Neurofibroma (All Variants)
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A benign peripheral nerve sheath tumor consisting of an admixture of Schwann cells, perineural-like cells, fibroblasts, mast cells, and residual axons. Three major types exist: localized (cutaneous or intraneural), diffuse, and plexiform, the latter being pathognomonic for neurofibromatosis type I (NF1).
Neurofibromas are the most common peripheral nerve sheath tumors. The vast majority (90%) is sporadic and solitary. In the context of NF1, multiple tumors are usually present (Fletcher 1990; Woodruff 1999).
The diffuse cutaneous and plexiform tumors are presumably congenital; the localized neurofibromas usually begin to appear in young adulthood.
Both sexes are equally affected.
Localized neurofibroma usually presents as a cutaneous nodule. Deep-seated localized lesions are less common and more often associated with NF1. Diffuse neurofibromas are ill-defined skin plaques, most common in the trunk/head and neck area. Plexiform...
References and Further Reading
- Beert, E., Brems, H., Daniëls, B., De Wever, I., Van Calenbergh, F., Schoenaers, J., Debiec-Rychter, M., Gevaert, O., De Raedt, T., Van Den Bruel, A., de Ravel, T., Cichowski, K., Kluwe, L., Mautner, V., Sciot, R., & Legius, E. (2011). Atypical neurofibromas in neurofibromatosis type 1 are premalignant tumors. Genes, Chromosomes & Cancer, 50, 1021–1032.CrossRefGoogle Scholar
- Michal, M., Fanburg-Smith, J. C., Mentzel, T., Kutzner, H., Requena, L., Zamecnik, M., & Miettinen, M. (2001). Dendritic cell neurofibroma with pseudorosettes: A report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant. The American Journal of Surgical Pathology, 25, 587–594.CrossRefGoogle Scholar
- Miettinen, M. M., Antonescu, C. R., Fletcher, C. D. M., Kim, A., Lazar, A. J., Quezado, M. M., Reilly, K. M., Stemmer-Rachamimov, A., Stewart, D. R., Viskochil, D., Widemann, B., & Perry, A. (2017). Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview. Human Pathology, 67, 1–10.CrossRefGoogle Scholar