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

Abstract

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.

Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India

“…However, in another series of 33 patients with embryonal CNS tumor from [4]. The authors opined that nonpineal SPNET, especially in teenagers and young adults, is clinically distinct from MB and resistant to Packer's regimen and suggested the use of alternative chemotherapy (ifosfamide/temozolomide based) regimens [4]. Extrapolating from the data on the use of an alternating VAC and IE regimen in peripheral PNET and Ewing's sarcoma of bone (CCG-7881 and POG-8850 protocol), we follow alternating VAC/IEbased adjuvant chemotherapy for a total of 12 cycles after the completion of radiation therapy, and all but two patients received the aforementioned regimen in the current series [15].…”

“…In another study of 22 patients, Reddy et al used eight cycles of a 6-weekly regimen of cisplatin, vincristine and lomustine after maximal safe resection and CSI with satisfactory results (5-year OS, 53 %) [36]. However, in another series of 33 patients with embryonal CNS tumor from [4]. The authors opined that nonpineal SPNET, especially in teenagers and young adults, is clinically distinct from MB and resistant to Packer's regimen and suggested the use of alternative chemotherapy (ifosfamide/temozolomide based) regimens [4].…”

“…Although histologically indistinguishable from other small round cell tumors of the brain, SPNET is characterized by its distinct aggressive clinical behavior and poorer outcome [19,33,37]. There is mounting evidence that SPNET and medulloblastoma (MB) have different molecular alterations and different responses to treatment [4,5,19,38]. Gain of chromosome 17q is more common in MB, whereas loss of chromosome 14q is more common in SPNET [38].…”

“…The diagnostic workup and treatment policy for infra-and supratentorial PNET are not clearly distinguishable. However, the prognosis in patients with SPNET is worse in comparison to those with posterior fossa PNET [4,30]. The current study was conducted at our institute to evaluate the clinical features, treatment protocol, patterns of failure and survival outcome in 15 consecutive patients with nonpineal SPNET.…”

Is there a role for high dose chemotherapy with hematopoietic stem cell rescue in patients with relapsed supratentorial PNET?

“…They occur mainly in the first decade of life, but they can also affect young adults [2]. Even though sPNETs have been grouped with medulloblastomas in many studies [3][4][5], their outcome is worse [2] and molecular studies have shown that sPNETs are distinct from medulloblastomas [6,7].…”