Cervicomedullary tumors in children: A distinct subset of brainstem gliomas

We reviewed the clinical course of 17 children who underwent surgical resection of an intra-axial cervicomedullary tumor between 1980 and 1992. The clinical symptoms, which reflected medullary dysfunction in nine children and cervical cord deficits in eight, were present for a mean of 2.1 years before diagnosis (range, 2 months to 7.5 years), and for at least 1 year in 80' of the patients. Neurodiagnostic imaging (MRI in 14, CT in 3) showed the tumor epicenter in the medulla in 11 and in the upper cervical cord in six. Surgery was performed for newly diagnosed tumor in 11 children, and for progressive disease in six who had received prior radiotherapy. The surgical resection was gross total in two and partial (60 to 95') in fifteen. Fifteen patients had low-grade glial tumors (10 astrocytomas, four gangliogliomas, and one mixed glioma), and two had anaplastic gangliogliomas. Four-year progression-free and total survival rates after surgery for patients who had surgery as initial therapy were 70 and 100' for those who had surgery at the time of progression, these were 41 and 62%. Postsurgical neurologic complications occurred in five children. Four of these children had received prior radiotherapy. Two of them already had severe preoperative deficits and three had moderate deficits that worsened after surgery. Twelve patients with mild deficits were unchanged or improved postoperatively. These observations confirm that intra-axial tumors arising at the cervicomedullary junction are a distinct subset of brainstem gliomas that generally have an indolent course and low-grade glial pathology; they are amenable to surgical resection and have a better prognosis than typical high-grade pontine brainstem lesions. With respect to these characteristics, they behave much more like pediatric spinal cord tumors. Although the surgical approach to these tumors was not without morbidity, it appeared to contribute to relatively long, good-quality survival in a large percentage of these children, even when it was done for progressive disease. We could not determine the role of radiotherapy from this series.