๐”– Bobbio Scriptorium
โœฆ   LIBER   โœฆ

Clinic-based study of plexiform neurofibromas in neurofibromatosis 1

โœ Scribed by Waggoner, Darrel J.; Towbin, Jennifer; Gottesman, Gary; Gutmann, David H.

Publisher
John Wiley and Sons
Year
2000
Tongue
English
Weight
19 KB
Volume
92
Category
Article
ISSN
0148-7299

No coin nor oath required. For personal study only.

โœฆ Synopsis

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 normal tissues. To determine the clinical profile of plexiform neurofibromas in NF1, we conducted a retrospective review of 68 NF1 patients with plexiform neurofibroma. In our series, 44% of tumors were detected by 5 years of age and most were located in the trunk and extremities. Only two patients developed malignant peripheral nerve sheath tumors in their preexisting plexiform neurofibromas. Lastly, we demonstrate that there were no specific clinical features of NF1 associated with the presence of plexiform neurofibroma. These results underscore the importance of careful serial examinations in the evaluation of patients with NF1.

๐Ÿ“œ SIMILAR VOLUMES

NF1 mutations in neurofibromatosis 1 pat
NF1 mutations in neurofibromatosis 1 patients with plexiform neurofibromas
โœ Lan Kluwe; Reinhard E. Friedrich; Bruce Korf; Raimund Fahsold; Victor-F. Mautner ๐Ÿ“‚ Article ๐Ÿ“… 2002 ๐Ÿ› John Wiley and Sons ๐ŸŒ English โš– 198 KB

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

Management issues in massive pediatric f
Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type 1
โœ Jeffrey B. Wise; Snehal G. Patel; Jatin P. Shah ๐Ÿ“‚ Article ๐Ÿ“… 2002 ๐Ÿ› John Wiley and Sons ๐ŸŒ English โš– 123 KB

## 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

Plexiform-like neurofibromas develop in
Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line
โœ George Q. Perrin; Lauren Fishbein; Susanne A. Thomson; Stacey L. Thomas; Karen S ๐Ÿ“‚ Article ๐Ÿ“… 2007 ๐Ÿ› John Wiley and Sons ๐ŸŒ English โš– 440 KB

## Abstract Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression. Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary