Advances Toward an Understanding of Brainstem Gliomas

Donaldson, Sarah S.; Laningham, Fred H.; Fisher, Paul G.

doi:10.1200/jco.2005.04.6599

Cited by 220 publications

(164 citation statements)

References 47 publications

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“…BSG make up 1% of all brain tumors of adults and 10% of all brain tumors pediatric ages. Initial presentation of these tumors is represented by two peaks of incidence at 4-13 years and the fourth decade of life (9,15,30). Supratentorial brain gliomas have a better prognosis in children than in adults, except glioma localized in the area of the brainstem where survival in children is significantly shorter than in adults (1,3,22,27).…”

Section: █ Resultsmentioning

confidence: 99%

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Adult brainstem gliomas - retrospective analysis of 51 patients

Tasić¹,

Repac²,

Nikolić³

et al. 2016

Turkish Neurosurgery

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ABSTRACTthe specific pathology all BSG must be considered malignant because their localization itself is inoperable (23). These tumors are most often localized in pons, often with infiltration in other regions of the brainstem (16). They characteristically present with multiple cranial nerve deficits, ataxia and long tract dysfunction (5,6,10). The simplest classification of BSG is into two groups as focal and diffuse (4,8). More complex grading system defined subtypes of tumors of the brainstem, according to localization, the presence of hydrocephalus and hemorrhage and growth model (5,7,11,13).umors of the central nervous system are present in about 2% compared to all malignant tumors of the human population. They represent the leading cause of death from malignant tumors in children population and fourth in the adult population (21). In the period before computed tomography (CT) and magnetic resonance imaging (MRI), all brainstem gliomas (BSG) were considered a pathological entity with a poor prognosis. Matson emphasized that regardless of RESUlTS:Of the 51 patients, 62.7% were male and 37.3% were female. The mean age was 30.6±19.3 years. High grade glioma (Astrocytoma grade III and IV) was most common at the age of 38.2±17.9 years (t=.481, p=0.017) while low grade glioma (Astrocytoma grade I and II) was common in younger age as 25.4±17.4 years (X 2 =4.013; p=0.045), with localization in the pons (X 2 =5.299; p=0.021) and exophytic presentation (X 2 =3.862; p=0.049). Ataxia, as initial symptom, was a predictor of poor outcome (HR:5.546, p=0.012).CONClUSION: Due to its specific localization, BSG present a major challenge for neurosurgery, because of the necessity of safe approach for radical resection. Histological verification of BSG determines the need for additional therapeutic procedures such as radiotherapy and chemotherapy. Benefit from correct diagnosis is reflected in the avoidance of potentially adverse effects of treatment.

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Section: █ Resultsmentioning

confidence: 99%

“…█ REFERENCES fact that diffuse BSG rarely stain with contrast (8,9,28). The short time interval between the occurrence of symptoms and the diagnosis, the presence of intra-tumoral necrosis and histologic verification of high grade BSG are predictors of shorter survival period (15,25,28,30).…”

Section: Follow-up In Months Follow-up In Monthsmentioning

confidence: 99%

Adult brainstem gliomas - retrospective analysis of 51 patients

Tasić¹,

Repac²,

Nikolić³

et al. 2016

Turkish Neurosurgery

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“…DIPGs primarily affect very young children, with peak incidence at 6 y of age, and have the highest mortality of all childhood solid tumors. 28 One of the obstacles to improving therapies is that the spectrum of molecular alterations is quite different in tumors from children and adults. Historically, pediatric highgrade glioma was considered similar to secondary glioblastoma multiform (GBM), an adult high-grade glioma that evolves from a less malignant precursor due to changes in gene expression, such as OLIG1 and OLIG2, important neuro-developmental genes.…”

Section: High-grade Gliomamentioning

confidence: 99%

Epigenetic modification in chromatin machinery and its deregulation in pediatric brain tumors: Insight into epigenetic therapies

Maury

Hashizume

2017

Epigenetics

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Malignancies are characterized by the reprogramming of epigenetic patterns. This reprogramming includes gains or losses in DNA methylation and disruption of normal patterns of covalent histone modifications, which are associated with changes in chromatin remodeling processes. This review will focus on the mechanisms underlying this reprogramming and, specifically, on the role of histone modification in chromatin machinery and the modifications in epigenetic processes occurring in brain cancer, with a specific focus on epigenetic therapies for pediatric brain tumors.

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“…Those arising in the dorsal pons (pontine tegmentum), midbrain, or medulla, in contrast, tend to be low-grade gliomas (often pilocytic astrocytomas or gangliogliomas) with a markedly more favorable course of survival, sometimes for years or decades (2,3). DIPGs of the ventral pons peak in incidence in middle childhood (at the age of 6-7 y) and are rare in adulthood (3,4) (Fig. 1B).…”

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confidence: 99%

“…Biopsy of this tumor is rare, because the diagnosis can typically be made by MRI appearance alone and brainstem surgery poses a significant risk of morbidity. With little human tissue available for study and no realistic animal models, few published studies address the underlying biology of DIPG and no advances in treatment have occurred since the application of radiation therapy 35 y ago (4,14).…”

mentioning

confidence: 99%

Hedgehog-responsive candidate cell of origin for diffuse intrinsic pontine glioma

Monje

Mitra

Freret

et al. 2011

Proc. Natl. Acad. Sci. U.S.A.

276

300

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Diffuse intrinsic pontine gliomas (DIPGs) are highly aggressive tumors of childhood that are almost universally fatal. Our understanding of this devastating cancer is limited by a dearth of available tissue for study and by the lack of a faithful animal model. Intriguingly, DIPGs are restricted to the ventral pons and occur during a narrow window of middle childhood, suggesting dysregulation of a postnatal neurodevelopmental process. Here, we report the identification of a previously undescribed population of immunophenotypic neural precursor cells in the human and murine brainstem whose temporal and spatial distributions correlate closely with the incidence of DIPG and highlight a candidate cell of origin. Using early postmortem DIPG tumor tissue, we have established in vitro and xenograft models and find that the Hedgehog (Hh) signaling pathway implicated in many developmental and oncogenic processes is active in DIPG tumor cells. Modulation of Hh pathway activity has functional consequences for DIPG selfrenewal capacity in neurosphere culture. The Hh pathway also appears to be active in normal ventral pontine precursor-like cells of the mouse, and unregulated pathway activity results in hypertrophy of the ventral pons. Together, these findings provide a foundation for understanding the cellular and molecular origins of DIPG, and suggest that the Hh pathway represents a potential therapeutic target in this devastating pediatric tumor.Hedgehog pathway | cancer stem cells | brainstem glioma

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Advances Toward an Understanding of Brainstem Gliomas

Cited by 220 publications

References 47 publications

Adult brainstem gliomas - retrospective analysis of 51 patients

Adult brainstem gliomas - retrospective analysis of 51 patients

Epigenetic modification in chromatin machinery and its deregulation in pediatric brain tumors: Insight into epigenetic therapies

Hedgehog-responsive candidate cell of origin for diffuse intrinsic pontine glioma

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