Surgical Management and Long-Term Outcomes of Mediastinal Paragangliomas: Case Series and Literature Review
Main Article Content
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.
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References
2. Lack EE. Tumors of the adrenal gland and extra-adrenal paraganglia. AFIP Atlas of Tumor Pathology. 1997.
3. Plouin PF, Gimenez-Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet Journal of Rare Diseases. 2006; 1:49.
4. Chen H, Sippel RS, O'Dorisio MS, et al. The clinical manifestations of PGL. World J Surg. 2002; 26(8):1023-1030.
5. Kanj A, et al. Mediastinal PGLs: a 51-case retrospective analysis. Ann Thorac Surg. 2023;115 (2):385-394.
6. Ilias I, Pacak K, et al. MIBG scintigraphy in functional PGLs. J Clin Endocrinol Metab. 2003; 88(9):4080-4086.
7. Janssen I, Chen CC, Millo CM, et al. PET/CT imaging of pheochromocytoma and paraganglioma with 68Ga-DOTATATE: a prospective single-center study. J Nucl Med. 2021;62(10):1428–1434.
8. Baysal BE, et al. Mutations in SDHD cause hereditary PGL type 1. Science. 2000;287(5454): 848-851.
9. Niemann S, Müller U. SDHC mutations in PGL. Nat Genet. 2000;26(3):268-270.
10. Burnichon N, et al. SDH mutations in PGL: genotype-phenotype correlations. J Clin Endocrinol Metab. 2010;95(2):959-968.
11. Ullah F, et al. Long term follow up of 3 patients after resection of mediastinal PGL necessitating cardiopulmonary bypass: case series. J Surg Case Rep. 2024;(12):501.
12. Brown ML, Zayas GE, Abel MD, et al. Mediastinal PGLs: the Mayo Clinic experience. Ann Thorac Surg. 2008;86(3):946-951.
13. Gurrieri C, D’Amico G, et al. Surgical management of mediastinal PGL: single-center experience and review. J Thorac Dis. 2018;10(5):2944-2953.
14. Lamy AL, et al. Mediastinal PGLs: a clinicopathologic and immunohistochemical study of 79 cases. Cancer. 1994;73(8):2248-2258.
15. Asa SL, Mete O. Endocrine Pathology: PGL. Springer; 2018
16. Li, Wilson W. L., et al. “Management of Large Mediastinal Masses: Surgical and Anesthesiological Considerations.” Journal of Thoracic Disease, vol. 8, no. 3, 2016, pp. E175–E184.
17. Ghayee, Hans K., et al. “Mediastinal Paragangliomas: Association with Mutations in the Succinate Dehydrogenase Genes and Aggressive Behavior.” Endocrine-Related Cancer, vol. 16, no. 1, 2009, pp. 291–299.
18. Burnichon, Nelly, et al. “The Succinate Dehydrogenase Genetic Testing in a Large Prospective Series of Patients with Paragangliomas.” J Clin Endocrinol Metab, vol. 94, no. 8, 2009, pp. 2817–2827.
19. Thorpe MP, et al. Long-Term Outcomes of 125 Patients with Metastatic Pheochromocytoma or PGL Treated With 131-I MIBG. J Clin Endocrinol Metab. 2020;105(3): e494–501.
20. Hamidi, O., et al. “Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years.” J Clin Endocrinol Metab, vol. 102, no. 9, 2017, pp. 3296–3305.
21. Taieb D, Pacak K. Current approaches and recent developments in the management of head and neck paragangliomas and pheochromocytomas. Endocr Rev. 2021;42(3):329–377.
22. Janssen I, et al. 68Ga-DOTATATE PET/CT in PGL surveillance. J Nucl Med. 2015;56(3):399-405.