Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET).
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.
Brandes
Alba A
AA
Department of Medical Oncology, Azienda USL-Bellaria-Maggiore Hospital Bologna, Italy. alba.brandes@yahoo.it
Franceschi
Enrico
E
Tosoni
Alicia
A
Reni
Michele
M
Gatta
Gemma
G
Vecht
Charles
C
Kortmann
Rolf D
RD
D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D008297:Male; D008527:Medulloblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D011379:Prognosis; D055815:Young Adult