Outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) - a retrospective analysis spanning 40 years of treatment.
BACKGROUND
Medulloblastoma (MB) and supratentorial primitive neuroectodermal tumor of the central nervous system (CNS-PNET) are among the most common pediatric brain tumors. The diagnosis, treatment, and outcome of MB/CNS-PNET patients treated during the last four decades at Oslo University Hospital (OUH) are described.


METHODS
All patients younger than 20 years of age diagnosed and treated for MB/CNS-PNET at OUH between 1 January 1974 and 31 December 2013 were identified.


RESULTS
We found 175 patients. In 13 of them, the diagnosis was changed upon histopathological review and in 4 patients part of the treatment was administered at other hospitals. Thus, 158 patients were included for further analysis. Eight patients did not receive adjuvant therapy because of a dismal clinical condition. The overall 5-year survival rate for MB and CNS-PNET was 54%, for MB 57%, and for CNS-PNET 41%. Gross total resection (GTR) was achieved in 118 patients and 5-year overall survival for patients with GTR versus those with non-GTR differed significantly with 64% versus 22%. Cytological examination of the cerebrospinal fluid was performed in 52 patients. A total of 126 patients received radiotherapy as part of the primary treatment and 24 did not due to young age. Median time from surgery to start of radiotherapy was 33 days. Duration of radiotherapy was more than 48 days in 22% of patients. At the time of analysis, 63 patients were alive and disease-free, one alive with disease, and 94 patients were deceased; 84 of these due to MB/CNS-PNET and 10 due to supposed late effects from the treatment.


CONCLUSIONS
Survival was comparable to data from other population-based studies. The importance of GTR for survival was corroborated. Reporting real-world data remains crucial to know the true outcome of patients treated outside clinical trials.
a The Faculty of Medicine , Institute of Clinical Medicine, University of Oslo , Oslo , Norway.|b Department of Paediatrics , Oslo University Hospital , Oslo , Norway.
Einar
E
Stensvold
c Department of Pathology , Oslo University Hospital , Oslo , Norway.
Bård Kronen
BK
Krossnes
d Department of Neurosurgery , Oslo University Hospital , Oslo , Norway.
Tryggve
T
Lundar
d Department of Neurosurgery , Oslo University Hospital , Oslo , Norway.
Bernt J
BJ
Due-Tønnessen
a The Faculty of Medicine , Institute of Clinical Medicine, University of Oslo , Oslo , Norway.|d Department of Neurosurgery , Oslo University Hospital , Oslo , Norway.
Radek
R
Frič
e Department of Radiology and Nuclear Medicine , Oslo University Hospital , Oslo , Norway.
Paulina
P
Due-Tønnessen
b Department of Paediatrics , Oslo University Hospital , Oslo , Norway.
Anne Grete
AG
Bechensteen
f Department of Registration , Cancer Registry of Norway , Oslo , Norway.
Tor Åge
TÅ
Myklebust
f Department of Registration , Cancer Registry of Norway , Oslo , Norway.
Tom Børge
TB
Johannesen
g Department of Oncology , Oslo University Hospital , Oslo , Norway.
Petter
P
Brandal
D000293:Adolescent; D000328:Adult; D002528:Cerebellar Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008527:Medulloblastoma / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D018242:Neuroectodermal Tumors, Primitive / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D015996:Survival Rate; D013997:Time Factors; D055815:Young Adult