Intrahepatic plexiform neurofibroma in neurofibromatosis 1
✍ Scribed by Isolina Vilas-Ferrol; Miguel Hernández-Giménez; M. Isabel Moya-García; M. Antonia Menargues-Irles; Carlos Muñoz-Núñez; Enrique Poblet-Martinez
- Book ID
- 105942898
- Publisher
- Springer-Verlag
- Year
- 1998
- Tongue
- English
- Weight
- 283 KB
- Volume
- 28
- Category
- Article
- ISSN
- 0301-0449
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Individuals with neurofibromatosis 1 (NF1) develop both benign and malignant tumors at an increased frequency. One of the most common benign tumors in NF1 is the plexiform neurofibroma. These tumors cause significant morbidity and mortality on account of their propensity to grow and affect adjacent
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