title: [Malignant intracerebral nerve sheath tumours: Two case reports and complete review of the literature cases].
abstract: Malignant peripheral nerve sheath tumours are extremely rare and can be associated with neurofibramatosis type 1. Their prognosis is poor and surgery remains the mainstay of therapy and should be the first line of treatment. Radiotherapy and chemotherapy are second line treatment and their effectiveness remains to demonstrate. The diagnosis is clinical, radiological, histological and immunohistochemical. Malignant peripheral nerve sheath tumours have a potential of local tumour recurrence very high and can metastasize. They often occur in extremity of the members but also rarely into brain. We report two cases of intracerebral nerve sheath tumour. The first was a 68-year-old woman who was admitted with progressive symptoms of headache and diplopia. A left frontotemporal malignant peripheral nerve sheath tumours was diagnosed and was treated by surgery and irradiation. Ten months later, she presented a local recurrence and spine bone's metastases were treated by vertebroplasty and irradiation. The patient died 15 months after the diagnosis. The second case was a 47-year-old woman who was referred because headache and vomiting symptoms. A right frontal malignant peripheral nerve sheath tumours was diagnosed and treated by surgery and irradiation. After that, the patient had three local recurrence operated and pulmonary and cranial bone's metastases. She was still alive after 20 months. We propose a literature review with 25 cases of intracerebral nerve sheath tumour identified, including the two current cases.
authors.affiliation: Département universitaire de radiothérapie, centre Paul-Strauss, Unicancer, 3, rue de la Porte-de-l'Hôpital, 67065 Strasbourg cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Le Fèvre
authors.affiliation: Département universitaire de radiothérapie, centre Eugène-Marquis, Unicancer, rue de la Bataille-Flandres-Dunkerque, 35042 Rennes cedex, France.
authors.forename: J
authors.initials: J
authors.lastname: Castelli
authors.affiliation: Service d'oncologie médicale, centre Eugène Marquis, Unicancer, rue de la Bataille-Flandres-Dunkerque, 35042 Rennes cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Perrin
authors.affiliation: Service de neurochirurgie, CHU Pontchaillou, rue Henri-Le-Guilloux, 35033 Rennes cedex 9, France.
authors.forename: P L
authors.initials: PL
authors.lastname: Hénaux
authors.affiliation: Département universitaire de radiothérapie, centre Paul-Strauss, Unicancer, 3, rue de la Porte-de-l'Hôpital, 67065 Strasbourg cedex, France; Laboratoire EA 3430, Fédération de médecine translationnelle de Strasbourg (FMTS), université de Strasbourg, 67000 Strasbourg, France. Electronic address: gnoel@strasbourg.unicancer.fr.
authors.forename: G
authors.initials: G
authors.lastname: Noël
mesh_terms: D000368:Aged; D001932:Brain Neoplasms / Q000473:pathology* / Q000628:therapy; D004172:Diplopia / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D008875:Middle Aged; D009362:Neoplasm Metastasis; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000628:therapy; D014839:Vomiting / Q000209:etiology
keywords: MINST; MPNST; Neurofibromatose type 1; Neurofibromatosis type 1; Schwannomes malins; Triton tumour; Tumeur de triton