Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis

Rutkowski, Stefan; Hoff, Katja von; Emser, Angela; Zwiener, Isabella; Pietsch, Torsten; Figarella‐Branger, Dominique; Giangaspero, Felice; Ellison, David W.; Garrè, Maria Luisa; Biassoni, Veronica; Grundy, Richard G.; Finlay, Jonathan L.; Dhall, Girish; Raquin, Marie Anne; Grill, Jacques

doi:10.1200/jco.2010.30.2299

Cited by 278 publications

(203 citation statements)

References 41 publications

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“…Meduloblastoma is a brain tumor of very poor prognosis whose treatment consists basically in surgery, radiotherapy and chemotherapy which can leave important sequelae in children [3]. For this reason research on molecular therapies is important to find alternative or complementary ways to make the treatment of medulloblastomas more effective.…”

Section: Discussionmentioning

confidence: 99%

“…There are genetic syndromes that can predispose to the development of medulloblastoma, like Gorling, Turcot, and Li-Fraumeni syndromes, although medulloblastomas associated to these syndromes happen in less than 7% of the cases [2]. The treatment of this embryonal tumor includes surgery, radiotherapy and chemotherapy, but due to the aggresiveness of these treatments patients suffer devastating neurocognitive sequelae [3]. For this reason, understanding the molecular mechanisms of the pathogenesis of medulloblastoma will help to find new specific treatments [1].…”

Section: Introductionmentioning

confidence: 99%

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EZH2 and Sonic Hedgehog Inhibition Reduce Proliferation, Migration, In Vitro Tumorigenesis, and CD133 Expression in Desmoplastic Medulloblastoma Cells

Rosa¹,

Tapia²,

Shahi³

et al. 2017

J of Tumor Res & Reports

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Medulloblastoma is the malignant brain tumor that most affects children and young people. Its treatment is very aggressive and can leave important neurocognitive sequelae in patients. Medulloblastoma can be classified histologically and molecularly in different subtypes. Our work focuses on the specific subtype in which the sonic hedgehog pathway is altered. DAOY cells, which correspond to desmoplastic Shh medulloblastoma, were independently treated with two pharmacological inhibitors: cyclopamine and DZNep. Cyclopamine directly inhibits Smo, thus inhibiting the sonic hedgehog pathway; while DZNep acts at the epigenetic level by inhibiting EZH2 function, a histone-lysine N-methyltransferase. The two inhibitions were compared cellularly and molecularly, demonstrating that both drugs reduced cell viability, colony formation, cell migration and the expression of cancer stem cells related genes, like CD133. In addition, the expression of different genes of the sonic hedgehog, EZH2, and other genes regulated by EZH2 and GLI1 were evaluated. The initial hypothesis, according to which the expression of EZH2 would regulate the sonic hedgehog pathway was not demonstrated. Quite the opposite, we observed that the sonic hedgehog pathway could positively regulate EZH2 expression. Our results, however, should be subjected to further experiments in order to elucidate the relationship between the epigenetic regulation exerted by EZH2 and the regulation of the sonic hedgehog pathway in medulloblastoma.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

EZH2 and Sonic Hedgehog Inhibition Reduce Proliferation, Migration, In Vitro Tumorigenesis, and CD133 Expression in Desmoplastic Medulloblastoma Cells

Rosa¹,

Tapia²,

Shahi³

et al. 2017

J of Tumor Res & Reports

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“…Los síntomas incluyen cefaleas diurnas o nocturnas, náusea, vómito y alteración del estado de consciencia. Los tumores de la línea media pueden producir marcha atáxica o inestabilidad truncal, [47][48][49][50][51][52][53][54] contraste, los que tienen meduloblastomas anaplásicos o de células grandes tiene un peor pronóstico 34 .…”

Section: Manifestaciones Clínicasunclassified

Meduloblastoma: de la clasificación histológica a la molecular

Pabón

Hoyos

Restrepo

et al. 2018

MedUPB

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RESUMENEl meduloblastoma es un tumor frecuente en la población pediátrica, pero es raro en pacientes adultos. Es la segunda causa de muerte por cáncer en menores de 15 años. El hallazgo de una lesión tumoral cerebelosa mediana o paramediana que capta el medio de contraste y que a menudo comprime el cuarto ventrículo sugiere la presencia de este tumor. A través del uso de estudios de expresión génica y marcadores moleculares se ha generado una nueva aproximación a la clasificación del meduloblastoma. Así, se ha venido a entender el concepto de meduloblastoma no como una patología sino como un grupo de patologías distintas clínica y molecularmente. La resonancia magnética nuclear espinal y la punción lumbar deben ser realizadas en todos los pacientes como parte de la evaluación de la extensión de la enfermedad, debido a que las leptomeninges espinales son un sitio frecuente de diseminación. El tratamiento implica idealmente la interacción de un grupo interdisciplinario que pueda ofrecer al paciente: cirugía, radioterapia y quimioterapia. El pronóstico depende de variables como edad (menor de tres años), diseminación de la enfermedad, residuo tumoral posquirúrgico, variante histológica de células grandes/anaplásico, y pertenecer al grupo 3 (grupo de amplificacón del MYC).Palabras clave: cerebelo; meduloblastoma; expresión génica; clasificación. ABSTRACTMedulloblastoma is a common tumor in children, but is rare in adults. It is the second most common cause of cancer-related death in patients under 15 years. The presence of a median or paramedian enhancing cerebellar mass, often compressing the fourth ventricle may indicate the presence of this tumor. Genetic and molecular markers offer a new approach to the understanding and classification of medulloblastomas. Hence, we have come to understand medulloblastoma not as a sole disease but rather, as a group of clinically and molecularly distinct pathologies. Magnetic resonance imaging of the spine and lumbar puncture must be performed in all patients as part of the assessment of the extent of disease, since spinal leptomeninges are common sites for metastatic dissemination. Ideally, its treatmeant involves an interdisciplinary group that provides surgery, radiotherapy, and chemotherapy. Prognosis depends upon variables such as age (under three years), metastatic dissemination, residual tumor after surgery, large cell/ anaplastic histological variant, and group 3 tumor (MYC amplification group).

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“…The interaction between biological markers and these treatment strategies in this age group is yet to be studied [8,58,110]. The role of chemotherapy is, therefore, established.…”

Section: Drug Administrationmentioning

confidence: 99%

Medulloblastoma in childhood: revisiting intrathecal therapy in infants and children

Conroy

Garnett

Vloeberghs

et al. 2009

Cancer Chemother Pharmacol

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Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis

Abstract: Our results confirm the high frequency of desmoplastic variants of medulloblastomas in early childhood and histopathology as a strong independent prognostic factor. A controlled de-escalation of treatment may be appropriate for young children with DNMB and MBEN in future clinical trials.

Cited by 278 publications

References 41 publications

EZH2 and Sonic Hedgehog Inhibition Reduce Proliferation, Migration, In Vitro Tumorigenesis, and CD133 Expression in Desmoplastic Medulloblastoma Cells

EZH2 and Sonic Hedgehog Inhibition Reduce Proliferation, Migration, In Vitro Tumorigenesis, and CD133 Expression in Desmoplastic Medulloblastoma Cells

Meduloblastoma: de la clasificación histológica a la molecular

Medulloblastoma in childhood: revisiting intrathecal therapy in infants and children

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