Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: a case report and review of the literature.
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.
Drago
G
G
Department of Pediatrics, University Hospital Center of Grenoble, France.
Pasquier
B
B
Pasquier
D
D
Pinel
N
N
Rouault-Plantaz
V
V
Dyon
J F
JF
Durand
C
C
Armari-Alla
C
C
Plantaz
D
D
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