Definition
Ganglioneuroma is a benign and differentiated tumor of sympathetic ganglia containing maturing or mature ganglion cells and nerve fibers and is without immature neuroblastic component, occurring most commonly in posterior mediastinum. It is the most differentiated tumor in neuroblastic tumors (neuroblastomas), which are composed of cells with different degrees of differentiation, from immature neuroblastomas, over more mature ganglioneuroblastomas, to mature ganglioneuromas. Their malignant potential correlates with the amount of immature cells. In most cases ganglioneuroma arises de novo, but can develop by maturation of neuroblastoma. In half of the patients, they are symptomatic, and in one third there might be calcifications on CT scan. However, levels of catecholamine metabolites in urine are usually normal, unless there is a large tumor mass, when they can be elevated.
Clinical Features
Incidence
Ganglioneuromas are rare if compared to benign neurogenic tumors like...
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References and Further Reading
de Chadarévian, J. P., MaePascasio, J., Halligan, G. E., Katz, D. A., Locono, J. A., Kimmel, S., & Katsetos, C. D. (2004). Malignant peripheral nerve sheath tumor arising from an adrenal ganglioneuroma in a 6-year-old boy. Pediatric and Developmental Pathology, 7, 277–284.
Drago, G., Pasquier, B., Pasquier, D., Pinel, N., Rouault-Plantaz, V., Dyon, J. F., Durand, C., Armari-Alla, C., & Plantaz, D. (1997). Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma: A case report and review of the literature. Medical and Pediatric Oncology, 28, 216–222.
Goldblum, J. R., Folpe, A. L., & Weiss, S. W. (2014). Benign tumors of peripheral nerves. In J. R. Goldblum, A. L. Folpe, & S. W. Weiss (Eds.), Enzinger and Weiss’s soft tissue tumors (pp. 791–792). Philadelphia: Elsevier Saunders.
Macchiarini, P., & Ostertag, H. (2004). Uncommon primary mediastinal tumours. Lancet Oncology, 5, 107–118.
Wirnsberger, G. H., Becker, H., Ziervogel, K., & Höfler, H. (1992). Diagnostic immunohistochemistry of neuroblastic tumors. American Journal of Surgical Pathology, 16, 49–57.
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Brcic, L. (2018). Ganglioneuroma, Mediastinal. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_20
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DOI: https://doi.org/10.1007/978-3-319-66796-6_20
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