The management of desmoplastic neuroepithelial tumours in childhood

The authors report on the clinicopathological aspects of and management str ategies for the group of rare, large hemispheric childhood rumours recently classified as desmoplastic infantile ganglioglioma (DIGG), desmoplastic as trocytoma of infancy (DACI) and pleomorphic xanthoastrocytoma (PXA). Betwee n 1985 and 1997, ten children (4 with DACIs, it with DIGGs and 2 with PXAs) with a median age of 9.5 months were operated on. All these patients had c omplete surgical resections, with two having a preoperative biopsy. This le d to an erroneous diagnosis in both cases of malignant grade 4 astrocytoma. As a result, one patient had preoperative chemotherapy with no effect. The re was one perioperative death. Histology revealed heterogeneous tumours wi th 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 rumours 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 erron eous diagnosis and subsequent mismanagement.