scholarly journals Pulmonary Function After Treatment for Embryonal Brain Tumors on SJMB03 That Included Craniospinal Irradiation

2015 ◽  
Vol 93 (1) ◽  
pp. 47-53 ◽  
Author(s):  
Daniel M. Green ◽  
Thomas E. Merchant ◽  
Catherine A. Billups ◽  
Dennis C. Stokes ◽  
Alberto Broniscer ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Pulmonary Function ◽  
Craniospinal Irradiation ◽  
Embryonal Brain

Pulmonary function after treatment for embryonal brain tumors on SJMB03 that included craniospinal irradiation.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10021-10021
Author(s):  
Daniel M. Green ◽  
Thomas E. Merchant ◽  
Catherine A Billups ◽  
Dennis C Stokes ◽  
Alberto Broniscer ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
High Dose Chemotherapy ◽  
Forced Expiratory Volume ◽  
Craniospinal Irradiation ◽  
Age At Diagnosis ◽  
High Dose ◽  
Embryonal Brain ◽  
Cyclophosphamide Dose

10021 Background: Treatment of children with embryonal brain tumors (EBT) includes craniospinal irradiation. There are limited data regarding the effect of radiation therapy (RT) on pulmonary function. Methods: Protocol SJMB03 enrolled patients 3 to 21 years of age with EBT. Pulmonary function tests (PFTs) [forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) by spirometry, total lung capacity (TLC) by plethysmography and diffusing capacity of the lung for carbon monoxide (DLCO)] were obtained following completion of RT, prior to each of four courses of high-dose chemotherapy (cumulative cyclophosphamide dose, 16 g/m2), and 24 months after completion of treatment (ACT). Differences between PFTs obtained following the completion of RT and 24 months ACT were compared using exact Wilcoxon signed rank tests. Results: 303 eligible patients were enrolled between June 24, 2003 and March 1, 2010, 258 of whom had at least one PFT. Median age at diagnosis - 8.9 years (range, 3.1 to 20.4 years). Median spinal RT dose - 23.4 Gy (range, 23.4 to 50.4 Gy). Median cyclophosphamide dose was 16.24 g (range, 0 to 34.38 g). 24 months ACT, DLCO was < 75% predicted in 23% (27/115 evaluated), FEV1 was < 80% predicted in 21% (32/150 evaluated), FVC was < 80% predicted in 27% (46/168 evaluated) and TLC was < 80% predicted in 18% (24/135 evaluated) of patients. DLCO was significantly decreased 24 months ACT compared to the end of RT (median difference (MD) in % predicted, - 3.00%; p = 0.035). Race and cumulative cyclophosphamide dose were not significant predictors of DLCO. DLCO was significantly higher among males (p = 0.037) than females in a model that included time point, sex, RT dose group, RT dose*time interaction and age at diagnosis. The differences in FEV1 ((MD, - 1.00%), FVC (MD, 0.00%) and TLC (MD, -2.00%) were not statistically significant. Conclusions: Among patients with EBT treated with spinal RT, DLCO was significantly decreased 24 months after completion of treatment compared to immediately post-RT. TLC was decreased 24 months ACT, suggesting that a significant minority of patients have restrictive lung disease. Continued monitoring of this cohort to five years ACT is planned. Clinical trial information: NCT00085202.

scholarly journals Cognitive Implications of Ototoxicity in Pediatric Patients With Embryonal Brain Tumors

2019 ◽  
Vol 37 (18) ◽  
pp. 1566-1575 ◽  
Author(s):  
Traci W. Olivier ◽  
Johnnie K. Bass ◽  
Jason M. Ashford ◽  
Rebecca Beaulieu ◽  
Sarah M. Scott ◽  
...  
Keyword(s):  
Working Memory ◽  
Reading Comprehension ◽  
Brain Tumors ◽  
Processing Speed ◽  
Reading Difficulties ◽  
Treatment Protocol ◽  
Craniospinal Irradiation ◽  
Average Risk ◽  
Speed Of Information Processing ◽  
Embryonal Brain

PURPOSE Sensorineural hearing loss (SNHL) is associated with intellectual and academic declines in children treated for embryonal brain tumors. This study expands upon existing research by examining core neurocognitive processes that may result in reading difficulties in children with treatment-related ototoxicity. PATIENTS AND METHODS Prospectively gathered, serial, neuropsychological and audiology data for 260 children and young adults age 3 to 21 years (mean, 9.15 years) enrolled in a multisite research and treatment protocol, which included surgery, risk-adapted craniospinal irradiation (average risk, n = 186; high risk, n = 74), and chemotherapy, were analyzed using linear mixed models. Participants were assessed at baseline and up to 5 years after diagnosis and grouped according to degree of SNHL. Included were 196 children with intact hearing or mild to moderate SNHL (Chang grade 0, 1a, 1b, or 2a) and 64 children with severe SNHL (Chang grade 2b or greater). Performance on eight neurocognitive variables targeting reading outcomes (eg, phonemics, fluency, comprehension) and contributory cognitive processes (eg, working memory, processing speed) was analyzed. RESULTS Participants with severe SNHL performed significantly worse on all variables compared with children with normal or mild to moderate SNHL ( P ≤ .05), except for tasks assessing awareness of sounds and working memory. Controlling for age at diagnosis and risk-adapted craniospinal irradiation dose, performance on the following four variables remained significantly lower for children with severe SNHL: phonemic skills, phonetic decoding, reading comprehension, and speed of information processing ( P ≤ .05). CONCLUSION Children with severe SNHL exhibit greater reading difficulties over time. Specifically, they seem to struggle most with phonological skills and processing speed, which affect higher level skills such as reading comprehension.

scholarly journals PATH-08. THE IMPORTANCE OF RE-DIAGNOSIS OF TUMORS PREVIOUSLY CLASSIFIED AS CENTRAL NERVOUS SYSTEM PRIMITIVE NEUROECTODERMAL TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii426-iii426
Author(s):  
Naohide Fujita ◽  
Osamu Akiyama ◽  
Akihide Kondo
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumors ◽  
Easy Access ◽  
Molecular Analyses ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Sanger Sequence ◽  
Embryonal Brain ◽  
Polymerase Chain

Abstract BACKGROUND The recent molecular analyses have revealed that central nervous system primitive neuroectodermal tumors (CNS PNETs) those having clusters of small round tumor cells are genetically different tumors. However, the concepts of CNS PNET are complicated, and it is difficult to diagnose them appropriately in clinical field. To overcome this difficulty, we reviewed previous studies associated with CNS PNETs, and carried out several approaches, those are relatively easy access to use in clinics, for our 8 samples of embryonal brain tumors diagnosed CNS PNETs in our institution, initially. METHODS We used in combination with immunohistochemistry (IHC), Sanger sequence, Pyrosequence, polymerase chain reaction (PCR), real time PCR and copy number analysis referring recent reports. RESULTS In terms of the diagnosis three out of 8 cases were changed based on the results in this study from previous diagnoses. CONCLUSION In this review, it seemed that either the histopathological evaluation or molecular analyses would be not enough to make accurate diagnosis of CNS embryonal brain tumors, and it is essential to combine both of them including recent comprehensive analysis methods.

scholarly journals Polo-Like Kinase 4 (PLK4) Is Overexpressed in Central Nervous System Neuroblastoma (CNS-NB)

2018 ◽  
Vol 5 (4) ◽  
pp. 96 ◽  
Author(s):  
Anders Bailey ◽  
Amreena Suri ◽  
Pauline Chou ◽  
Tatiana Pundy ◽  
Samantha Gadd ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumors ◽  
Meta Analysis ◽  
P Value ◽  
Embryonal Tumors ◽  
Expression Levels ◽  
Embryonal Brain

Neuroblastoma (NB) is the most common extracranial solid tumor in pediatrics, with rare occurrences of primary and metastatic tumors in the central nervous system (CNS). We previously reported the overexpression of the polo-like kinase 4 (PLK4) in embryonal brain tumors. PLK4 has also been found to be overexpressed in a variety of peripheral adult tumors and recently in peripheral NB. Here, we investigated PLK4 expression in NBs of the CNS (CNS-NB) and validated our findings by performing a multi-platform transcriptomic meta-analysis using publicly available data. We evaluated the PLK4 expression by quantitative real-time PCR (qRT-PCR) on the CNS-NB samples and compared the relative expression levels among other embryonal and non-embryonal brain tumors. The relative PLK4 expression levels of the NB samples were found to be significantly higher than the non-embryonal brain tumors (p-value < 0.0001 in both our samples and in public databases). Here, we expand upon our previous work that detected PLK4 overexpression in pediatric embryonal tumors to include CNS-NB. As we previously reported, inhibiting PLK4 in embryonal tumors led to decreased tumor cell proliferation, survival, invasion and migration in vitro and tumor growth in vivo, and therefore PLK4 may be a potential new therapeutic approach to CNS-NB.

scholarly journals MEDU-34. INTELLECTUAL OUTCOME IN CHILDREN WITH SENSORINEURAL HEARING LOSS FOLLOWING TREATMENT FOR EMBRYONAL BRAIN TUMORS.

2017 ◽  
Vol 19 (suppl_4) ◽  
pp. iv44-iv45 ◽  
Author(s):  
Christine Dahl ◽  
Iska Moxon-Emre ◽  
Vijay Ramaswamy ◽  
Ute Bartels ◽  
Uri Tabori ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Brain Tumors ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Intellectual Outcome ◽  
Embryonal Brain

Concurrent cyclophosphamide and craniospinal radiotherapy for pediatric high-risk embryonal brain tumors

2012 ◽  
Vol 110 (2) ◽  
pp. 287-291 ◽  
Author(s):  
Cynthia J. Campen ◽  
Joanna Dearlove ◽  
Sonia Partap ◽  
Patricia Murphy ◽  
Iris C. Gibbs ◽  
...  
Keyword(s):  
High Risk ◽  
Brain Tumors ◽  
Craniospinal Radiotherapy ◽  
Embryonal Brain

Endocrine outcome for children with embryonal brain tumors treated with craniospinal irradiation on the SJMB96 trial

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 9571-9571
Author(s):  
A. Gajjar ◽  
T. Merchant ◽  
C. Sklar ◽  
D. Wallace ◽  
Z. Xiong ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Thyroid Hormone ◽  
Craniospinal Irradiation ◽  
Screening Tests ◽  
Hormone Deficiency ◽  
Study Cohort ◽  
Central Hypothyroidism ◽  
Primary Tumor Site ◽  
Acth Deficiency ◽  
Embryonal Brain

9571 Background: To investigate the relationship between endocrine deficits with the radiation (RT) dose to the hypothalamic- pituitary axis (HPA) in medulloblastoma/PNET patients treated with risk-adapted craniospinal irradiation (CSI) followed by chemotherapy. Methods: 88 patients who survived at least 2 years from diagnosis were included in this analysis. CSI doses were 23.4 Gy or 36–39.6 Gy depending on clinical risk; the primary tumor site received 55.8 Gy. All pts had regular endocrine follow-up and screening to test for growth hormone (GH), thyroid hormone (TH) and adrenocorticotrophin hormone (ACTH) deficiency. Patients that had abnormal results on the screening tests underwent further evaluation by stimulation testing of the HPA. Only patients diagnosed with central hypothyroidism were included in the analysis for TH deficiency. Results: Of the 88 pts included in the study cohort, 77 pts had abnormal screening tests and underwent provocative testing. The median radiation dose to the hypothalamus was 42 Gy (26–57 Gy) and pituitary was 44 Gy (26–58 Gy). Patients receiving higher RT doses to the pituitary had a significantly higher incidence of growth hormone deficiency with 4-year estimates of 100 ±3% for those receiving = 44Gy and 82±7% for those receiving < 44 Gy (p=0.024). Four-year estimates of thyroid hormone deficiency for patients receiving = 44 Gy to the pituitary were 67±21% and 18±14% for those receiving < 44 Gy (p<0.010). There was no correlation between the dose to the pituitary with the incidence of ACTH deficiency. The estimated change in height z-score for patients receiving < 44 Gy to the pituitary was - 0.53 units per year (-0.44 to -0.63) compared with -0.70 units per year (-0.62 to -0.78) for those receiving = 44 Gy. Conclusions: Data from this prospective study demonstrate that RT to the HPA is the key determinant for developing endocrinopathies in patients with medulloblastoma/PNET. No significant financial relationships to disclose.

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