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Neurofibroma is a benign peripheral nerve sheath tumor composed of Schwann cells, fibroblasts, perineural cells, and mast cells. It arises from peripheral nerves and is non-encapsulated, often infiltrating along nerve fascicles.

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Etiology

Neurofibromas can occur as:

• Solitary (sporadic): Usually isolated, not associated with systemic disease
• Multiple (plexiform): Strongly associated with Neurofibromatosis type 1 (NF1)
• Diffuse: Superficial, infiltrative form often seen in NF1

![Classifcation of neurofbromas

Rasulic, L., Lepić, M., Savić, A., Samardžić, M. (2021). Neurofibromas. In: Guedes, F., Zager, E.L., Garozzo, D., Rasulic, L., Socolovsky, M. (eds) Diagnostic Assessment and Treatment of Peripheral Nerve Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-77633-6_16](attachment:2e51dbd6-788c-430b-aa4f-ef874abdb01c:image.png)

Classifcation of neurofbromas

Rasulic, L., Lepić, M., Savić, A., Samardžić, M. (2021). Neurofibromas. In: Guedes, F., Zager, E.L., Garozzo, D., Rasulic, L., Socolovsky, M. (eds) Diagnostic Assessment and Treatment of Peripheral Nerve Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-77633-6_16

Neurofibromatosis Type 1 (NF1)

• Autosomal dominant disorder (mutation in NF1 gene on chromosome 17)
• Characterized by multiple neurofibromas, café-au-lait spots, Lisch nodules, axillary freckling, optic gliomas, and skeletal dysplasia

Histopathology

Histopathology Skin--Neurofibroma

https://doi.org/10.1007/978-3-030-77633-6_16

• Composed of interlacing bundles of spindle cells with wavy nuclei
• No true capsule (in contrast to schwannoma)
• Myxoid stroma, scattered mast cells
• Plexiform type shows expanded nerve branches in a tortuous mass

Clinical Features