Pediatric Central Nervous System Tumors

Pinho, Ricardo Silva; Andreoni, Solange; Silva, Nasjla Saba; Cappellano, Andréa; Masruha, Marcelo Rodrigues; Cavalheiro, Sérgio; Vilanova, Luiz Celso Pereira

doi:10.1097/mph.0b013e31822031d9

Cited by 19 publications

(9 citation statements)

References 14 publications

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“…Other studies around the world have reported similar ratios: 1.1 in France (population-based study), 1.31 in Japan (population-based study), and 1.27 in Brazil (single-center study) [9,10,12]. From our data, males accounted for 74.4% of the germ cell tumors in the pediatric cases, a percentage that was similar to the 69.6% reported by CBTRUS and to those reported by other studies from various regions [8-12], indicating that intrinsic factors also play important roles in oncogenesis.…”

Section: Discussionmentioning

confidence: 52%

Central nervous system tumors: a single center pathology review of 34,140 cases over 60 years

et al. 2013

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BackgroundTumor epidemiology is a significant part of CNS (central nervous system) tumor studies. Reassessment of original sections can update our knowledge of tumor spectrum. Here, we discuss the features of CNS tumor pathology in a single center.MethodsA total of 34140 cases from 1950 to 2009 were collected; sections from 1990 to 2009 were reassessed according to WHO 2007 classification, and cases from 1950 to 1989 were classified according to the previous pathological diagnosis.ResultsSeven CNS tumor categories during 1990 to 2009 were as follow: neuroepithelial tissue (38.0%), tumors of the meninges (36.5%), tumors of the sellar region (4.1%), germ cell tumors (1.3%), tumors of cranial and paraspinal nerves (13.3%), lymphomas and hematopoietic neoplasm (1.7%), metastatic tumors (5.1%), where histological types by age and sex were diverse. Overall, males exceeded females in distributions of most CNS tumor subtypes, while tumors of the meninges occurred more frequently in females. The case number of lymphomas and hematopoietic neoplasms grew the fastest during the past five years, and the distribution of neuroepithelial tumors remained stable over the past twenty years.ConclusionsDespite the possibilities of cross sample biases, the data in this series could suggest a similar CNS tumor spectrum as might occur in other developing countries.

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Section: Discussionmentioning

confidence: 52%

Central nervous system tumors: a single center pathology review of 34,140 cases over 60 years

et al. 2013

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“…In other studies from Brazil, Nigeria, Pakistan, Medulloblastomas were the second most common. [ 18 20 24 ]…”

Section: Discussionmentioning

confidence: 99%

Profile and outcome of pediatric brain tumors – Experience from a tertiary care pediatric oncology unit in South India

et al. 2017

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Context:Tumors of the central nervous system (CNS) constitute the second most common pediatric cancers. Unlike leukemia, management of CNS tumors requires a good multidisciplinary team. Higher rates of treatment abandonment are documented in view of complexity of the treatment with long duration, involving neurosurgery, radiation, chemotherapy, and high cost of treatment. Morbidity associated with CNS tumors may be significant in terms of physical deficits as well as neuropsychological and neuroendocrine sequelae. Pediatric neurooncology is still at a very nascent stage in the developing countries. There are only a few reports on the multidisciplinary approach and outcomes of pediatric brain tumors in developing countries.Aims:The aim of this study is to identify the clinicopathological profile of Pediatric CNS tumors in a tertiary care center located in South India in comparison with reports from other low-and middle-income Countries.Settings and Design:A retrospective analysis of medical records of all children diagnosed with brain tumors from January 2012 to November 2016 at our institute was done.Subjects and Methods:A retrospective study of clinical, pathological profile, and outcomes of children <18 years diagnosed with brain tumors at our institute from January 2012 to November 2016 was done. Histopathological categorization was done as per the WHO classification 2007. The multidisciplinary treatment with respect to surgery, radiation, and chemotherapy was noted and the outcomes were recorded.Statistical Analysis Used:R for Statistical Computing (Version 3.0.2; 2013-09-25).Results:A total of 52 children were diagnosed with male preponderance of 66.6%. Highest incidence was noted in the age group of 0–4 years (50%). Majority of them were supratentorial (59.6%). CNS embryonal tumors contributed to 48% of all our brain tumors. 73% of them underwent either resection or biopsy. Eight (15.3%) of them died due to the progression of disease, but 44% abandoned treatment due to the progression/recurrence of disease. Those lost to follow-up were mostly among the high-risk groups with poor prognosis such as pontine glioma, medulloblastoma (high risk), and primitive neuroectodermal tumor.Conclusions:Although brain tumors constituted 30% of all our solid tumors, only 56% of them received appropriate treatment and 25% abandoned treatment. High rates of abandonment were a consequence of late diagnosis, complex multidisciplinary treatment involved, high treatment cost, lack of uniformity in management between different oncology centers and poor prognosis of the tumor subtype.

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“…It is characterized by four major subgroups, one of which displays constitutively activated Sonic Hedgehog (SHH) signaling. 14–17 BMP2 and BMP4 antagonize SHH signaling, 18 and BMP4 signaling induces irreversible differentiation of cerebellar granule neuronal progenitors (GNPs) and tumor cells from the SHH medulloblastoma subgroup. 19 This finding suggested that BMP agonists might be useful as differentiation therapy for SHH medulloblastoma.…”

Section: Introductionmentioning

confidence: 99%

Ventromorphins: A New Class of Small Molecule Activators of the Canonical BMP Signaling Pathway

et al. 2017

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Here we describe three new small-molecule activators of BMP signaling found by high throughput screening of a library of ~600,000 small molecules. Using a cell-based luciferase assay in the BMP4-responsive human cervical carcinoma clonal cell line, C33A-2D2, we identified three compounds with similar chemotypes that each ventralize zebrafish embryos and stimulate increased expression of the BMP target genes, bmp2b and szl. Because these compounds ventralize zebrafish embryos, we have termed them “ventromorphins.” As expected for BMP pathway activators, they induce the differentiation of C2C12 myoblasts to osteoblasts. Affymetrix RNA analysis confirmed the differentiation results and showed that ventromorphins treatment elicits a response similar to BMP4 treatment. Unlike Isoliquiritigenin (SJ000286237), a flavone that maximally activates the pathway after 24 hours of treatment, all three ventromorphins induced SMAD1/5/8 phosphorylation within 30 minutes of treatment and achieved peak activity within 1 hour, indicating that their responses are consistent with directly activated BMP signaling.

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Pediatric Central Nervous System Tumors

Cited by 19 publications

References 14 publications

Central nervous system tumors: a single center pathology review of 34,140 cases over 60 years

Central nervous system tumors: a single center pathology review of 34,140 cases over 60 years

Profile and outcome of pediatric brain tumors – Experience from a tertiary care pediatric oncology unit in South India

Ventromorphins: A New Class of Small Molecule Activators of the Canonical BMP Signaling Pathway

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