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Pediatric low-grade gliomas (PLGG) are commonly treated with a combination of surgery, radiotherapy, and chemotherapy. Recent trends prioritize reducing long-term morbidities, particularly in younger patients. While historically chemotherapy was reserved for cases progressing after radiotherapy, evolving recommendations now advocate for its early use, particularly in younger age groups. The carboplatin and vincristine (CV) combination stands as a standard systemic therapy for PLGG, varying in dosage and administration between North America and Europe. Clinical trials have shown promising response rates, albeit with varying toxicity profiles. Vinblastine has emerged as another effective regimen with minimal toxicity. TPCV, a regimen combining thioguanine, procarbazine, lomustine, and vincristine, was compared to CV in a Children's Oncology Group trial, showing comparable outcomes, but more toxicity. Vinorelbine, temozolomide, and metronomic chemotherapy have also been explored, with varied success rates and toxicity profiles. Around 40-50% of PLGG patients require subsequent chemotherapy lines. Studies have shown varied efficacy in subsequent lines, with NF1 patients generally exhibiting better outcomes. The identification of molecular drivers like BRAF mutations has led to targeted therapies' development, showing promise in specific molecular subgroups. Trials comparing targeted therapy to conventional chemotherapy aim to delineate optimal treatment strategies based on molecular profiles. The landscape of chemotherapy in PLGG is evolving, with a growing focus on molecular subtyping and targeted therapies. Understanding the role of chemotherapy in conjunction with novel treatments is crucial for optimizing outcomes in pediatric patients with low-grade gliomas., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Intoduction: Diffuse leptomeningeal glioneuronal tumors (DLGNTs) pose a rare and challenging entity within pediatric central nervous system neoplasms. Despite their rarity, DLGNTs exhibit complex clinical presentations and unique molecular characteristics, necessitating a deeper understanding of their diagnostic and therapeutic nuances., Methods: This review synthesizes contemporary literature on DLGNT, encompassing epidemiology, clinical manifestations, pathological features, treatment strategies, prognostic markers, and future research directions. To compile the existing body of knowledge on DLGNT, a comprehensive search of relevant databases was conducted., Results: DLGNT primarily affects pediatric populations but can manifest across all age groups. Its diagnosis is confounded by nonspecific clinical presentations and overlapping radiological features with other CNS neoplasms. Magnetic resonance imaging (MRI) serves as a cornerstone for DLGNT diagnosis, revealing characteristic leptomeningeal enhancement and intraparenchymal involvement. Histologically, DLGNT presents with low to moderate cellularity and exhibits molecular alterations in the MAPK/ERK signalling pathway. Optimal management of DLGNT necessitates a multidisciplinary approach encompassing surgical resection, chemotherapy, radiotherapy, and emerging targeted therapies directed against specific genetic alterations. Prognostication remains challenging, with factors such as age at diagnosis, histological subtypes, and genetic alterations influencing disease progression and treatment response. Long-term survival data are limited, underscoring the need for collaborative research efforts., Conclusion: Advancements in molecular profiling, targeted therapies, and international collaborations hold promise for improving DLGNT outcomes. Harnessing the collective expertise of clinicians, researchers, and patient advocates, can advance the field of DLGNT research and optimize patient care paradigms., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: Currently, the BRAF status of pediatric low-grade glioma (pLGG) patients is determined through a biopsy. We established a nomogram to predict BRAF status non-invasively using clinical and radiomic factors. Additionally, we assessed an advanced thresholding method to provide only high-confidence predictions for the molecular subtype. Finally, we tested whether radiomic features provide additional predictive information for this classification task, beyond that which is embedded in the location of the tumor., Methods: Random forest (RF) models were trained on radiomic and clinical features both separately and together, to evaluate the utility of each feature set. Instead of using the traditional single threshold technique to convert the model outputs to class predictions, we implemented a double threshold mechanism that accounted for uncertainty. Additionally, a linear model was trained and depicted graphically as a nomogram., Results: The combined RF (AUC: 0.925) outperformed the RFs trained on radiomic (AUC: 0.863) or clinical (AUC: 0.889) features alone. The linear model had a comparable AUC (0.916), despite its lower complexity. Traditional thresholding produced an accuracy of 84.5%, while the double threshold approach yielded 92.2% accuracy on the 80.7% of patients with the highest confidence predictions., Conclusion: Models that included radiomic features outperformed, underscoring their importance for the prediction of BRAF status. A linear model performed similarly to RF but with the added benefit that it can be visualized as a nomogram, improving the explainability of the model. The double threshold technique was able to identify uncertain predictions, enhancing the clinical utility of the model., Clinical Relevance Statement: Radiomic features and tumor location are both predictive of BRAF status in pLGG patients. We show that they contain complementary information and depict the optimal model as a nomogram, which can be used as a non-invasive alternative to biopsy., Key Points: • Radiomic features provide additional predictive information for the determination of the molecular subtype of pediatric low-grade gliomas patients, beyond what is embedded in the location of the tumor, which has an established relationship with genetic status. • An advanced thresholding method can help to distinguish cases where machine learning models have a high chance of being (in)correct, improving the utility of these models. • A simple linear model performs similarly to a more powerful random forest model at classifying the molecular subtype of pediatric low-grade gliomas but has the added benefit that it can be converted into a nomogram, which may facilitate clinical implementation by improving the explainability of the model., (© 2023. The Author(s), under exclusive licence to European Society of Radiology.)

Purpose: Cancer survivors treated with stem-cell transplant (SCT) and radiation therapy are at a high risk for late effects including the metabolic syndrome. This study reviewed the prevalence of the metabolic syndrome in pediatric central nervous system (CNS) tumor survivors treated with autologous SCT and craniospinal radiation., Methods: A prospective, cross-sectional study in pediatric CNS tumor patients, who underwent a one-time evaluation at least 18 months post-autologous SCT for the presence of components of metabolic syndrome: obesity, hypertension, hyperlipidemia, and abnormal glucose levels., Results: Twelve patients were evaluated, and two (16%) met full criteria for the metabolic syndrome. Seven patients (58%) had at least one component of metabolic syndrome: elevated glucose levels in 8% (1/12), obesity 17% (2/12), hypertriglyceridemia 17% (2/12), and reduced HDL cholesterol in 25% (3/12). None had hypertension. Nine patients (75%) demonstrated abnormal fasting lipid profiles with elevated total cholesterol levels, although only 25% (3/12) fulfilled criteria for a diagnosis of dyslipidemia., Conclusion: Pediatric CNS tumor survivors treated with autologous SCT and craniospinal radiation are at risk for early signs of metabolic syndrome, most commonly hyperlipidemia. Further studies evaluating the progression of these early signs to full criteria for the metabolic syndrome diagnosis are required.

Dissemination of diffuse intrinsic pontine glioma (DIPG) outside the central nervous system is exceptional. Here, we present a child diagnosed with DIPG who developed seeding along the track of the ventriculoperitoneal shunt and review the literature on this unusual occurrence.

Background: Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few., Patients and Methods: The study provides a retrospective chart review of pediatric patients <18 years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan., Results: Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4 years (0.9-16.4 years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years' survival was 87 ± 7% with a median follow-up of 4.5 years (0.3-12.3 years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community., Conclusions: Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

Diffuse intrinsic pontine glioma (DIPG) is an aggressive infiltrative glioma for which no curative therapy is available. Radiation therapy (RT) is the only potentially effective intervention in delaying tumor progression, but only transiently. At progression, re-irradiation is gaining popularity as an effective palliative therapy. However, at second progression, exclusive symptomatic treatment is usually offered. Here we report two patients with DIPG at second progression who were treated with a second re-irradiation course with good response. Importantly, treatment was well tolerated with no irradiation associated acute toxicity identified.

Hemispheric low-grade gliomas account for the second most common location in pediatric low-grade gliomas (PLGGs) after the cerebellum. The pathological spectrum includes gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), diffuse astrocytomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas (PXAs), among others. Clinically, hemispheric PLGGs represent a well-recognized cause of intractable epilepsy in children and adolescents. With an excellent long-term outcome, surgery remains the cornerstone and patients with gross total resection typically do not need any further therapies. The recent literature about hemispheric PLGGs was reviewed to provide an up-to-date overview of the molecular and cell biology of these tumors. Hemispheric PLGGs can harbor multiple alterations involving BRAFV600E, FGFR, NTRK, MYB/MYBL1, IDH, and BRAF-KIAA1549 fusions. However, the clinical significance of most of these alterations is still to be defined. The role of RAS/MAPK mutations and other alterations in hemispheric PLGGs is of interest from diagnostic, prognostic, and therapeutic perspectives. Molecular testing for these tumors should be encouraged, since the findings can have an important impact not only in prognosis but also in therapeutic strategies.

Synchronous primary malignant brain tumors are rare. We present a 5-year-old boy with synchronous glioblastoma and medulloblastoma. Both tumor samples had positive p53 stain and loss of PMS2 and MLH1 stains. The child had multiple café au lait spots and a significant family history of cancer. After subtotal resection of both tumors, he received craniospinal radiation with concomitant temozolomide followed by chemotherapy, alternating cycles of cisplatin/lomustine/vincristine with temozolomide. Then, he started maintenance treatment with cis-retinoic acid (100 mg/m(2)/day for 21 days). He remained asymptomatic for 34 months despite a follow-up brain MRI consistent with glioblastoma relapse 9 months before his death. Cis-retinoic acid may have contributed to prolong survival in this child with a probable biallelic mismatch repair syndrome.

Tumors of the posterior fossa (PF) account for up to 60 % of all childhood intracranial tumors. Over the last decades, the mortality rate of children with posterior fossa tumors has gradually decreased. While survival has been the primary objective in most reports, quality of survival increasingly appears to be an important indicator of a successful outcome. Children with a PF tumor can sustain damage to the cerebellum and other brain structures from the tumor itself, concomitant hydrocephalus, the consequences of treatment (surgery, chemotherapy, radiotherapy), or a combination of these factors. Together, these contribute to long-term sequelae in physical functioning, neuropsychological late outcomes (including academic outcome, working memory, perception and estimation of time, and selective attention, long-term neuromotor speech deficits, and executive functioning). Long-term quality of life can also be affected by endocrinological complication or the occurrence of secondary tumors. A significant proportion of survivors of PF tumors require long-term special education services and have reduced rates of high school graduation and employment. Interventions to improve neuropsychological functioning in childhood PF tumor survivors include (1) pharmacological interventions (such as methylphenidate, modafinil, or donepezil), (2) cognitive remediation, and (3) home-based computerized cognitive training. In order to achieve the best possible outcome for survivors, and ultimately minimize long-term complications, new interventions must be developed to prevent and ameliorate the neuro-toxic effects experienced by these children.

Introduction: Information about pediatric survivors of brain tumors in developing countries is scant., Purpose: In this study, we aimed to investigate the availability of resources for treatment and long-term follow-up of children with central nervous system tumors in developing countries., Materials and Methods: A web-based questionnaire on available services and follow-up of brain tumor survivors was posted at www.cure4kids.org , and registered users were invited to participate., Results: A total of 140 evaluable responses from developing countries (n = 103) and high-income countries (n = 37) were obtained. There was a significant correlation between gross national income and the availability of services for treatment and follow-up and between patient load and the availability of some services., Conclusion: The resources for treatment and long-term follow-up of children with brain tumors need to be improved in developing countries.

Background: Tumors of the spinal cord are exceedingly rare in infancy and only a paucity of literature exists describing the spectrum of this disease and its management. The objectives of our study were to describe the demographic characteristics of spinal cord tumors (SCT) in children less than 3 years of age at diagnosis and to review their treatment and outcome., Methods: A national retrospective chart review was conducted on patients under the age of 3 years diagnosed with a primary tumor of the central nervous system (CNS) between 1990 and 2005 across Canada. Inclusion criteria were: age ≤ 3 years, histologic confirmation of the diagnosis, and residency in Canada. A centralized database was created and information regarding SCT was extracted., Results: Twenty-five of five hundred seventy-nine patients (4.3%) in the data bank had a SCT. The majority of tumors were low-grade astrocytomas (14/25). Leptomeningeal dissemination based on neuroradiologic imaging and/or cerebrospinal fluid cytology was present in five (20%) patients. The majority of patients underwent an incomplete surgical resection (52%). Most patients (64%) did not receive postoperative radiotherapy or chemotherapy. Seventy-two percent (18/25) developed recurrent/progression of disease. Overall 2- and 5-year survival for low- and high-grade malignancies was 93 ± 6.4% and 37.5 ± 17.1% respectively. Significant predictors of survival included mean duration of symptoms prior to initial diagnosis and recurrence/progression of disease., Conclusions: Relapse/progression of disease in infant SCT is frequent. Prolonged survival of low-grade tumors is possible with further therapy; however, the prognosis of high-grade malignancies remains poor.

Background: Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors., Methods: The CPT population from the Canadian databank of CNS tumor in children ≤ 36 months diagnosed between 1990 and 2005 was reviewed, Results: Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children < 3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC., Conclusion: By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy.

Prior to resection of a cerebral brain tumor, mapping of the functional and structural anatomy of the adjacent tissue is essential to reduce the risk of damage to descending and ascending pathways. We investigated the effectiveness of concurrent magnetoencephalography (MEG) and diffusion tensor imaging (DTI) tractography to delineate the motor cortex and associated corticospinal tract (CST) in a case series of children with brain tumors seen for pre-surgical evaluation. Using activation points generated from MEG to launch tractography, we delineated the CST of four patients and eight control subjects. Displacement of the CST was considerably larger in children with tumors located in the center of the hemisphere than in children whose tumors were more posteriorly located. Our findings suggest that the use of concurrent MEG and DTI may be an effective tool in the pre-surgical evaluation of eloquent cortex and associated white matter tracts in pediatric brain tumor patients.

Introduction: Chemotherapy has limited role in the up-front management of ependymoma. At the time of recurrence, the role of chemotherapy is also ill defined and the choice of chemotherapeutic agents is often arbitrary, based on anecdotal data and personal experience., Methods: The purpose of this review is to describe and critically analyze the published literature on chemotherapy in patients with recurrent and metastatic ependymoma., Discussion: The disappointing response rate with single agents (12.9%) and combinations (17.4%) emphasizes the need to re-evaluate the current chemotherapeutic approach of intracranial ependymoma, and biological studies are needed to identify targets that may be considered for clinical trials.

Introduction: Some children with optic pathway gliomas present with proptosis related to intraorbital tumor extension. The radiological assessment of chemotherapeutic response in these patients can be complicated by irregular tumor shape and lack of relation between tumor volume and cosmetic effect., Method: We propose that proptosis measurements and derivation of a proptosis index can be a useful adjunct to the measurement of tumor volume in the radiological assessment of chemotherapeutic response. The proptosis index was derived as the ratio of the difference in proptosis between eyes postchemotherapy to that prechemotherapy. A series of six patients with proptosis and the diagnosis of an optic nerve tumor from an optic pathway glioma registry demonstrate by case example the correlation between the proptosis index and the clinical and radiographic response to chemotherapy., Conclusions: We have found that a proptosis index <1 correlates with a chemotherapeutic maintained response and an index >1 correlates with progressive disease.

Background: Internet information, now available in many different languages, can become a major source of information for patients and families in their own mother tongue. Chinese represent one of most frequently spoken language in the world. The aims of this study were to critically appraise the quantity and quality of Internet health information in childhood brain tumour in the Chinese language and to identify sufficient quality websites that can potentially be recommended to Chinese-speaking parents., Methods: Internet information on six common paediatric brain tumours was searched using six commonly used Chinese search engines. Websites were assessed systematically using two rating tools: DISCERN instrument and Checklist Rating System Instrument., Results: Out of 946 sites accessed, only 13 assessable Chinese websites, displaying Traditional Chinese characters and providing information on brain tumour, were identified. Only four sites included specific discussion on brain tumours in children. Ten websites failed to provide satisfactory information on brain tumour as rated by DISCERN instrument. Overall only 41% of the 13 specific items relevant to brain tumours were mentioned (Checklist Rating System Instrument)., Conclusion: Only a few satisfactory websites can be recommended to Chinese-speaking families for general information on brain tumour with caution from health care providers that such information may not apply to the child's individual condition.

Patients and Methods: The clinical, radiological, surgical, and pathological findings of 16 children with a primary central nervous system (CNS) sarcoma are reported. There were 8 (50%) girls and 8 (50%) boys ranging in age from 4 months to 14 years (mean age 4.8 years). Four patients (23%) were in their 1st year of life. Fourteen children (87%) had an intracranial sarcoma, and 2 (13%) had intraspinal tumors. Nine intracranial tumors (60%) were supratentorial. The parietal and temporal regions were the most frequently involved sites., Results: Characteristic imaging findings included tumor cysts in 7 patients and marked tumoral enhancement in 9 (69%) with intratumoral calcification and hemorrhage. All patients underwent at least one operation to surgically remove the tumor with the aim of maximal resection and 3 patients underwent a second resection due to a recurrent tumor. Resection was total in 9 (53%) patients and subtotal in another 7 (41%). Dural attachment by tumor was confirmed in 7 (44%) patients and parenchymal invasion was present in 9 (56%). In one-third of the patients there was a well-defined plane of dissection around the tumor. Postoperative radiation was used in 10 patients. Postoperative chemotherapy was used in all but 2 patients. Immunohistochemical studies were available in 13 patients with the most consistent finding being strong vimentin positivity. Five out of the 6 patients in whom the proliferation markers were obtained demonstrated a high proliferation index (Ki-67 labeling index, 20-50%). The mean length of survival in the group was 4.6 years (range 1 month to 16 years). Children who presented in the 1st year of life had shorter survival than those who presented at an older age. Six patients (40%) had cerebrospinal fluid (CSF) dissemination of the tumor. CSF dissemination was associated with a shorter mean survival of 1.9 years., Conclusions: Our review of this series of patients indicates the requirement for adjuvant therapy and for continued efforts to classify tumor subtypes aimed at optimizing future treatments for patients with a primary CNS sarcoma.

Dramatic advances have been made in the diagnosis of childhood brain tumours thanks to the development of modern imaging techniques. Advances in the management of these tumours have, however, been slow because of the limitations of an aggressive surgical approach and the risks associated with radiotherapy on the growing, and still immature, brain. The role of chemotherapy remains ill-defined in many patients with brain tumours and large variations in practice exist between groups and institutions. This article provides an overview of the most common paediatric brain tumours, mainly gliomas, medulloblastomas, ependymomas, germ-cell tumours and craniopharyngiomas. Considerations regarding the management of brain tumours in very young children are also examined. The long term outcome for children with brain tumours is discussed, stressing the need to focus on quality of life for survivors.

Chemotherapy has been used extensively in the management of children with intracranial ependymoma. In this review, we discuss the results of phase II studies and clinical trials conducted in newly diagnosed and recurrent ependymomas. There is little evidence that chemotherapy is effective in this tumour. The response rate to single agents is 11%, with less than 5% complete responses, cisplatin being the most active agent in phase II studies. Combinations may be more effective, although the response rate with high-dose regimens is disappointing. Early results of protocols conducted in infants and young children do not suggest that chemotherapy is beneficial. A more rigorous assessment of chemotherapy is required in order to define its role in patients with intracranial ependymomas. Indeed, it is difficult to justify the use of chemotherapy outside such studies. More large studies, perhaps intergroup, limited to children with ependymomas would be of particular value.

Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.

We report a case of a malignant rhabdoid tumor of the kidney (MRTK) associated with a cerebellar tumor. Diagnosis was confirmed before neoadjuvant chemotherapy by a percutaneous fine-needle biopsy of the abdominal tumor. The clinical and radiologic features of this rare association of childhood neoplasms are reviewed.

Over a 9-year period, 35 out of 614 children with malignant tumours who were treated at the Centre Léon Bérard developed spinal metastases. Of these, 18 with known malignancies before the development of spinal cord compression are reviewed. The most common tumours causing spinal metastases were Ewing's sarcoma, neuroblastoma and renal tumours. Cord compression occurred 5-88 months after the diagnosis of systemic cancer. The median interval from first symptoms to the diagnosis of compression was 17 days. There were 16 patients with neurological deficit, including 5 with paraplegia. Specific imaging procedures were performed in 16 patients. Treatment included operation in 8 patients, followed by chemotherapy (6 patients) and/or radiotherapy (4 patients); 9 of the 10 non-operated patients received radiotherapy. Only 6 patients had a significant neurological improvement. All patients but 1 died within a median time of 2 months. Early diagnosis might prevent permanent disability in these children with a short survival expectancy.

The cases of 15 children operated on for cerebral pure oligodendroglioma were studied. Two groups of children were distinguished. Group I children presented with epilepsy (7 cases); their tumor was histologically benign in all cases. These children are all alive after a median follow-up of 72 months: however, 2 of them presented with a local recurrence, which was operated on, and are actually disease-free. Group 2 children presented with intracranial hypertension (8 cases); the tumor was anaplastic in 7 cases. Despite postoperative radiotherapy and chemotherapy 6 children died; their median survival time was 17 months. The present series shows the existence of a clear correlation between clinical presentation, histological grading and survival in childhood cerebral oligodendrogliomas.

To estimate the side effects of chemotherapy and the influence of age at the time of nephrectomy on renal function, we investigated renal function in 34 uninephrectomised children with neuroblastoma (NB) or Wilms' tumour (WT). The results were compared with 6 controls who underwent nephrectomy for non-malignant disease. Study of renal function was primarily based on the clearance of inulin and para-aminohippuric acid (Cin and CPAH, ml/min per 1.73 m2). No significant differences in Cin/CPAH (mean +/- SD) were found between the NB group (90 +/- 24/421 +/- 95), WT group (85 +/- 17/386 +/- 104) and the controls (93 +/- 13/430 +/- 61). Children with NB and WT were divided into two subgroups according to the theoretical nephrotoxic risk. There was no significant difference in renal function between NB or between WT subgroups. Cumulative cisplatin doses in children with NB did not affect renal function significantly. The age at time of unilateral nephrectomy (< or = 2 years vs. > 2 years) was not associated with a higher risk of renal damage in WT children, whereas in NB children the filtration fraction (Cin:CPAH) was higher in younger children (mean +/- SD: 0.243 +/- 0.023 vs. 0.191 +/- 0.041). In conclusion, uninephrectomised children with NB are supposed to have a higher risk of drug-induced renal impairment compared with those with WT. Our data do not confirm this hypothesis, since renal function was comparable to controls in both groups, except in younger patients with NB who show a high filtration fraction. Since the survival of children with NB has improved, a longer follow-up of their renal function in needed.

Recombinant interleukin-2 (rIL-2) is a new promising treatment for cancer, but is associated with severe renal toxicity. This study is the first to analyse the renal effects of rIL-2 in children. Twenty-one cycles of continuous rIL-2 infusion were studied in 15 patients; mean age was 6.9 years and average weight 18.9 kg. Interstitial fluid retention and oliguria (baseline, 1.7 ml/kg per hour; nadir, 0.5 mg/kg per hour) were associated with hypotension (baseline, 101/56 mm Hg; nadir, 85/43 mm Hg) and decreased intravascular volume (plasma renin activity increased x 10). Weight gain (+7.9%) was observed in 13 cycles whereas weight loss (-6.3%) was shown in 8 cycles because of digestive and cutaneous losses, mainly in the youngest patients. This prerenal azotaemia was characterized by a decrease in creatinine clearance (from 101 to 36 ml/min per 1.73 m2) and a low fractional excretion of sodium (FENa) (from 0.70% to 0.09%). Hypotension and hypovolaemia needed vascular filling (n = 12), dopamine (n = 7) and interruption of rIL-2 (n = 2). Most abnormalities occurred as early as day 2 of therapy and were always reversible after a short period with sodium leakage (diuresis = 2.2 ml/kg per hour, FENa = 2.01%). Hypophosphataemia was associated with low urinary excretion of phosphorus, suggesting an increased uptake of inorganic phosphorus by rapidly proliferating lymphoid cells.