Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET)

Abstract Medulloblastoma and supratentorial primitive neuroectodermal tumors are rare diseases in adults. Due to this rarity, few prospective clinical trials have been conducted on medulloblastoma in adults, investigations being based exclusively on retrospective studies; the populations considered...

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Bibliographic Details
Published in:Critical reviews in oncology/hematology 2009-08, Vol.71 (2), p.165-179
Main Authors: Brandes, Alba A, Franceschi, Enrico, Tosoni, Alicia, Reni, Michele, Gatta, Gemma, Vecht, Charles, Kortmann, Rolf D
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Brain Neoplasms - diagnosis
Brain Neoplasms - epidemiology
Brain Neoplasms - genetics
Brain Neoplasms - therapy
Chemotherapy
Child
Female
Genetic Predisposition to Disease
Hematology, Oncology and Palliative Medicine
Humans
Incidence
Long-term survival
Male
Medical sciences
Medulloblastoma
Medulloblastoma - diagnosis
Medulloblastoma - epidemiology
Medulloblastoma - therapy
Middle Aged
Neoplasm Recurrence, Local - prevention & control
Neoplasm Staging
Neuroectodermal Tumors, Primitive - diagnosis
Neuroectodermal Tumors, Primitive - epidemiology
Neuroectodermal Tumors, Primitive - therapy
Neurology
Prognosis
Radiotherapy
Supratentorial PNET
Surgery
Tumors of the nervous system. Phacomatoses
Young Adult
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Summary:Abstract Medulloblastoma and supratentorial primitive neuroectodermal tumors are rare diseases in adults. Due to this rarity, few prospective clinical trials have been conducted on medulloblastoma in adults, investigations being based exclusively on retrospective studies; the populations considered in literature are small, and the different treatments given span decades, during which diagnostic procedures, neurosurgical skills and radiotherapy techniques have changed. Unlike pediatric patients, adult medulloblastoma patients have been treated according to risk-adapted therapeutic strategies in only a few series and despite risk-tailored treatments, 20–30% of patients experience recurrence. Although patients could respond to second line treatments, the prognosis of relapsed patients remains dismal. An important challenge for the future will be the biological characterization of medulloblastoma, with the identification of specific genetic patterns of patients with a better or a worse prognosis.
ISSN:1040-8428
1879-0461
DOI:10.1016/j.critrevonc.2009.02.002