complete (R0) resection. One patient experienced unilateral vocal cord paralysis that required treatment; no additional significant complications arose. There were no biochemical, radiographic, or clinical recurrences during the 7–12 years of follow-up. A literature review of 169 reported resections indicated that complete resection is the most significant predictor of local control. Historical recurrence rates (approximately 55%) have diminished to less than 10% in contemporary series utilizing CPB and multidisciplinary planning. Metastasis rates (6–27%) seem to be more closely related to the status of the SDH mutation than to the anatomical location.
Conclusion: Complete surgical resection, frequently aided by planned CPB, ensures lasting local control for resectable mediastinal PGLs with minimal morbidity, though long-term monitoring remains essential due to genotype-linked metastatic risk. Our prolonged follow-up substantiates the effectiveness and safety of surgical intervention for mediastinal PGLs in contemporary practice.