Synonyms
Localized neurofibroma; Plexiform neurofibroma; Solitary neurofibroma
Definition
Neurofibroma is a benign, well-defined, and usually not encapsulated peripheral nerve sheath tumor. It consists of all nerve elements like Schwann cells, myelinated and unmyelinated axons, and fibroblasts, with mast cells and perineurial-like cells. They mostly occur sporadically (up to 55%) or in patients with neurofibromatosis 1 (von Recklinghausen’s disease). It can grow as localized, diffuse, or plexiform lesion.
Clinical Features
Incidence
Neurofibromas are the second most common neurogenic tumors in mediastinum, following schwannomas. They comprise 2–4% of all mediastinal tumors.
Age
Neurofibromas can be found in all age groups, mostly between the age of 20 and 30 years, but are occurring in younger age if located in mediastinum as well as in congenital forms and in patients with neurofibromatosis 1.
Sex
Men and women are equally affected.
Site
Mediastinal neurofibromas occur mostly in...
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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. 796–813). Philadelphia: Elsevier Saunders.
Kanzaki, R., Inoue, M., Minami, M., Sawabata, N., Shintani, Y., Nakagiri, T., & Okumura, M. (2013). Bilateral mediastinal neurofibroma of the vagus nerves in a patient with neurofibromatosis type 1. Annals of Thoracic and Cardiovascular Surgery, 19, 293–296.
Macchiarini, P., & Ostertag, H. (2004). Uncommon primary mediastinal tumours. Lancet Oncology, 5, 107–118.
Scheithauer, B. W., Louis, D. N., Hunter, S., Woodruff, J. M., & Antonescu, C. R. (2007). Neurofibroma. In D. N. Louis, H. Ohgaki, O. D. Wiestler, & W. K. CAvenee (Eds.), WHO classification of tumours of the central nervous system (pp. 156–157). Lyon: International Agency for Research on Cancer.
Shintani, Y., Ohta, M., Hazama, K., Minami, M., Okumura, M., Hirabayashi, H., & Matsuda, H. (2002). Bilateral cervicomediastinal neurofibroma originating from the vagal nerve in a patient with von Recklinghausen’s disease: Report of a case. Surgery Today, 32, 1068–1071.
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Brcic, L. (2018). Neurofibroma, 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_42
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DOI: https://doi.org/10.1007/978-3-319-66796-6_42
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Publisher Name: Springer, Cham
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