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.
Neurofibromas are the second most common neurogenic tumors in mediastinum, following schwannomas. They comprise 2–4% of all mediastinal tumors.
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.
Men and women are equally affected.
Mediastinal neurofibromas occur mostly in...
References and Further Reading
- 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
- 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