Evolving of therapeutic strategies for CNS-PNET

Maura Massimino, Lorenza Gandola, Veronica Biassoni, Filippo Spreafico, Elisabetta Schiavello, Geraldina Poggi, Emilia Pecori, Marco Vajna De Pava, Piergiorgio Modena, Manila Antonelli, Felice Giangaspero

Research output: Contribution to journalArticlepeer-review

Abstract

Background: A protocol for the intensive treatment of non-cerebellar PNET (CNS-PNET) combining chemotherapy and radiotherapy was launched in 2000. Efforts were subsequently made to improve the prognosis and to de-escalate the treatment for selected patient groups. Procedure: Twenty-eight consecutive patients were enrolled for a high-dose drug schedule (methotrexate, etoposide, cyclophosphamide, and carboplatin±vincristine), followed by hyperfractionated accelerated CSI (HART-CSI) at total doses of 31-39Gy, depending on the patient's age, with two high-dose thiotepa courses following CSI. After the first 15 patients had been treated, craniospinal irradiation (CSI) was replaced with focal radiotherapy (RT) for selected cases (non-metastatic and not progressing during induction chemotherapy). Eight of the 28 children received the same chemotherapy but conventionally fractionated focal RT at 54Gy. Results: The 5-year progression-free survival (PFS), event-free survival (EFS), and overall survival (OS) rates were 62%, 53%, and 52%, respectively, for the whole series, and 70%, 70%, and 87% for the eight focally irradiated children. Residual disease and metastases were not prognostically significant. In children with residual disease, response to RT was significant (5-year PFS 59% vs. 20%, P=0.01), while the total dose of CSI was not. There were three treatment-related toxic events. Relapses were local in seven cases (including two of the eight focally irradiated patients), and both local and disseminated in 2. Conclusions: This intensive schedule enabled treatment stratification for the purposes of radiation, thereby sparing some children full-dose CSI. Local control is the main goal of treatment for CNS-PNET. Pediatr Blood Cancer 2013;60:2031-2035.

Original languageEnglish
Pages (from-to)2031-2035
Number of pages5
JournalPediatric Blood and Cancer
Volume60
Issue number12
DOIs
Publication statusPublished - Dec 2013

Keywords

  • Childhood brain tumors
  • CNS-PNET
  • Dissemination
  • Local relapse
  • Treatment stratification
  • Treatment toxicity

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology

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