The management of desmoplastic neuroepithelial tumours in childhood

摘要: The authors report on the clinicopathological aspects of and management strategies for group rare, large hemispheric childhood tumours recently classified as desmoplastic infantile ganglioglioma (DIGG), astrocytoma infancy (DACI) pleomorphic xanthoastrocytoma (PXA). Between 1985 1997, ten children (4 with DACIs, 4 DIGGs 2 PXAs) a median age 9.5 months were operated on. All these patients had complete surgical resections, two having preoperative biopsy. This led to an erroneous diagnosis in both cases malignant grade astrocytoma. As result, one patient chemotherapy no effect. There was perioperative death. Histology revealed heterogeneous looking areas 8 specimens. None has any postoperative adjuvant treatment. surviving are alive at follow-up (median 4.2 years). Despite their often appearance, have excellent prognosis, but they can present formidable challenges when occur very young patients. We believe that excision offer cure treatment is not necessary. Finally, biopsy little value may even lead subsequent mismanagement.