A significant proportion of children with ependymoma can avoid radiotherapy with prolonged adjuvant chemotherapy. Deferring irradiation at the time of relapse did not compromise overall survival of the entire patient population.

BackgroundThe use of chemotherapy to manage newly diagnosed low grade glioma (LGG) was first introduced in the 1980s. One randomised trial has studied two- versus four-drug regimens with a duration of 12 months of treatment after resection.MethodsWithin the European comprehensive treatment strategy for childhood LGG, the International Society of Paediatric Oncology–Low Grade Glioma (SIOP LGG) Committee launched a randomised trial involving 118 institutions and 11 countries to investigate the addition of etoposide (100 mg/m2, days 1, 2 & 3) to a four-course induction of vincristine (1.5 mg/m2 × 10 wkly) and carboplatin (550 mg/m2 q 3 weekly) as part of 18-month continuing treatment programme. Patients were recruited after imaging diagnosis, resection or biopsy with progressive disease/symptoms. Some 497 newly diagnosed patients (M/F 231/266; median age 4.26 years (interquartile range (IQR) 2.02–7.06)) were randomised to receive vincristine carboplatin (VC) (n = 249) or VC plus etoposide (VCE) during induction (n = 248), stratified by age and tumour site.FindingsNo differences between the two arms were found in term of survival and radiological response. Response and non-progression rates at 24 weeks for VC and VCE, were 46% versus 41%, and 93% versus 91% respectively; 5-year Progression-Free Survival (PFS) and Overall Survival (OS) were 46% (StDev 3.5) versus 45% (StDev 3.5) and 89% (StDev 2.1) versus 89% (StDev 2.1) respectively. Age and diencephalic syndrome are adverse clinical risk factors for PFS and OS. 5-year OS for patients in early progression at week 24 were 46% (StDev 13.8) and 49% (StDev 16.5) in the two arms, respectively.InterpretationThe addition of etoposide to VC did not improve PFS or OS. High non-progression rates at 24 weeks justify retaining VC as standard first-line therapy. Infants with diencephalic syndrome and early progression need new treatments to be tested. Future trials should use neurological/visual and toxicity outcomes and be designed to discriminate between the impact on disease outcomes of ‘duration of therapy’ and ‘age at stopping therapy’.

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A significant proportion of children with OPT can avoid radiotherapy after prolonged chemotherapy. Deferring irradiation with chemotherapy protocols did not compromise overall survival of the entire population or visual function.

BACKGROUNDThe detection of hepatic nodules, particularly in patients treated for a previous malignancy, raises a diagnostic dilemma. Focal nodular hyperplasia (FNH) of the liver is an uncommon, benign tumor in children and must be differentiated from malignant hepatic lesions. The etiology of FNH is obscure, and its pathogenesis is poorly understood. FNH may be a reaction to localized vascular abnormalities and circulatory disturbances. The goal of the current study was to identify risk factors for the occurrence of FNH in children who had received prior treatment for a malignant tumor.METHODSThe current retrospective study examined 14 cases of FNH in pediatric patients who previously had been treated for a malignancy. Diagnosis was based on clinical and radiologic findings and was proven histologically in four cases.RESULTSFNH lesions were discovered by chance during routine examination in 78% of patients. The incidence of FNH was particularly high in the current series (0.45%) compared with the incidence in the general pediatric population. High doses of alkylating agents (e.g., busulfan or melphalan), venoocclusive disease, and liver radiotherapy may be responsible for injury to the vascular endothelium and subsequent localized circulatory disturbances. FNH is characterized by the absence of complications after its detection; therefore, only close follow‐up is recommended.CONCLUSIONSFNH appears to be a late complication of an iatrogenic vascular disease in children with a history of malignancy. Cancer 2003;97:3107–13. © 2003 American Cancer Society.DOI 10.1002/cncr.11452

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Despite moderate toxicity, objective response rates to temozolomide have been low, indicating that temozolomide has minimal activity in the high-grade gliomas of childhood.

Occlusive vasculopathy is a potential complication of radiotherapy in children with optic pathway glioma. With a median follow‐up of 7 years, 13 of 69 children in this study developed clinical and radiological signs of occlusive vasculopathy after radiotherapy within a median interval of 36 months. The major risk factor was neurofibromatosis type 1. Radiotherapy should no longer be the first treatment in these settings. When radiotherapy is unavoidable, regular screening for cerebral vasculopathy is mandatory, as preventive treatment is available. Ann Neurol 1999;45:393–396