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Favorable prognosis in pediatric brainstem low-grade glioma: Report from the German SIOP-LGG 2004 cohort

International Journal of Cancer Dec 11, 2019

Holzapfel J, Kandels D, Schmidt R, et al. - Using the prospectively registered, population-based German SIOP-LGG 2004 cohort, researchers examined the natural history of pediatric low-grade glioma (LGG) of the caudal brainstem in 116 patients (age 0.2–16.5 years, 10% neurofibromatosis type 1) who had received the diagnosis of LGG of the pons (27%) and medulla oblongata (73%). They also investigated if brainstem location proved unfavorable for survival within the framework of the comprehensive SIOP-LGG 2004 management strategy. No adjuvant treatment was given to 59 patients following biopsy (23%), variable resection (63%) or radiologic diagnosis only (14%). Chemo- (n = 39) or radiotherapy (n = 18) was prompted by radiologic progression or severe neurologic symptoms. Salvage treatments were used, after further progression (28/57), including multiple treatment lines for 12/28 patients. A fall to 0.40 was noted in 5-years event-free survival, while 5-years overall survival was estimated to be 0.95. High overall survival for pediatric brainstem LGG was achieved with the treatment algorithm used. In this often-chronic disease, additional postoperative neurologic deficits were increased but not overall survival as a result of extensive neurosurgical resection. Among all patients, more than half could be safely followed by observation, progressive tumors could be controlled by multimodal adjuvant treatment. Low-grade diagnosis should be verified by molecular evaluation, which may identify patterns prognostic for malignant evolution.
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