Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: A case report and review of the literature

• Published in: Medical and pediatric oncology 1997-03, Vol.28 (3), p.216-222
• Main Authors: Drago, Gisela, Pasquier, Basile, Pasquier, Dominique, Pinel, Nicole, Rouault-Plantaz, Veronique, Dyon, Jean-François, Durand, Chantal, Armari-Alla, Corinne, Plantaz, Dominique
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Child; Female; ganglioneuroma; Ganglioneuroma - diagnostic imaging; Ganglioneuroma - pathology; Humans; malignant peripheral nerve sheath tumor; Medical sciences; Neoplasms, Multiple Primary - diagnostic imaging; Neoplasms, Multiple Primary - pathology; Nerve Sheath Neoplasms - diagnostic imaging; Nerve Sheath Neoplasms - pathology; neuroblastoma; Neurology; Radiography; Schwann cell; Thoracic Neoplasms - diagnostic imaging; Thoracic Neoplasms - pathology; Tumors of the nervous system. Phacomatoses
• ISSN: 0098-1532; 1096-911X
• DOI: 10.1002/(SICI)1096-911X(199703)28:3<216::AID-MPO13>3.0.CO;2-C

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. Med. Pediatr. Oncol. 28:216–222 © 1997 Wiley‐Liss, Inc.