Definition
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).
Clinical Features
Incidence
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).
Age
The diffuse cutaneous and plexiform tumors are presumably congenital; the localized neurofibromas usually begin to appear in young adulthood.
Sex
Both sexes are equally affected.
Site
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...
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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.
Fletcher, C. D. (1990). Peripheral nerve sheath tumors. A clinicopathological update. Pathology Annual, 25(Pt1), 53–74.
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.
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.
Woodruff, J. M. (1999). Pathology of the peripheral nerve sheath in type 1 neurofibromatosis. American Journal of Medical Genetics, 89, 23–30.
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Sciot, R. (2020). Neurofibroma (All Variants). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5438-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5438-1
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