Neurofibfrome plexiforme cervical: à propos d’un cas

Abstract

Le neurofibrome plexiforme est une tumeur bénigne rare des nerfs périphériques aux dépens des cellules conjonctives du périnevre. Il est pathognomonique de la neurofibromatose de type 1 (NF1 ou maladie de Von Recklinghausen). L'IRM est d'une grande aide au diagnostic de cette pathologie. La confirmation anatomopathologique est parfois nécessaire en particulier en dehors d'un contexte évocateur d'une NF1. Nousrapportonsl'observation d'une petite fille atteinte de neurofibrome plexiforme cervical révélateur d'une neurofibromatose Type 1.

A case report and review of the literature on Von Recklinghausen’s disease complicated with cervicofacial plexiform neurofibromas

“…The NIH (National Institutes of Health Consensus Conference) identified four forms of neurofibromas: Cutaneous neurofibromas, subcutaneous neurofibromas, nodular neurofibromas, and diffuse plexiform neurofibroma [1]. The presence of at least two of the following criteria is required for a diagnosis of the disease: At least six café au lait spots that are larger than 5 mm before puberty and 15 mm after puberty, One or more plexiform neuromas, with two or more neurofibromas axillary and inguinal lentiginous spots; One optic tract glioma and two or more iris hamartomas (Lisch nodules); A bony lesion with a distinct appearance (pseudarthrosis of a long bone, spheno-orbital dysplasia, cervical kyphosis) [7].…”

Type I Neurofibromatosis: Case Report and Review of the Literature Focused on Oral and Cutaneous Lesions

“…Type I neurofibromatosis (NF1) is one of the most common genetic diseases and, given its genetic characteristic, affects individuals at a young age. Oral manifestations in relation to NF1 have been known for a long time, but their occurrence frequency was underestimated for a long period until recent studies increased their prevalence to around 70% of all NF1 individuals [ 2 , 4 , 6 , 10 , 15 ]. This article aimed to highlight the importance of oral examination when NF1 is suspected and to discuss neurofibromas as a potential differential diagnosis of oral nodules.…”

“…Plexiform neurofibroma is a poorly circumscribed and locally invasive tumor mass which extends along the length of a nerve trunk growing around distorted nerve fascicules and may spread along adjacent nervous rami, muscles, and skin [ 9 , 12 , 13 , 15 ]. These lesions may occur superficially or deeper inside the whole body and are a high source of morbidity as they grow to reach a great size, often producing disfigurement [ 9 , 13 ].…”

“…Plexiform neurofibromas appear at birth until 5 years of age and grow during childhood and adolescence [ 1 ], and then begin to decrease their growth potential with increasing age [ 16 ]. Sixteen to twenty-five percent of NF1 patients present with plexiform neurofibroma [ 12 ] which is pathognomonic of NF1 and does not occur sporadically [ 15 ].…”

Plexiform Neurofibroma of Arm: A Case Report

“…Plexiform neurofibromas are pathognomonic of NF1. They are generally slow growing tumors [1]. Their symptomatology is variable depending on their topography.…”