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Related to Recklinghausen's disease: type 1 neurofibromatosis
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Despite the fact that most MPNST are diagnosed in patients with von Recklinghausen's disease,[sup.
One patient had a radical cystectomy due to the uncertain pathogenesis of the inflammatory myofibroblastic tumour and the rarity of Von Recklinghausen's disease.
She was known to have von Recklinghausen's disease with the typical cafe au lait spots.
Solitary neurofibroma without von Recklinghausen's disease is the rarest presentation of all neurofibromas.
They differ from neurofibromas in that the latter are unencapsulated, they do not cause symptoms, and they may be associated with von Recklinghausen's disease.
Neurofibroma is a neurogenic tumor originating from the peripheral nerve, which occurs sporadically or in part with von Recklinghausen's disease.