Outcome of craniofacial surgery in children and adolescents with malignant tumors involving the skull base: an international collaborative study.

Abstract

The aim of the study was to characterize the distribution of anterior skull base (ASB) cancers in the pediatric population and to identify predictors of outcome. This was an international study of 1307 patients undergoing craniofacial surgery for malignant tumors; 6.4% (n = 84) among these were <or=21 years old. The most common histologic type was sarcoma (40%), followed by squamous cell carcinoma (14%). Higher incidence of tumors was found in older children (7.5-21 years) compared to younger children. The 5-year disease-specific and overall survival rates were 61% and 55%, respectively (median follow-up of 30 months). The best prognosis was associated with low-grade sarcomas and the worst with salivary and squamous cell carcinomas. High-grade sarcoma represented an intermediate risk group. On multivariate analysis, prior radiotherapy and histology were independent predictors of survival. Surgery for malignant tumors involving the ASB is feasible and safe in children. Histology is significant determinant of poor outcome in this population.