The management of desmoplastic neuroepithelial tumours in childhood

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