BACKGROUND: Supratentorial PNET (sPNET) are rare CNS @entity5 of embryonal origin arising in @entity1 and adults. The treatment of sPNET for all age groups at our @entity5 center has been based on the management of @entity2946 ( @entity2946 ), 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 @entity5 in @entity1 and adults from 2001 to 2007. @entity5 pathology, clinical management and survival data were also assessed, particularly as regards those @entity1 who received the Packer chemotherapy regimen for either sPNET or @entity2946 . RESULTS: Eleven @entity1 (five @entity1 and six adults) were identified with non-pineal sPNET, three @entity1 with pineal sPNET, and 19 @entity1 (18 @entity1 and 1 adult) with @entity2946 . There was no difference in overall survival (OS) rates between pediatric and adult sPNET. When all sPNET were compared to all @entity2946 , 5-year OS was 14% versus 73%, respectively, but was only 9% for non-pineal sPNET. When only considering those @entity1 treated with the Packer chemotherapy regimen, the 5-year OS was 12% for sPNET versus 79% for @entity2946 . CONCLUSION: This retrospective study demonstrates that non-pineal sPNET are clinically distinct from @entity2946 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.

Non-pineal supratentorial primitive neuro-ectodermal XXXX (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?

@entity5 :: ('MESH:D009369', 'Disease') :: ['cancer', 'Tumor', 'tumors'] @entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity2946 :: ('MESH:D008527', 'Disease') :: ['medulloblastoma', 'MB']
@entity5 :: (MESH:D009369,Disease) :: ['tumors']
