Tectal plate tumours. Our experience with a paediatric surgical series.
CONCLUSION: Exophytic tectal plate tumours can be treated based on a microsurgical approach in paediatric patients. In experienced hands surgery can be performed with an acceptable morbidity and with zero percent mortality. In our experience, the sub-occipital transtentorial approach permits a wide view of the region and safe surgical removal.
PMID: 25441707 [PubMed - as supplied by publisher] (Source: Neuro-Chirurgie)
Source: Neuro-Chirurgie - October 16, 2014 Category: Neurosurgery Authors: Mottolese C, Szathmari A, Beuriat PA, Frappaz D, Jouvet A, Hermier M Tags: Neurochirurgie Source Type: research
Abstract PR03: The genomic landscape of diffuse intrinsic pontine glioma and pediatric non-brainstem high-grade glioma
Pediatric high-grade glioma (HGG) remains a tremendous clinical challenge, with a two-year survival of less than 20%. We analyzed 127 pediatric HGGs, including diffuse intrinsic pontine gliomas (DIPGs) and non-brainstem HGGs (NBS-HGGs) by whole-genome, whole-exome, and/or transcriptome sequencing. Somatic mutations in the bone morphogenetic protein (BMP) receptor ACVR1 occurred in 32% of DIPG, a finding exclusive to brainstem HGG. Structural variants generating fusion genes were found in 47% of pediatric HGG, with recurrent fusions involving the neurotrophin receptor genes NTRK1, 2, or 3 in 40% of infant NBS-HGGs and 5% of...
Source: Cancer Research - October 9, 2014 Category: Cancer & Oncology Authors: Diaz, A. K., Wu, G., Paugh, B. S., Li, Y., Zhu, X., Rankin, S., Qu, C., Chen, X., Zhang, J., Easton, J., Edmonson, M., Lu, C., Nagahawatte, P., Hedlund, E., Rusch, M., Pounds, S., Lin, T., Onar–Thomas, A., Huether, R., Kriwacki, R., Parker, M., Gupt Tags: Brain Tumors Source Type: research
Erratum to: Temozolomide after radiotherapy in recurrent ‘‘low grade’’ diffuse brainstem glioma in adults
(Source: Journal of Neuro-Oncology)
Source: Journal of Neuro-Oncology - October 9, 2014 Category: Cancer & Oncology Source Type: research
Incidence and dosimetric parameters of pediatric brainstem toxicity following proton therapy.
Conclusion. Utilization of current national brainstem dose guidelines is associated with a low risk of brainstem toxicity in pediatric patients. For young patients with posterior fossa tumors, particularly those who undergo aggressive surgery, our data suggest more conservative dosimetric guidelines should be considered.
PMID: 25279957 [PubMed - as supplied by publisher] (Source: Acta Oncologica)
Source: Acta Oncologica - October 3, 2014 Category: Cancer & Oncology Authors: Indelicato DJ, Flampouri S, Rotondo RL, Bradley JA, Morris CG, Aldana PR, Sandler E, Mendenhall NP Tags: Acta Oncol Source Type: research
Histopathological spectrum of paediatric diffuse intrinsic pontine glioma: diagnostic and therapeutic implications
Abstract
Diffuse intrinsic pontine glioma (DIPG) is the main cause of brain tumour-related death in children. In the majority of cases diagnosis is based on clinical and MRI findings, resulting in the scarcity of pre-treatment specimens available to study. Our group has developed an autopsy-based protocol to investigate the histologic and biologic spectrum of DIPG. This has also allowed us to investigate the terminal pattern of disease and gain a better understanding of what challenges we are facing in treating DIPG. Here, we review 72 DIPG cases with well documented clinical history and molecular data ...
Source: Acta Neuropathologica - September 25, 2014 Category: Neurology Source Type: research
The Genetic Signatures of Pediatric High-Grade Glioma: No Longer a One-Act Play
Advances in understanding pediatric high-grade glioma (pHGG) genetics have revealed key differences between pHGG and adult HGG and have uncovered unique molecular drivers among subgroups within pHGG. The 3 core adult HGG pathways, the receptor tyrosine kinase-Ras-phosphatidylinositide 3-kinase, p53, and retinoblastoma networks, are also disrupted in pHGG, but they exhibit a different spectrum of effectors targeted by mutation. There are also similarities and differences in the genomic landscape of diffuse intrinsic pontine glioma (DIPG) and pediatric nonbrainstem (pNBS)-HGG. In 2012, histone H3 mutations were identified in...
Source: Seminars in Radiation Oncology - September 15, 2014 Category: Cancer & Oncology Authors: Alexander K. Diaz, Suzanne J. Baker Source Type: research
Molecular genetics of ependymomas and pediatric diffuse gliomas: a short review
Abstract
Here, we review the recent literature on molecular discoveries in ependymomas and pediatric diffuse gliomas. Ependymomas can now be categorized into three location-related subgroups according to their biological profile: posterior fossa ependymomas, group A (PFA) and B (PFB), and supratentorial ependymomas. Although no recurrently mutated genes were found throughout these groups of ependymomas, PFA exhibited a CpG island methylator phenotype, PFB was associated with extensive chromosomal aberrations, and the C11orf95-RELA fusion gene was frequently observed in supratentorial ependymomas. Meanwhil...
Source: Brain Tumor Pathology - September 3, 2014 Category: Neurology Source Type: research
ACVR1 in FOP and DIPG
Whole-genome sequencing studies have recently identified a quarter of cases of the rare childhood brainstem tumor diffuse intrinsic pontine glioma to harbor somatic mutations in ACVR1. This gene encodes the type I bone morphogenic protein receptor ALK2, with the residues affected identical to those that, when mutated in the germline, give rise to the congenital malformation syndrome fibrodysplasia ossificans progressiva (FOP), resulting in the transformation of soft tissue into bone. This unexpected link points toward the importance of developmental biology processes in tumorigenesis and provides an extensive experience in...
Source: Cancer Research - September 1, 2014 Category: Cancer & Oncology Authors: Taylor, K. R., Vinci, M., Bullock, A. N., Jones, C. Tags: Review Source Type: research
Long-term tumor control of spinal dissemination of cerebellar glioblastoma multiforme by combined adjuvant bevacizumab antibody therapy: a case report.
Conclusion:
Due to the limited overall prognosis of the disease, spinal manifestations of glioma are rarely clinically relevant. The results of our instructive case, however, with a positive effect on both life quality and survival warrant treating future patients in the frame of a prospective clinical study. (Source: BMC Research Notes)
Source: BMC Research Notes - August 7, 2014 Category: Research Authors: Thomas LinsenmannCamelia MonoranuGiles VinceThomas WestermaierCarsten HagemannAlmuth KesslerRalf-Ingo ErnestusMario Löhr Source Type: research
Antigen-Specific Immune Responses and Clinical Outcome After Vaccination With Glioma-Associated Antigen Peptides and Polyinosinic-Polycytidylic Acid Stabilized by Lysine and Carboxymethylcellulose in Children With Newly Diagnosed Malignant Brainstem and Nonbrainstem Gliomas [Pediatric Oncology]
Conclusion
GAA peptide vaccination in children with gliomas is generally well tolerated and has preliminary evidence of immunologic and clinical responses. Careful monitoring and management of pseudoprogression is essential. (Source: Journal of Clinical Oncology)
Source: Journal of Clinical Oncology - June 27, 2014 Category: Cancer & Oncology Authors: Pollack, Jakacki, Butterfield, Hamilton, Panigrahy, Potter, Connelly, Dibridge, Whiteside, Okada Tags: Brain Tumors, CNS Tumors Pediatric Oncology Source Type: research
Exome sequencing identifies somatic gain-of-function PPM1D mutations in brainstem gliomas
This study led to the discovery of tumor-specific mutations in PPM1D, encoding wild-type p53–induced protein phosphatase 1D (WIP1), in 37.5% of the BSGs that harbored hallmark H3F3A mutations encoding p.Lys27Met substitutions. PPM1D mutations were mutually exclusive with TP53 mutations in BSG and attenuated p53 activation in vitro. PPM1D mutations were truncating alterations in exon 6 that enhanced the ability of PPM1D to suppress the activation of the DNA damage response checkpoint protein CHK2. These results define PPM1D as a frequent target of somatic mutation and as a potential therapeutic target in brainstem gli...
Source: Nature Genetics - June 1, 2014 Category: Genetics & Stem Cells Authors: Liwei ZhangLee H ChenHong WanRui YangZhaohui WangJie FengShaohua YangSiân JonesSizhen WangWeixin ZhouHuishan ZhuPatrick J KillelaJunting ZhangZhen WuGuilin LiShuyu HaoYu WangJoseph B WebbHenry S FriedmanAllan H FriedmanRoger E McLendonYiping HeZachary J Tags: Letter Source Type: research
[Low-grade gliomas: Review of 10 years.]
CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae.
PMID: 24863616 [PubMed - as supplied by publisher] (Source: Anales de Pediatria)
Source: Anales de Pediatria - May 23, 2014 Category: Pediatrics Authors: Pardal Souto MJ, Hernández Marqués C, Lassaletta Atienza A, Ruano D, Cormenzana M, Madero L Tags: An Pediatr (Barc) Source Type: research
Genetics: ACVR1 mutations—a key piece in paediatric diffuse glioma
Nature Reviews Clinical Oncology 11, 300 (2014).
doi:10.1038/nrclinonc.2014.74
Author: M. Teresa Villanueva
High-grade glioma—or high-grade astrocytoma (HGA)—have a devastating prognosis, with survival ranging from 20–30% for tumours outside the brainstem, and less than 10% for diffuse intrinsic pontine gliomas (DIPGs), which arise in the brainstem and are found almost exclusively in children. Aside from radiotherapy, there is (Source: Nature Clinical Practice Oncology)
Source: Nature Clinical Practice Oncology - May 20, 2014 Category: Cancer & Oncology Authors: M. Teresa Villanueva Tags: Research Highlight Source Type: research
Oligodendroglial Gliomatosis Cerebri Exhibiting Leptomeningeal And Nerve Root Involvement (P7.271)
Conclusions:In the context of gliomatosis cerebri, the extent and infiltrative pattern, the positive CSF cytology and the histological subtype, make this case highly unusual. The lack of specific findings both clinically and on imaging, despite the level of neoplastic infiltration seen post-mortem, highlight the diagnostic challenge this neoplasm presents.Disclosure: Dr. Mcmacken has nothing to disclose. Dr. Herron has nothing to disclose. Dr. Fulton has nothing to disclose. (Source: Neurology)
Source: Neurology - April 9, 2014 Category: Neurology Authors: Mcmacken, G., Herron, B., Fulton, A. Tags: Neuro-oncology: Primary CNS Tumors: Gliomas Source Type: research
Brainstem Glioma 8 Years After External Beam Radiotherapy for Pituitary Macroadenoma: Correlation with Radiation Treatment Dose and Volume (P7.257)
CONCLUSIONS: The rare occurrence of brainstem RAG after radiation therapy for pituitary tumors supports the need for long-term imaging monitoring of such patients.Disclosure: Dr. Abboud has nothing to disclose. Dr. Wolansky has nothing to disclose. Dr. Manjila has nothing to disclose. Dr. Lo has nothing to disclose. Dr. Arafah has nothing to disclose. Dr. Rogers has received personal compensation for activities with Sigma Tau Pharmaceuticals. (Source: Neurology)
Source: Neurology - April 9, 2014 Category: Neurology Authors: Abboud, S., Wolansky, L., Manjila, S., Lo, S., Arafah, B., Selman, W., Rogers, L. Tags: Neuro-oncology: CNS Metastases and Complications of Treatment Source Type: research
