The authors analyzed a large, population-based cancer registry to examine the relationship of incidence to patient age, sex, race, and tumor location, and to determine incidence trends over the past 3 decades.Data were obtained from the Surveillance, Epidemiology, and End Results (SEER-9) study, which was conducted from 1973 to 2003. Patients receiving GTR may have better 5-year overall survival than those receiving STR. He is also the Director of the Center for Brain and Behavior. Peginterferon Alfa-2b in Younger Patients With Craniopharyngioma That is Recurrent or Cannot Be Removed By Surgery. We demonstrate a persistent radiation-induced microglial inflammation that is accompanied by nearly complete inhibition of neurogenesis after cancer treatment. Biologic risk stratification of medulloblastoma: The real time is now. Six case-control and 10 cohort studies remained after applying stringent inclusion criteria. A., Ormond, K. E., Hanson-Kahn, A. K. Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial. Schoch, K., Meng, L., Szelinger, S., Bearden, D. R., Stray-Pedersen, A., Busk, O. L., Stong, N., Liston, E., Cohn, R. D., Scaglia, F., Rosenfeld, J. Verbal working memory (VWM) tasks were administered to children following surgical resection of cerebellar pilocytic astrocytomas and age-matched controls. Overall survival (OS) from relapse was significantly longer for radiographic compared with clinical detection (median, 10.8 months; 1-year OS, 46% v median, 5.5 months; 1-year OS, 33%; P = .002), but this trend did not retain significance when analyzed by pathology subgroups.Surveillance neuroimaging detects a proportion of asymptomatic relapses, particularly late relapses, and may provide lead time for other therapies on investigational trials. Childhood primary central nervous system tumors remain a therapeutic conundrum. The National Institutes of Health (NIH) Common Fund supports the Undiagnosed Diseases Network (UDN) as an exemplar of this model of precise diagnosis. Four children experienced clearance of malignant cells from the spinal fluid, but this response was sustained in only two. While tumor location and resection extent affect outcome, pathologic diagnosis when carefully interpreted significantly influences long-term survival. Five-year survival by location was 59.5 +/- SE 5.5% supratentorial, 57.1 +/- SE 4.1% infratentorial and 86.7 +/- SE 5.2% spinal. Geng, L. N., Kohler, J. N., Levonian, P., Bernstein, J. The PRT-C scored 10.3 and 10.5 points higher than the XRT-C in the physical (PhSD) and psychosocial (PsSD) summary domains of the total core score (TCS, p=0.015; p=0.001). Telomerase activation is critical in many cancers including central nervous system (CNS) tumors. A prospective study is needed to formulate a rational surveillance schedule based on the biologic behavior of these tumors. This case illustrates the importance of establishing an accurate neuropathologic tissue diagnosis of all pediatric cortical tumors. Selumetinib in Treating Young Patients With Recurrent or Refractory Low Grade Glioma, Single-agent Erlotinib in Patients Previously Treated With Oral Etoposide in Protocol OSI-774-205, Veliparib, Radiation Therapy, and Temozolomide in Treating Younger Patients With Newly Diagnosed Diffuse Pontine Gliomas, Vismodegib in Treating Younger Patients With Recurrent or Refractory Medulloblastoma, XERECEPT® (hCRF) for Patients Requiring Dexamethasone to Treat Edema Associated With Brain Tumors, XERECEPT® (hCRF) for Primary Glioma Patients Requiring Dexamethasone to Treat Peritumoral Brain Edema. Patients with low-grade glioma were reported to have the highest total HRQOL. The overall and progression-free survival was not significantly different compared to controls.Gadolinium enhancement of the meninges on MRI may occur in a significant number of children with LGA, particularly juvenile pilocytic astrocytoma, but does not appear to affect progression-free or overall survival. Notably, Hu5F9-G4 showed minimal activity against normal human neural cells in vitro and in vivo, a phenomenon reiterated in an immunocompetent allograft glioma model. The frequency of subspecialty consults for diagnostic purposes prior to UDN application was similar in both groups.The preponderance of subjective and lack of objective findings in the Not Accepted applications distinguish these from applicants that are accepted for evaluation and diagnostic efforts through the UDN. Males had significantly higher rates of CNS GCTs than females. Mobley, B. C., McKenney, J. K., Bangs, C. D., Callahan, K., Yeom, K. W., Schneppenheim, R., Hayden, M. G., Cherry, A. M., Gokden, M., Edwards, M. S., Fisher, P. G., Vogel, H. Birth Weight and Order as Risk Factors for Childhood Central Nervous System Tumors. Stanford, CA 94305-3082. Additional investigation is warranted to validate and further define these findings. Arsenic Trioxide and Radiation Therapy in Treating Young Patients With Newly Diagnosed Gliomas. Kirschen, M. P., Davis-Ratner, M. S., Milner, M. W., Chen, S. H., Schraedley-Desmond, P., Fisher, P. G., Desmond, J. E. Congenital glioblastoma multiforme: Case report and review of the literature. Males were more commonly affected than females (58% vs 42%, respectively; p < 0.006). Before surgery, 19 children had visual loss and 15, endocrine deficits; after surgery, 21 children had visual loss and 29, endocrine deficits. Birth defects increased risk for the CNS cancers medulloblastoma (OR: 1.70 [95% CI: 1.12-2.57]), primitive neuroectodermal tumor (OR: 3.64 [95% CI: 1.54-8.56]), and germ cell tumors (OR: 6.40 [95% CI: 2.09-19.56]).Multiple pregnancy losses after 20 weeks' gestation and birth defects increase the risk of a childhood CNS tumor. View details for Web of Science ID 000251383300012. ASXL2 has not yet been associated with a human Mendelian disorder. Paul has 1 job listed on their profile. Shashi, V., Geist, J., Lee, Y., Yoo, Y., Shin, U., Schoch, K., Sullivan, J., Stong, N., Smith, E., Jasien, J., Kranz, P., Undiagnosed Diseases Network, Lee, Y., Shin, Y. Yecies, D., Fisher, P., Cheshier, S., Edwards, M., Grant, G. R-SCAN: Imaging for Pediatric Simple Febrile Seizures. Phase I Trial of Arsenic Trioxide and Stereotactic Radiotherapy for Recurrent Malignant Glioma. The median ADC of 1,295 × 10(-6) mm(2)/s for the cohort partitioned tumors into low or high diffusion groups, which had distinct median survivals of 3 and 13 months, respectively (log-rank p, View details for DOI 10.1007/s11060-014-1375-8. The median lapatinib pre-dose trough concentration was 3.72 µM. Ten consecutive children (aged: 3.5-15.5 years, median: 10.2 years, six male) with high-risk embryonal tumors, including: large cell/anaplastic medulloblastoma (6), atypical teratoid rhabdoid tumor (1), and leptomeningeal primitive neuroectodermal tumor (3), were treated with IV cyclophosphamide 1 g/M(2) on days 1 and 2 of CSI. Dulai, M. S., Caccamo, D. V., Briley, A. L., Edwards, M. S., Fisher, P. C., Lehman, N. L. 50 Years Ago in THE JOURNAL OF PEDIATRICS A Critical Evaluation of Therapy of Febrile Seizures, Oncogenic BRAF Mutation with CDKN2A Inactivation Is Characteristic of a Subset of Pediatric Malignant Astrocytomas. A., Sampson, J. No initial isolated spinal relapse was identified in patients with glioma, supratentorial primitive neuroectodermal tumor and ATRT. De novo EIF2AK1 and EIF2AK2 Variants Are Associated with Developmental Delay, Leukoencephalopathy, and Neurologic Decompensation. All four children with cytologic response received concurrent radiotherapy, chemotherapy, or both. All 30 children have died. Grade 4 toxicities included peri-tracheostomy hemorrhage (n = 1) and elevated creatinine phosphokinase (n = 1). Childhood cancer is increased in those with birth defects, including those with congenital heart disease (CHD). We postulate that damage to left hemispheral lobule VIII may interfere with encoding of auditory stimuli into the phonological store. Isolated spinal recurrences are infrequent in children with malignant CNS tumors and the yield of spine MRI is very low. The incidence of MB and PNET from 1985 to 2002 was determined from the Central Brain Tumor Registry of the United States, a large population-based cancer registry, using strict histologic and site codes. The most common toxicities were hematologic.Daily oral topotecan at a dose of 0.8 mg/m(2)/day can be safely administered to children with recurrent or refractory brain tumors. 50 Years Ago in The Journal of Pediatrics: Neonatal Myasthenia Gravis. A., Schoch, K., Spillmann, R., Undiagnosed Diseases Network, Loscalzo, J., Krier, J., Stoler, J., Sweetser, D., Palmer, C. G., Phillips, J. Long-term health and social function in adult survivors of paediatric astrocytoma: A report from the Childhood Cancer Survivor Study. Hence an approach using chemotherapy initially and reserving radiation for progressive disease is attractive. Co-expression of ERBB2 and ERBB4, reported in 75% of pediatric ependymomas, correlates with worse overall survival. The authors report a rare case of a cortical ependymoma in a 10-year-old boy. View details for DOI 10.1200/JCO.2014.59.5132, View details for PubMedCentralID PMC4507465. View details for Web of Science ID 000076744200006, View details for Web of Science ID 000075744700002, View details for Web of Science ID 000081492800006. Routine microscopy showed a glial tumor with diverse histologic features. A., Karaviti, L., Schlesinger, A. E., Earl, D., Bamshad, M., Savarirayan, R., Doddapaneni, H., Muzny, D., Jhangiani, S. N., Eng, C. M., Gibbs, R. A., Bi, W., Emrick, L., Rosenfeld, J. MB was defined strictly to exclude non-cerebellar primitive neuro-ectodermal tumors. Dr. Fischer graduated from the University of California Davis School of Medicine in 1980. The UDN, which is funded by the National Institutes of Health, was formed in 2014 as a network of seven clinical sites, two sequencing cores, and a coordinating center. Migrant mothers and risks of developmental disabilities in their children. Paul Graham Fisher, MD is part of Stanford Profiles, official site for faculty, postdocs, students and staff information (Expertise, Bio, Research, Publications, and more). That childhood brain tumor program is now one of the largest, comprehensive childhood brain tumor centers for research and care in … Stanford Libraries' official online search tool for books, media, journals, databases, government documents and more. A study from the SEER registry. Shaken baby syndrome and abusive head trauma are real problems. A., Urv, T. K., Vogel, T. P., Waggott, D. M., Wahl, C. E., Walley, N. M., Walsh, C. A., Walker, M., Wambach, J., Wan, J., Wang, L., Wangler, M. F., Ward, P. A., Waters, K. M., Webb-Robertson, B. M., Wegner, D., Westerfield, M., Wheeler, M. T., Wise, A. L., Wolfe, L. A., Woods, J. D., Worthey, E. A., Yamamoto, S., Yang, J., Yoon, A. J., Yu, G., Zastrow, D. B., Zhao, C., Zuchner, S. Molecular Grouping and Outcomes of Young Children with Newly Diagnosed Ependymoma Treated on the Multi-Institutional SJYC07 Trial. Among patients with measurable disease, 8 % achieved complete remission, 56 % had stable disease and 36 % had progression. Posterior fossa syndrome (PFS), also known as cerebellar affective syndrome, is characterized by emotional lability and decreased speech production following injury or surgery to the cerebellum. Intrathecal thiotepa is recommended as a treatment of leptomeningeal metastases (LM) in children, although published data to support this approach are limited. Meet … PK analysis revealed a profile similar to adults; however, an increased area under the curve was observed in pediatric patients. We hypothesized that birth anomalies and a mother's history of previous pregnancy losses, as a proxy for genetic defects, increase the risk for CNS tumors.From the California Cancer Registry, we identified 3733 patients aged 0 to 14 years with CNS tumors, diagnosed from 1988 through 2006 and linked to a California birth certificate. Glioblastoma multiforme patients who received radiotherapy were at lower risks for female breast and prostate cancers, though at an elevated risk for cancers of the thyroid and brain. View details for DOI 10.1016/j.jpeds.2017.01.004. A., Adams, D. R., Aday, A., Alejandro, M. E., Allard, P., Azamian, M. S., Bacino, C. A., Baker, E., Balasubramanyam, A., Barseghyan, H., Batzli, G. F., Beggs, A. H., Behnam, B., Bellen, H. J., Bican, A., Bick, D. P., Birch, C. L., Bonner, D., Boone, B. E., Bostwick, B. L., Briere, L. C., Brokamp, E., Brown, D. M., Brush, M., Burke, E. A., Burrage, L. C., Butte, M. J., Chen, S., Clark, G. D., Coakley, T. R., Cogan, J. D., Colley, H. A., Cooper, C. M., Cope, H., Craigen, W. J., D'Souza, P., Davids, M., Davidson, J. M., Dayal, J. G., Dell'Angelica, E. C., Dhar, S. U., Dipple, K. M., Donnell-Fink, L. A., Dorrani, N., Dorset, D. C., Douine, E. D., Draper, D. D., Eckstein, D. J., Emrick, L. T., Enns, G. M., Eskin, A., Esteves, C., Estwick, T., Fairbrother, L., Fernandez, L., Ferreira, C., Fieg, E. L., Fogel, B. L., Friedman, N. D., Gahl, W. A., Glanton, E., Godfrey, R. A., Goldman, A. M., Goldstein, D. B., Gould, S. E., Gourdine, J. F., Groden, C. A., Gropman, A. L., Haendel, M., Hamid, R., Hanchard, N. A., High, F., Holm, I. Prior research on trends in neuroblastoma incidence has conflicted. Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). A., Wheeler, M. T. A Recurrent De Novo Variant in NACC1 Causes a Syndrome Characterized by Infantile Epilepsy, Cataracts, and Profound Developmental Delay. Dewire, M., Fouladi, M., Stewart, C., Wetmore, C., Hawkins, C., Jacobs, C., Yuan, Y., Goldman, S., Fisher, P., Rytting, M., Bouffet, E., Khakoo, Y., Hwang, E., Foreman, N., Gilbert, M., Gilbertson, R., Gajjar, A. Kelly, M., Park, M., Mihalek, I., Rochtus, A., Gramm, M., Pérez-Palma, E., Axeen, E. T., Hung, C. Y., Olson, H., Swanson, L., Anselm, I., Briere, L. C., High, F. A., Sweetser, D. A., Kayani, S., Snyder, M., Calvert, S., Scheffer, I. E., Yang, E., Waugh, J. L., Lal, D., Bodamer, O., Poduri, A. The vast amount of genomic and molecular data generated over the last 5-10 years encourages optimism that improved risk stratification and new molecular targets will improve outcomes. Individual studies generally have lacked samples of sufficient size to examine interactions. A., Bhoj, E., Kaplan, P., Li, D., Oegema, R., van Binsbergen, E., van der Zwaag, B., Smeland, M. F., Cutcutache, I., Page, M., Armstrong, M., Lin, A. E., Steeves, M. A., Hollander, N. d., Hoffer, M. J., Reijnders, M. R., Demirdas, S., Koboldt, D. C., Bartholomew, D., Mosher, T. M., Hickey, S. E., Shieh, C., Sanchez-Lara, P. A., Graham, J. M., Tezcan, K., Schaefer, G. B., Danylchuk, N. R., Asamoah, A., Jackson, K. E., Yachelevich, N., Au, M., Pérez-Jurado, L. A., Kleefstra, T., Penzes, P., Wood, S. A., Burne, T., Pierson, T. M., Piper, M., Gécz, J., Jolly, L. A. Genomics in medicine: a novel elective rotation for internal medicine residents. Korones, D. N., Fisher, P. G., Cohen, K. J., Dubowy, R. L. Meningeal leukemia with cerebrospinal fluid block, Case study: Suprasellar germinoma presenting with psychotic and obsessive-compulsive symptoms. Although the combination of bevacizumab and lapatinib was well tolerated in children with recurrent ependymoma, it proved ineffective. View details for Web of Science ID 000183529400012. 97 % of all relapses occurred in the brain and 3 % were isolated to the spine. Data were stratified by histology, age, and stage.Overall peripheral neuroblastic tumor incidence increased by an APC of 0.47 (P=0.045).
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