Ganglioneurome médiastianal au cours de la neurofibromatose de Von Recklinghausen Mediastinal ganglioneuroma in the neurofibromatosis of Von Recklinghausen
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Abstract
Introduction - La neurofibromatose de Von Recklinghausen (NF1) est une maladie à transmission autosomique dominante, son incidence est estimée à 1/3000 naissances.
La localisation médiastinale (neurofibrome) est la 2 ème manifestation par ordre de fréquence après les déformations thoraciques à type de cyphoscoliose.
Observation - Un patient âgé de 23 ans était hospitalisé pour une neurofibromatose de Von Recklinghausen (NF1) confirmée par les critères cliniques, et associée à un ganglioneurome médiastinal, tumeur neurogéne bénigne, rare chez l’adulte.
L’imagerie a permis d’apprécier, outre le siège et le caractère hétérogène de la tumeur, son extension et ses rapports osseux et vasculaires et a laissé prévoir les difficultés opératoires rencontrées. Le diagnostic a été confirmé par l’étude histologique
de la pièce opératoire.
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