Diffuse neurofibromas are poorly defined, benign tumors of the peripheral nerve sheath with an invasive growth pattern. They are not commonly associated with neurofibromatosis or other neurocutaneous disorders. This manuscript aims to document the occurrence of diffuse neurofibromas in patients with NF1, while providing a comprehensive review of this type of tumor. We present three cases highlighting diffuse neurofibromas’ clinical, radiological, or histological characteristics of diffuse neurofibromas in patients with a long-standing history of NF1. These rare tumors have a low prevalence but have defined and characteristic pathologic, imaging, and immunohistochemistry features for diagnosis. Treatment is challenging due to the lack of standardization in therapies and should be analyzed on a case-by-case basis. Diffuse neurofibromas should be included in the differential diagnosis in patients with NF1 and any associated cutaneous lesions. Further studies are needed to standardize an integral approach in these patients.

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