| Childhood Brain Tumours |
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Brain tumours are the most common solid tumour of childhood. Some are benign others are malignant. There are a number of different types of brain tumour; how they are classified depends on the histology and location within the brain. This page contains links to information specifically related to Childhood Brain Tumours, other relevant resources are availible via the Main Menu of Children's Cancer Web.
Menu: Childhood Brain Tumours
Information for Patients and Family
Information for Health Professionals / Researchers
Latest Research Publications
Childhood Astrocytoma
Childhood Brain Stem Glioma
Childhood Craniopharyngioma
Childhood Ependymoma
MedulloblastomaInformation Patients and Family (10 links)
- Pediatric Brain Tumors
Comer Children's Hospital at the University of Chicago
Pediatric oncologist and brain tumor expert Charles M. Rubin, MD, describes the diagnosis, treatment and recovery for pediatric brain tumors. - Childhood Brain and Spinal Cord Tumors - Overview
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - Childhood CNS Atypical Teratoid/Rhabdoid Tumor - Treatment
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - Childhood CNS Germ Cell Tumors - Treatment
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - American Brain Tumor Association
ABTA
A national nonprofit organisation founded in 1973 to advance the understanding and treatment of brain tumors with the goals of improving, extending and, ultimately, saving the lives of those impacted by a brain tumor diagnosis. - Brain Tumors (Central Nervous Tumors)
Childrens' Oncology Group
Includes information, with sections on newly diagnosed, in treatment and after treatment. - Children's Brain Tumour Research Centre
Nottingham University
The Centre set up in 1991 to provide a centre of excellence for the treatment and study of tumours of the brain and spine in children. - HeadSmart campaign
HeadSmart
The aim of the HeadSmart campaign is to reduce the time it takes to diagnose children and young people with brain tumours in the UK by educating healthcare professionals and the public about the symptoms of brain tumours in children and young people. - Jargon buster
The Brain Tumour Charity
list of the terms and words related to brain tumours - The Brain Tumour Charity
The Brain Tumour Charity
A UK charity founded in1996 which funds scientific and clinical research into brain tumours and offers information and support to those affected, whilst raising awareness and influencing policy.
Information for Health Professionals / Researchers (5 links)
- PubMed search for publications about Brain Tumours, Childhood - Limit search to: [Reviews]
PubMed Central search for free-access publications about Brain Tumours, Childhood
US National Library of Medicine
PubMed has over 22 million citations for biomedical literature from MEDLINE, life science journals, and online books. Constantly updated. - Childhood Brain and Spinal Cord Tumors - Overview
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - Childhood CNS Atypical Teratoid/Rhabdoid Tumor - Treatment
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - Childhood CNS Germ Cell Tumors - Treatment
National Cancer Institute
PDQ summaries are written and frequently updated by editorial boards of experts Further info. - CNS and Miscellaneous Intracranial and Intraspinal Neoplasms
SEER, National Cancer Institute
Part of a SEER report on statistical trends and risk factors associated with childhood cancers. From: Cancer Incidence and Survival Among Children and Adolescents: United States SEER Program 1975-1995. (PDF)
Latest Research Publications
This list of publications is regularly updated (Source: PubMed).
H3F3A K27M mutation in pediatric CNS tumors: a marker for diffuse high-grade astrocytomas.
Am J Clin Pathol. 2013; 139(3):345-9 [PubMed]
Treatment of children with glioblastoma with conformal radiation, temozolomide, and bevacizumab as adjuncts to surgical resection.
J Pediatr Hematol Oncol. 2013; 35(3):e123-6 [PubMed]
Outcome of surgical treatment of 200 children with Cushing's disease.
J Clin Endocrinol Metab. 2013; 98(3):892-901 [PubMed] Article available free on PMC after 01/03/2014
OBJECTIVE: The aim of this study was to examine features influencing the outcome of surgery for pediatric CD.
DESIGN: In this prospective observational study, the clinical, imaging, endocrinological, and operative outcomes were analyzed in consecutive patients treated at the National Institutes of Health (NIH) from 1982 through 2010.
SETTING: The study was conducted in a tertiary referral center.
RESULTS: Two hundred CD patients (106 females, 94 males) were included. Mean age at symptom development was 10.6 ± 3.6 years (range, 4.0 to 19.0 y). Mean age at NIH operation was 13.7 ± 3.7 years. Twenty-seven patients (13%) had prior surgery at another institution. Magnetic resonance imaging identified adenomas in 97 patients (50%). When positive, magnetic resonance imaging accurately defined a discrete adenoma in 96 of the 97 patients (99%), which was more accurate than the use of ACTH ratios during inferior petrosal sinus sampling to determine adenoma lateralization (accurate in 72% of patients without prior surgery). A total of 195 of the 200 patients (98%) achieved remission after surgery (189 [97%] were hypocortisolemic; 6 [3%] were eucortisolemic postoperatively). Factors associated with initial remission (P < .05) included identification of an adenoma at surgery, immunohistochemical ACTH-producing adenoma, and noninvasive ACTH adenoma. Younger age, smaller adenoma, and absence of cavernous sinus wall or other dural invasion were associated with long-term remission (P < .05). A minimum morning serum cortisol of less than 1 μg/dl after surgery had a positive predictive value for lasting remission of 96%.
CONCLUSIONS: With rare disorders, such as pediatric CD, enhanced outcomes are obtained by evaluation and treatment at centers with substantial experience. Resection of pituitary adenomas in pediatric CD in that setting can be safe, effective, and durable. Early postoperative endocrine testing predicts lasting remission. Because lasting remission is associated with younger age at surgery, smaller adenomas, and lack of dural invasion, early diagnosis should improve surgical outcome.
Cerebellar metastatic papillary thyroid carcinoma in a pediatric patient with complex congenital heart disease.
J Pediatr Endocrinol Metab. 2012; 25(11-12):1195-9 [PubMed]
Hyperprolactinemia in children: clinical features and long-term results.
J Pediatr Endocrinol Metab. 2012; 25(11-12):1123-8 [PubMed]
Pharmacotherapeutic management of pediatric gliomas : current and upcoming strategies.
Paediatr Drugs. 2013; 15(1):29-42 [PubMed]
Management and outcome of focal low-grade brainstem tumors in pediatric patients: the St. Jude experience.
J Neurosurg Pediatr. 2013; 11(3):274-81 [PubMed]
METHODS: The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Event-free survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS.
RESULTS: Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42%), pons (n = 15, 29%), and medulla (n = 15, 29%). Surgical extirpation was the primary treatment in 25 patients (48%). The 5- and 10-year EFS and overall survival were 59%/98% and 52%/90%, respectively. An event or treatment failure occurred in 24 patients (46%), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS.
CONCLUSIONS: Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.
The clinical results of pediatric brain tumors treated with Linac-based stereotactic radiosurgery and radiotherapy.
J Med Assoc Thai. 2012; 95(11):1466-71 [PubMed]
RESULTS: The median follow-up time was 26 months (range, 1 to 154 months). The local progression free survival (LPFS) at one and five years after SRS/FSRT for all patients was 87.5% and 54.2%, respectively. The 5-year LPFS by tumor histology was as follow, pituitary adenoma 100%, meningioma 100%, ependymoma, and low-grade astrocytoma 75%, and craniopharyngioma 68.6%. High-grade tumor had the worst LPFS and the median LPFS of this group was only 12 months. On univariate analysis, low-grade tumor (pituitary adenoma and menigioma) and small tumor volume (< 10 ml) were the factors that correlated significantly with good local control. After multivariate analysis, small tumor volume was the only factor associated with good LPFS (HR = 2.35, p = 0.042). No other radiation complication except panhypopituitarism was reported.
CONCLUSION: SRS/FSRT in pediatric brain tumor is technically feasible, with minimal acute side effects. SRS/FSRT plays an important role for the small low-grade tumor
Longitudinal investigation of adaptive functioning following conformal irradiation for pediatric craniopharyngioma and low-grade glioma.
Int J Radiat Oncol Biol Phys. 2013; 85(5):1301-6 [PubMed]
METHODS AND MATERIALS: Children with craniopharyngioma (n=62) and LGG (n=77) were treated using conformal or intensity modulated radiation therapy. The median age was 8.05 years (3.21-17.64 years) and 8.09 years (2.20-19.27 years), respectively. Serial cognitive evaluations including measures of intelligence quotient (IQ) and the Vineland Adaptive Behavior Scales (VABS) were conducted at preirradiation baseline, 6 months after treatment, and annually through 5 years. Five hundred eighty-eight evaluations were completed during the follow-up period.
RESULTS: Baseline assessment revealed no deficits in IQ and VABS indices for children with craniopharyngioma, with significant (P<.05) longitudinal decline in VABS Communication and Socialization indices. Clinical factors associated with more rapid decline included females and preirradiation chemotherapy (interferon). The only change in VABS Daily Living Skills correlated with IQ change (r=0.34; P=.01) in children with craniopharyngioma. Children with LGG performed below population norms (P<.05) at baseline on VABS Communication, Daily Living Indices, and the Adaptive Behavior Composite, with significant (P<.05) longitudinal decline limited to VABS Communication. Older age at irradiation was a protective factor against longitudinal decline. Severe visual impairment did not independently correlate with poorer adaptive outcomes for either tumor group.
CONCLUSIONS: There was relative sparing of postirradiation functional outcomes over time in this sample. Baseline differences in functional abilities before the initiation of irradiation suggested that other factors influence functional outcomes above and beyond the effects of irradiation.
Disease control after reduced volume conformal and intensity modulated radiation therapy for childhood craniopharyngioma.
Int J Radiat Oncol Biol Phys. 2013; 85(4):e187-92 [PubMed] Article available free on PMC after 15/03/2014
METHODS AND MATERIALS: Eighty-eight children (median age, 8.5 years; range, 3.2-17.6 years) received conformal or intensity modulated radiation therapy between 1998 and 2009. The study group included those prospectively treated from 1998 to 2003, using a 10-mm CTV, defined as the margin surrounding the solid and cystic tumor targeted to receive the prescription dose of 54 Gy. The CTV margin was subsequently reduced after 2003, yielding 2 groups of patients: those treated with a CTV margin greater than 5 mm (n=26) and those treated with a CTV margin less than or equal to 5 mm (n=62). Disease progression was estimated on the basis of additional variables including sex, race, extent of resection, tumor interventions, target volume margins, and frequency of weekly surveillance magnetic resonance (MR) imaging during radiation therapy. Median follow-up was 5 years.
RESULTS: There was no difference between progression-free survival rates based on CTV margins (>5 mm vs ≤5 mm) at 5 years (88.1% ± 6.3% vs 96.2% ± 4.4% [P=.6386]). There were no differences based on planning target volume (PTV) margins (or combined CTV plus PTV margins). The PTV was systematically reduced from 5 to 3 mm during the time period of the study. Factors predictive of superior progression-free survival included Caucasian race (P=.0175), no requirement for cerebrospinal fluid shunting (P=.0066), and number of surveillance imaging studies during treatment (P=.0216). Patients whose treatment protocol included a higher number of weekly surveillance MR imaging evaluations had a lower rate of tumor progression.
CONCLUSIONS: These results suggest that targeted volume reductions for radiation therapy using smaller margins are feasible and safe but require careful monitoring. We are currently investigating the differences in outcome based on host factors to explain the results.
Convection-enhanced delivery of topotecan into diffuse intrinsic brainstem tumors in children.
J Neurosurg Pediatr. 2013; 11(3):289-95 [PubMed]
Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients.
J Neurosurg Pediatr. 2013; 11(3):245-9 [PubMed]
METHODS: One hundred consecutive children and adolescents (0-19 years old) who underwent primary tumor resection for a low-grade cerebellar astrocytoma during the years 1980-2011 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.
RESULTS: Of the 100 patients, 61 children were in the 1st decade, and 39 were 10-19 years old. The male/female ratio was 1.13:1 (53 males, 47 females). No patients were lost to follow-up. There were no deaths in this series and all 100 patients are currently alive. In 29 patients, the follow-up duration was less than 10 years, in 37 it was between 10 and 19 years, and in 34 it was between 20 and 31 years. The Barthel Index was 100 (normal) in 97 patients, 90 in 2 patients, and 40 in the last patient. A total of 113 tumor resections were performed. Two patients underwent further tumor resection due to MRI-confirmed residual tumor demonstrated on the immediate postoperative MR image (obtained the day after the initial procedure). Furthermore, 9 children underwent repeat tumor resection after MRI-confirmed progressive tumor recurrence up to 10 years after the initial operation. Two of these patients also underwent a third resection, without subsequent radiation therapy, and have experienced 8 and 12 years of tumor-free follow-up thereafter, respectively. A total of 15% of the patients required treatment for persistent hydrocephalus.
CONCLUSIONS: Low-grade cerebellar astrocytoma is a surgical disease, in need of long-term follow-up, but with excellent long-term results. Nine percent of the children in this study underwent repeated surgery due to progressive tumor recurrence, and 15% were treated for persistent hydrocephalus.
Seizure outcome of surgical treatment of focal epilepsy associated with low-grade tumors in children.
J Neurosurg Pediatr. 2013; 11(2):214-23 [PubMed]
METHODS: In the present study, the authors retrospectively reviewed 30 patients (age range 3-18 years) who underwent surgery for histopathologically confirmed LGTs, in which seizures were the only clinical manifestation. The patients were divided into 2 groups according to the type of surgical treatment: patients in Group A (20 cases) underwent only tumor removal (lesionectomy), whereas patients in Group B (11 cases) underwent removal of the tumor and the adjacent epileptogenic zone (tailored surgery). One of the patients, who underwent 2 operations, is included in both groups. Follow-up ranged from 1 to 17 years.
RESULTS: Sixteen (80%) of 20 patients in Group A had an Engel Class I outcome. In this group, 3 of 4 patients who were in Engel Classes II and III had temporomesial lesions. All patients in Group B had temporomesial tumors and were seizure free (Engel Class I). In this series, in temporolateral and extratemporal tumor locations, lesionectomy yielded a good seizure outcome. In addition, a young age at seizure onset (in particular < 4 years) was associated with a poor seizure outcome.
CONCLUSIONS: Tailored resection in temporomesial LGTs was associated with excellent seizure outcome, indicating that an adequate presurgical evaluation including extensive neurophysiological evaluation (long-term videoelectroencephalography monitoring) to plan appropriate surgical strategy is advised.
Viability screen on pediatric low grade glioma cell lines unveils a novel anti-cancer drug of the steroid biosynthesis inhibitor family.
Cancer Lett. 2013; 330(1):96-105 [PubMed]
Recurrent pediatric central nervous system low-grade gliomas: the role of surveillance neuroimaging in asymptomatic children.
J Neurosurg Pediatr. 2013; 11(2):119-26 [PubMed]
METHODS: The authors performed a retrospective analysis of data obtained in 102 consecutive patients diagnosed at Rady Children's Hospital-San Diego between 1994 and 2010 with a biopsy-proven LGG exclusive of a diagnosis of neurofibromatosis. Tumor location, patient age, sex, and symptomatology were correlated with tumor progression or recurrence. Magnetic resonance imaging characteristics and neuroimaging surveillance frequency were analyzed in those children with progressive or recurrent disease.
RESULTS: Forty-six of 102 children diagnosed with an LGG had evidence of recurrent or progressive disease between 2 months and 11 years (mean 27.3 months) after diagnosis. In the larger group of 102 children, gross-total resection was associated with improved progression-free survival (p = 0.012). The location of tumor (p = 0.26), age at diagnosis (p = 0.69), duration of symptoms (p = 0.72), histological subtype (p = 0.74), sex (p = 0.53), or specific chemotherapeutic treatment regimen (p = 0.24) was not associated with tumor progression or recurrence. Sixty-four percent of children with recurrent or progressive disease were asymptomatic, and recurrence was diagnosed by surveillance MRI alone. All children less than 2 years of age in whom the tumor was diagnosed were asymptomatic at the time of progression (p = 0.04). Thirteen percent (6 of 46) of the children had disease recurrence 5 years after initial diagnosis; all of them had undergone an initial subtotal resection. Tumor progression was associated with either homogeneous or patchy T1-weighted post-Gd administration MRI enhancement in 94% of the cases (p = 0.0001).
CONCLUSIONS: Children diagnosed with recurrent LGG may be asymptomatic at the time of recurrence. The authors' findings support the need for routine neuroimaging in a subset of children with LGGs, even when gross-total resection has been achieved, up to 5 years postdiagnosis. The authors found that T1-weighted MR images obtained before and after Gd administration alone may be sufficient to diagnose LGG recurrence and may represent an effective strategy worthy of further validation in a larger multiinstitutional cohort.
Tumors of the superior medullary velum in infancy and childhood: report of 6 cases.
J Neurosurg Pediatr. 2013; 11(1):52-9 [PubMed]
METHODS: The authors observed 6 infants and children harboring neoplasms of the SMV who were treated at Ann & Robert Lurie Children's Hospital of Chicago (formerly Children's Memorial Hospital) in Chicago, Illinois. Pathological diagnosis of the neoplasms was an atypical teratoid/rhabdoid tumor (ATRT) in 5 patients, and a juvenile pilocytic astrocytoma (JPA) in the remaining child. The tumors were diagnosed during infancy in all patients, with ages ranging from 3 months to 10 months, except for the patient with a JPA (diagnosed at 5 years old). All patients presented with signs and symptoms of increased intracranial pressure due to obstructive hydrocephalus.
RESULTS: Characteristic MRI features were noted, consistent with a mass in both the fourth ventricle and the cerebellomesencephalic fissure and quadrigeminal cistern, resulting in the circumferential displacement of the neural structures surrounding the SMV. The tumor was removed effectively in gross-total fashion through the occipital transtentorial approach in all patients. This approach offers a wide exposure of the region. However, all infants with ATRT suffered tumor dissemination and died between 4 and 11 months after diagnosis, in spite of radical resection and oncological treatment. The 1 child with JPA is alive and well 30 months after tumor resection.
CONCLUSIONS: To the best of the authors' knowledge, this is the first description in the literature that focuses on tumors originating from the SMV. This entity must be promptly recognized on preoperative radiological studies to carefully plan the subsequent surgical and clinical management.
Pathological and molecular advances in pediatric low-grade astrocytoma.
Annu Rev Pathol. 2013; 8:361-79 [PubMed] Article available free on PMC after 24/01/2014
Motexafin-gadolinium and involved field radiation therapy for intrinsic pontine glioma of childhood: a children's oncology group phase 2 study.
Int J Radiat Oncol Biol Phys. 2013; 85(1):e55-60 [PubMed] Article available free on PMC after 01/01/2014
METHODS AND MATERIALS: Patients with newly diagnosed intrinsic pontine glioma were treated with MGd daily for 5 consecutive days each week, for a total of 30 doses. Patients received a 5- to 10-min intravenous bolus of MGd, 4.4 mg/kg/day, given 2 to 5 h prior to standard dose irradiation. Radiation therapy was administered at a daily dose of 1.8 Gy for 30 treatments over 6 weeks. The total dose was 54 Gy.
RESULTS: Sixty eligible children received MGd daily, concurrent with 6 weeks of radiation therapy. The estimated 1-year EFS was 18%±5%, and the estimated 1-year OS was 53%±6.5%. The most common grade 3 to 4 toxicities were lymphopenia, transient elevation of liver transaminases, and hypertension.
CONCLUSIONS: Compared to historical controls, the addition of MGd to a standard 6-week course of radiation did not improve the survival of pediatric patients with newly diagnosed intrinsic pontine gliomas.
Cytogenetic findings in pediatric radiation-induced atypical meningioma after treatment of medulloblastoma: case report and review of the literature.
J Neurooncol. 2012; 110(3):397-402 [PubMed]
Predictors of outcome in an AIEOP series of childhood ependymomas: a multifactorial analysis.
Neuro Oncol. 2012; 14(11):1346-56 [PubMed] Article available free on PMC after 01/11/2013
Treatment of pediatric patients and young adults with particle therapy at the Heidelberg Ion Therapy Center (HIT): establishment of workflow and initial clinical data.
Radiat Oncol. 2012; 7:170 [PubMed] Article available free on PMC after 01/11/2013
MATERIALS AND METHODS: We treated 36 pediatric patients (aged 21 or younger) with particle therapy at HIT. Median age was 12 years (range 2-21 years), five patients (14%) were younger than 5 years of age. Indications included pilocytic astrocytoma, parameningeal and orbital rhabdomyosarcoma, skull base and cervical chordoma, osteosarcoma and adenoid-cystic carcinoma (ACC), as well as one patient with an angiofibroma of the nasopharynx. For the treatment of small children, an anesthesia unit at HIT was established in cooperation with the Department of Anesthesiology.
RESULTS: Treatment concepts depended on tumor type, staging, age of the patient, as well as availability of specific study protocols. In all patients, particle radiotherapy was well tolerated and no interruptions due to toxicity had to be undertaken. During follow-up, only mild toxicites were observed. Only one patient died of tumor progression: Carbon ion radiotherapy was performed as an individual treatment approach in a child with a skull base recurrence of the previously irradiated rhabdomyosarcoma. Besides this patient, tumor recurrence was observed in two additional patients.
CONCLUSION: Clinical protocols have been generated to evaluate the real potential of particle therapy, also with respect to carbon ions in distinct pediatric patient populations. The strong cooperation between the pediatric department and the department of radiation oncology enable an interdisciplinary treatment and stream-lined workflow and acceptance of the treatment for the patients and their parents.
Parent-reported social outcomes after treatment for pediatric embryonal tumors: a prospective longitudinal study.
J Clin Oncol. 2012; 30(33):4134-40 [PubMed] Article available free on PMC after 20/11/2013
PATIENTS AND METHODS: Patients (N=220) were enrolled onto a multisite clinical treatment protocol. Parents completed the Child Behavior Checklist/6-18 at the time of their child's diagnosis and yearly thereafter. A generalized linear mixed effects model regression approach was used to examine longitudinal changes in parent ratings of social competence, social problems, and withdrawn/depressed behaviors with demographic and treatment factors as covariates.
RESULTS: During the 5-year period following diagnosis and treatment, few patients were reported to have clinically elevated scores on measures of social functioning. Mean scores differed significantly from population norms, yet remained within the average range. Several factors associated with unfavorable patterns of change in social functioning were identified. Patients with high-risk treatment status had a greater increase in parent-reported social problems (P=.001) and withdrawn/depressed behaviors (P=.01) over time compared with average-risk patients. Patients with posterior fossa syndrome had greater parent-reported social problems over time (P=.03). Female patients showed higher withdrawn/depressed scores over time compared with male patients (P<.001). Patient intelligence, age at diagnosis, and parent education level also contributed to parent report of social functioning.
CONCLUSION: Results of this study largely suggest positive social adjustment several years after diagnosis and treatment of a pediatric embryonal tumor. However, several factors, including treatment risk status and posterior fossa syndrome, may be important precursors of long-term social outcomes. Future research is needed to elucidate the trajectory of social functioning as these patients transition into adulthood.
Glutathione S-transferase P1 single nucleotide polymorphism predicts permanent ototoxicity in children with medulloblastoma.
Pediatr Blood Cancer. 2013; 60(4):593-8 [PubMed] Article available free on PMC after 01/04/2014
PROCEDURE: The study included 106 medulloblastoma/primitive neuroectodermal tumor (PNET) patients seen at Texas Children's Cancer Center. Genotyping was performed using an Illumina HumanOmni1-Quad BeadChip and GSTpi expression was assessed using immunohistochemistry. We used the Kaplan-Meier method for survival analyses and logistic regression for toxicity comparisons.
RESULTS: Patients with a GSTP1 105 AG/GG genotype (vs. AA) or who had received high dose craniospinal radiation (≥34 Gy vs. <26 Gy) had a greater risk of requiring hearing aids than their counterparts (OR 4.0, 95% CI 1.2-13.6, and OR 3.1, 95% CI 1.1-8.8, respectively, n = 69). Additionally, there was a statistically significant interaction between these variables. Compared with the lowest risk group (GSTP1 105 AA-low dose radiation), patients with a GSTP1 105 AG/GG genotype who received high dose radiation were 8.4 times more likely to require hearing aids (95% CI 1.4-49.9, p-trend = 0.005, n = 69). When adjusted for age, cumulative cisplatin dose, and amifostine use, the association remained.
CONCLUSIONS: The GSTP1 105 G-allele is associated with permanent ototoxicity in pediatric medulloblastoma/PNET and strongly interacts with radiation dose. Patients with this allele should be considered for clinical trials employing radiation dose modifications and cytoprotectant strategies.
Pegylated interferon-α-2b for children with recurrent craniopharyngioma.
J Neurosurg Pediatr. 2012; 10(6):498-503 [PubMed]
METHODS: Five children, ranging in age from 9 to 15 years, with recurrent craniopharyngiomas were treated for up to 2 years with subcutaneous injections of PI at a dose of 1-3 μg/kg/week. Tumor response was assessed using MRI.
RESULTS: All patients had stable disease or better in response to PI. One patient experienced a recurrence after gross-total resection (GTR). She initially showed an increase in the predominantly cystic tumor after 3 months of treatment, followed by a complete response. She required no further intervention and remains without evidence of disease 10 years after starting treatment. Another patient experienced recurrence 3.3 years after subtotal resection (STR) and radiation therapy. He had complete disappearance of the predominantly cystic component after 4 months of treatment, and a small residual calcified mass remains 5 years later. The third patient experienced recurrence after 3 GTRs. He had a complete response after 7 months of treatment and remains without evidence of disease 19 months after starting treatment. The fourth patient experienced recurrence after 2 STRs. He had a 30% decrease in tumor size after 4 months of treatment, which was maintained for 12 months at which point the cyst began to increase in size. The final patient experienced recurrence after GTR and has stable disease 6 months after starting treatment with PI.
CONCLUSIONS: The use of PI in children with recurrent craniopharyngiomas can result in significant and durable responses and potentially delay or avoid the need for radiation therapy.
Gamma knife surgery of pediatric gliomas.
J Neurosurg Pediatr. 2012; 10(6):471-7 [PubMed]
METHODS: The authors reviewed a consecutive series of 24 pediatric patients treated at the authors' institution between 1989 and 2011. All patients harbored tumors that were either surgically inaccessible or had evidence of residual or recurrent growth after resection. Progression-free survival was evaluated and correlated with clinical variables. Additional outcomes evaluated were clinical outcome, imaging response, and overall survival.
RESULTS: Between 1989 and 2011, 13 male and 11 female patients (median age 11 years, range 4-18 years) with gliomas were treated. Tumor pathology was pilocytic astrocytoma (WHO Grade I) in 15 patients (63%), WHO Grade II in 4 (17%), and WHO Grade III in 1 (4%). The tumor pathology was not confirmed in 4 patients (17%). The mean tumor volume at the time of treatment was 2.4 cm(3). Lesions were treated with a median maximum dose of 36 Gy, median of 3 isocenters, and median marginal dose of 15 Gy. The median duration of imaging follow-up was 74 months, and the median duration of clinical follow-up was 144 months. The tumors responded with a median decrease in volume of 71%. At last follow up, a decrease in tumor size of at least 50% was demonstrated in 18 patients (75%) and complete tumor resolution was achieved in 5 (21%). Progression-free survival at last follow-up was achieved in 20 patients (83%). Progression was documented in 4 patients (17%), with 3 patients requiring repeat resection and 1 patient dying. The initial tumor volume was significantly greater in patients with disease progression (mean volume 4.25 vs 2.0 cm(3), p < 0.001). Age, tumor pathology, tumor location, previous radiation, Karnofsky Performance Scale score, symptom duration, and target dosage did not differ significantly between the 2 groups.
CONCLUSIONS: Gamma Knife surgery can provide good clinical control of residual or recurrent gliomas in pediatric patients. Worse outcomes in the present series were associated with larger tumor volumes at the time of treatment.
An fMRI investigation of working memory and its relationship with cardiorespiratory fitness in pediatric posterior fossa tumor survivors who received cranial radiation therapy.
Pediatr Blood Cancer. 2013; 60(4):669-75 [PubMed] Article available free on PMC after 01/04/2014
PROCEDURE: Nine adolescent survivors of pediatric posterior fossa tumor completed an n-back working memory task during a functional MRI scan, as well as cardiorespiratory fitness testing on a cycle ergometer.
RESULTS: Neuroimaging findings indicated typical activation patterns associated with working memory, mainly in the frontal-parietal network. Higher cardiorespiratory fitness was related to better performance on a behavioral measure of working memory and more efficient neural functioning.
CONCLUSIONS: This study provides preliminary evidence that cardiorespiratory fitness may be related to executive functioning, particularly working memory, in pediatric brain tumor survivors. Descriptions of the brain regions recruited for working memory by pediatric brain tumor survivors may be used to inform future interventions or indicators of treatment efficacy.
Pseudoprogression after high-dose busulfan-thiotepa with autologous stem cell transplantation and radiation therapy in children with brain tumors: Impact on survival.
Neuro Oncol. 2012; 14(11):1413-21 [PubMed] Article available free on PMC after 01/11/2013
Sustained response to weekly vinblastine in 2 children with pilomyxoid astrocytoma associated with diencephalic syndrome.
J Pediatr Hematol Oncol. 2013; 35(2):e53-6 [PubMed]
Second malignant neoplasms in childhood malignant brain tumour: a long-term population-based study.
J Paediatr Child Health. 2012; 48(11):990-6 [PubMed]
METHODS: Surveillance, Epidemiology, and End Results (SEER) database (1973-2007) was used to identify above-stated patients. SMN patients were further identified, and standardised incidence ratios (SIRs) and excess absolute risks (EARs) for risk-factor-decided subgroups were calculated. Univariate and multivariate analyses of the association between cumulative incidence of SMN and the risk factors were performed in the whole population.
RESULTS: A total of 106 patients were identified as having SMNs. EARs peaked at age at primary diagnosis of 10-14. Males had higher SIRs and EARs than females. Both SIRs and EARs increased after 1990. Age was statistically significant in both univariable and multivariable analyses for cumulative incidence of SMN and RT was not significant in both the analyses, in the whole population of 9075 patients. After follow-up recalculation, matched patients in the ≥1990 group had slightly shorter median interval between primary and secondary cancer than those in the <1990 group, but with no significance.
CONCLUSION: The risk of SMN in children with primary malignant brain tumours in a more advanced treatment era might have changed. During making further advances in the treatment of these neoplasms, minimising toxicities while maintaining promising prognostic outcomes will keep being our goal.
Accurate classification of childhood brain tumours by in vivo ¹H MRS - a multi-centre study.
Eur J Cancer. 2013; 49(3):658-67 [PubMed]
METHODS: Seventy-eight patients under 16 years old with histologically proven brain tumours from 10 international centres were investigated. Discrimination of 29 medulloblastomas, 11 ependymomas and 38 pilocytic astrocytomas (PILOAs) was evaluated. Single-voxel MRS was undertaken prior to diagnosis (1.5 T Point-Resolved Spectroscopy (PRESS), Proton Brain Exam (PROBE) or Stimulated Echo Acquisition Mode (STEAM), echo time (TE) 20-32 ms and 135-136 ms). MRS data were processed using two strategies, determination of metabolite concentrations using TARQUIN software and automatic feature extraction with Peak Integration (PI). Linear Discriminant Analysis (LDA) was applied to this data to produce diagnostic classifiers. An evaluation of the diagnostic accuracy was performed based on resampling to measure the Balanced Accuracy Rate (BAR).
RESULTS: The accuracy of the diagnostic classifiers for discriminating the three tumour types was found to be high (BAR 0.98) when a combination of TE was used. The combination of both TEs significantly improved the classification performance (p<0.01, Tukey's test) compared with the use of one TE alone. Other tumour types were classified accurately as glial or primitive neuroectodermal (BAR 1.00).
CONCLUSION: (1)H MRS has excellent accuracy for the non-invasive diagnosis of common childhood brain tumours particularly if the metabolite information is maximised and should become part of routine clinical assessment for these children.
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