Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Neurofibroma, Mediastinal

  • Luka BrcicEmail author
Reference work entry


Localized neurofibroma; Plexiform neurofibroma; Solitary neurofibroma


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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References and Further Reading

  1. 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.Google Scholar
  2. 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.CrossRefPubMedGoogle Scholar
  3. Macchiarini, P., & Ostertag, H. (2004). Uncommon primary mediastinal tumours. Lancet Oncology, 5, 107–118.CrossRefPubMedGoogle Scholar
  4. 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.Google Scholar
  5. 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.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2018

Authors and Affiliations

  1. 1.Institute of PathologyMedical University of GrazGrazAustria