title: [Intrathoracic giant peripheral nerve sheath tumor during Von Recklinghausen disease].
abstract: We report the case of a patient aged 23, admitted for bilateral intrathoracic tumor, including a giant right. Surgery was performed by right sternothoracotomy. After 7 days, she presented an irreversible cardiac arrest. The malignant peripheral nerve sheath tumors are rare and aggressive. Their incidence is 0.001% in the general population and 0.16% in patients with neurofibromatosis type 1. These tumors are characterized by their risk of recurrence and poor prognosis. The treatment is the surgical resection. We analyze incidence, diagnosis and prognosis of these tumors.
authors.affiliation: Service de chirurgie viscérale et thoracique, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon. Electronic address: ngabou12@yahoo.fr.
authors.forename: U D
authors.initials: UD
authors.lastname: Ngabou
authors.affiliation: Service de pneumologie, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: D
authors.initials: D
authors.lastname: Mounguengui
authors.affiliation: Service de chirurgie viscérale et thoracique, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: J P
authors.initials: JP
authors.lastname: Owono Mbouengou
authors.affiliation: Service de réanimation, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: A
authors.initials: A
authors.lastname: El Wali
authors.affiliation: Service d'imagerie médicale, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: B
authors.initials: B
authors.lastname: Nguema Edzang
authors.affiliation: Service de pneumologie, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: J B
authors.initials: JB
authors.lastname: Boguikouma
authors.affiliation: Service de réanimation, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: R
authors.initials: R
authors.lastname: Tchoua
authors.affiliation: Service d'imagerie médicale, hôpital d'instruction des armées Omar Bongo-Ondimba, BP 20404, Libreville, Gabon.
authors.forename: N E
authors.initials: NE
authors.lastname: Aziz
mesh_terms: D017809:Fatal Outcome; D005260:Female; D006323:Heart Arrest; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000150:complications*; D013899:Thoracic Neoplasms / Q000175:diagnosis*; D055815:Young Adult
keywords: Giant intrathoracic tumors; Malignant peripheral nerve sheath; Neurofibromatose de type I; Neurofibromatosis type I; Tumeur géante intra-thoracique; Tumeurs malignes des gaines des nerfs périphériques