Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?
BACKGROUND
Supratentorial PNET (sPNET) are rare CNS tumors of embryonal origin arising in children and adults. The treatment of sPNET for all age groups at our cancer center has been based on the management of medulloblastoma (MB), involving neurosurgical debulking followed by cranio-spinal irradiation (CSI) and systemic chemotherapy.


METHODS
Medical records were reviewed to gather demographic and clinical data about all embryonal CNS tumors in children and adults from 2001 to 2007. Tumor pathology, clinical management and survival data were also assessed, particularly as regards those patients who received the Packer chemotherapy regimen for either sPNET or MB.


RESULTS
Eleven patients (five children and six adults) were identified with non-pineal sPNET, three children with pineal sPNET, and 19 patients (18 children and 1 adult) with MB. There was no difference in overall survival (OS) rates between pediatric and adult sPNET. When all sPNET were compared to all MB, 5-year OS was 14% versus 73%, respectively, but was only 9% for non-pineal sPNET. When only considering those patients treated with the Packer chemotherapy regimen, the 5-year OS was 12% for sPNET versus 79% for MB.


CONCLUSIONS
This retrospective study demonstrates that non-pineal sPNET are clinically distinct from MB and are resistant to the Packer chemotherapy regimen. We suggest that it is time to reconsider the use of this regimen in teenage and young adult non-pineal sPNET and to investigate the utility of alternative approaches.
Biswas
Swethajit
S
Division of Oncology, Oncology Centre, Addenbrooke's Hospital, Cambridge, United Kingdom.
Burke
Amos
A
Cherian
Sheen
S
Williams
Denise
D
Nicholson
James
J
Horan
Gail
G
Jefferies
Sarah
S
Williams
Michael
M
Earl
Helena M
HM
Burnet
Neil G
NG
Hatcher
Helen
H
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002528:Cerebellar Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D016371:Cranial Irradiation; D005500:Follow-Up Studies; D006801:Humans; D008130:Lomustine / Q000008:administration & dosage; D008527:Medulloblastoma / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D012189:Retrospective Studies; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
D014750:Vincristine; D008130:Lomustine; D002945:Cisplatin