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. © 2013 Wiley Periodicals, Inc.

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; Antonelli, Manila; Giangaspero, Felice. - In: PEDIATRIC BLOOD & CANCER. - ISSN 1545-5009. - ELETTRONICO. - 60:12(2013), pp. 2031-2035. [10.1002/pbc.24540]

Evolving of therapeutic strategies for CNS-PNET

ANTONELLI, MANILA;GIANGASPERO, FELICE
2013

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. © 2013 Wiley Periodicals, Inc.
2013
childhood brain tumors; treatment stratification; cns-pnet; treatment toxicity; dissemination; local relapse
01 Pubblicazione su rivista::01a Articolo in rivista
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; Antonelli, Manila; Giangaspero, Felice. - In: PEDIATRIC BLOOD & CANCER. - ISSN 1545-5009. - ELETTRONICO. - 60:12(2013), pp. 2031-2035. [10.1002/pbc.24540]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/650094
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