Tsai Ming Horng, Wong Alex Mun-Ching, Jaing Tang-Her, Wang Huei-Shyong, Hsueh Chuen, Wu Chieh-Tsai
Division of Hematology and Oncology, Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan, Taiwan.
J Pediatr Hematol Oncol. 2009 Nov;31(11):832-4. doi: 10.1097/MPH.0b013e3181acd842.
The authors discuss the current management for cerebellopontine angle (CPA) tumors in children. CPA tumors accounted for 1% to 3% of intracranial tumors in children. There had been much controversy with the management of these tumors. A total of 29 eligible patients were enrolled to the study and 5 patients had multiple lesions at diagnosis. Eight patients with tumors exclusively confined in the CPA. Sixteen patients with tumors occurred predominantly within CPA and 5 arising from the vicinity and growing mainly into the CPA. Twelve tumors were located in the right CPA (41%) and 5 (17%) on the left. Thirteen of the 29 patients developed hydrocephalus and 3 required placement of a shunt. Lesions of the CPAs were divided into those native to the angle and those extending to the angle from adjacent structures. Gross total removal was achieved in 9 cases, subtotal in 14, and 2 had biopsies only. Four patients were diagnosed with pontine glioma solely by magnetic resonance imaging without histologic confirmation. Two died soon after the operation. Ten patients died with a mortality rate of 34.5%. The median follow-up in this study was 38 months (range: 4 to 225 mo). The CPA is a rare location for lesions in children, with clear predominance on the right side. Although low-grade lesions are more frequent, the histology varies widely and is limited by the lack of radiologic-pathologic correlation.