Neurofibromatosis 1 (NF1) is an autosomal dominant disorder caused by genetic alterations of the NF1 gene on 17q11.2. About 30% of NF1 patients develop plexiform neurofibromas (PNFs), which often cause severe clinical deficits. To determine whether there is a certain genotype underlying PNFs or subt
MRI growth patterns of plexiform neurofibromas in patients with neurofibromatosis type 1
✍ Scribed by V. F. Mautner; M. Hartmann; L. Kluwe; R. E. Friedrich; C. Fünsterer
- Publisher
- Springer
- Year
- 2006
- Tongue
- English
- Weight
- 190 KB
- Volume
- 48
- Category
- Article
- ISSN
- 0028-3940
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## Abstract ## Background Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF1). Surgical management is the mainstay of therapy, but within the head and neck region it is limited by the infiltrating nature of these tumors, inheren
4 from patients with Von Hippel-Lindau syndrome (m). The diagnosis was confirmed in each NF1 patient and conformed to internationally agreed criteria (Riccardi and Eichner, 1986;Mulvihill and Parry, 1987). The diagnosis of MEN 2 and VHL was similarly con-