title: Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: a case report and review of the literature.
abstract: A case of a "de novo" ganglioneuroma showing a large area of malignant peripheral nerve sheath tumor (MPNST) is described. The tumor arose in an 11.5-year-old girl with neither stigmata nor family history of von Recklinghausen's neurofibromatosis. In addition, the patient had no previous history of a neuroblastoma or radiation therapy. This report provides new evidence that, although rare, the spontaneous development of an MPNST in a benign ganglioneuroma can occur. Immunohistochemical and electron microscopy studies supported the finding that the spindle cell component was of nerve sheath origin.
authors.affiliation: Department of Pediatrics, University Hospital Center of Grenoble, France.
authors.forename: G
authors.initials: G
authors.lastname: Drago
authors.forename: B
authors.initials: B
authors.lastname: Pasquier
authors.forename: D
authors.initials: D
authors.lastname: Pasquier
authors.forename: N
authors.initials: N
authors.lastname: Pinel
authors.forename: V
authors.initials: V
authors.lastname: Rouault-Plantaz
authors.forename: J F
authors.initials: JF
authors.lastname: Dyon
authors.forename: C
authors.initials: C
authors.lastname: Durand
authors.forename: C
authors.initials: C
authors.lastname: Armari-Alla
authors.forename: D
authors.initials: D
authors.lastname: Plantaz
mesh_terms: D002648:Child; D005260:Female; D005729:Ganglioneuroma / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000473:pathology; D018317:Nerve Sheath Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D013899:Thoracic Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology