Synonyms
Mediastinal ganglioneuroblastoma
Definition
Ganglioneuroblastoma is a tumor of sympathetic ganglia with intermediate degree of maturation of tumor cells toward ganglion cells. It belongs to the neuroblastic tumors which range from the immature neuroblastoma with the highest malignant potential over ganglioneuroblastomas to the benign ganglioneuromas composed of maturing or mature ganglion cells with different amounts of Schwannian stroma. In ganglioneuroblastoma Schwannian stroma is present in more than 50% of the tumor, and histologic characteristics of neuroblastoma and ganglioneuroma are present. Because of neuroblastoma component, they can have elevated plasma levels of catecholamines and vasoactive intestinal peptides.
Clinical Features
Incidence
There is no data about incidence. However, after leukemia and brain tumors, it is the third most common malignancy in children and the most common solid extracranial tumor in childhood. Around 20% of cases are in the thorax, and...
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References and Further Reading
Hsiao, C. C., Huang, C. C., Sheen, J. M., Tai, M. H., Chen, C. M., Huang, L. L., & Chuang, J. H. (2005). Differential expression of delta-like gene and protein in neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Modern Pathology, 18, 656–662.
Macchiarini, P., & Ostertag, H. (2004). Uncommon primary mediastinal tumours. Lancet Oncology, 5, 107–118.
Peuchmaur, M., d’Amore, E. S., Joshi, V. V., Hata, J., Roald, B., Dehner, L. P., Gerbing, R. B., Stram, D. O., Lukens, J. N., Matthay, K. K., & Shimada, H. (2003). Revision of the International Neuroblastoma Pathology Classification: Confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer, 98, 2274–2281.
Shimada, H., Ambros, I. M., Dehner, L. P., Hata, J., Joshi, V. V., Roald, B., Stram, D. O., Gerbing, R. B., Lukens, J. N., Matthay, K. K., & Castleberry, R. P. (1999). The International Neuroblastoma Pathology Classification (the Shimada system). Cancer, 86, 364–372.
Shimosato, Y., Mukai, K., & Matsuno, Y. (2010). Mesenchymal and neurogenic tumors of the mediastinum. In S. G. Silverberg (Ed.), AFIP Atlas of tumor pathology. Tumors of the mediastinum (pp. 297–300). Washington, DC: American Registry of Pathology.
Spix, C., Berthold, F., Erttmann, R., Fehse, N., Hero, B., Klein, G., Sander, J., Schwarz, K., Treuner, J., Zorn, U., & Michaellis, J. (2002). Neuroblastoma screening at one year of age. New England Journal of Medicine, 346, 1047–1053.
Strollo, D. C., Rosado-de-Christenson, M. L., & Jett, J. R. (1997). Primary mediastinal tumors: Part II. Tumors of the middle and posterior mediastinum. Chest, 112, 1344–1357.
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Brcic, L. (2018). Ganglioneuroblastoma, 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_19
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DOI: https://doi.org/10.1007/978-3-319-66796-6_19
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