Neurofibromatosis type 2


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Neurofibromatosis type 2 (NF2)

A hereditary condition associated with an increased risk of bilateral acoustic neuromas, other nerve cell tumors and cataracts.
Mentioned in: Acoustic Neuroma
References in periodicals archive ?
Tanycytic ependymoma in association with neurofibromatosis type 2.
Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2.
Neurofibromatosis type 2 is the major genetic condition that predisposes to meningioma formation, which are often multiple.
However, they are more commonly associated with neurofibromatosis type 2, and the more recently established entity of schwannomatosis.
Currently, we are not aware of a tumor predisposition syndrome other than neurofibromatosis type 2.
Neurofibromatosis type 2 genetic and clinical features.
Background: The auditory brainstem implants (ABIs) have been used to treat deafness for patients with neurofibromatosis Type 2 and nontumor patients.
1,2) Neurofibromas are usually multiple upon presentation and are usually part of two autosomal dominant disorders with variable penetrance: neurofibromatosis type 1 (NF1, von Recklinghausen's disease) and neurofibromatosis type 2 (NF2, central or bilateral acoustic neurofibromatosis).
Neurofibromatosis type 2, which is rarer, can cause cranial nerve palsies.
1-4) Oral cavity neurofibromas are characteristically associated with multiple lesions elsewhere and are manifestations of neurofibromatosis type 1 (yon Recklinghausen syndrome) or neurofibromatosis type 2 (bilateral acoustic neuroma/ central neurofibromatosis); isolated neurofibromas in the oral cavity, on the other hand, are very rare.
Two patients had neurofibromatosis type 2 (NF-2), and 1 had inherited retinoblastoma with prior radiation therapy including the orbit and brain.