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 31-39 Gy, 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 54 Gy. 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.
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 31-39 Gy, 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 54 Gy. 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.
Authors: Jordan R Hansford; Jie Huang; Raelene Endersby; Andrew J Dodgshun; Bryan K Li; Eugene Hwang; Sarah Leary; Amar Gajjar; Katja Von Hoff; Olivia Wells; Alison Wray; Rishi S Kotecha; David R Raleigh; Schuyler Stoller; Sabine Mueller; Steven E Schild; Pratiti Bandopadhayay; Maryam Fouladi; Eric Bouffet; Annie Huang; Arzu Onar-Thomas; Nicholas G Gottardo Journal: Neurooncol Adv Date: 2022-04-14
Authors: Teresa de Rojas; Francisco Bautista; Miguel Flores; Lucía Igual; Raquel Rubio; Eduardo Bardón; Lucía Navarro; Laura Murillo; Raquel Hladun; Adela Cañete; Miguel Garcia-Ariza; Carmen Garrido; Ana Fernández-Teijeiro; Eduardo Quiroga; Carlota Calvo; Anna Llort; Inmaculada de Prada; Luis Madero; Ofelia Cruz; Lucas Moreno Journal: J Neurooncol Date: 2017-12-16 Impact factor: 4.130
Authors: Annalisa Passariello; Maria Elena Errico; Vittoria Donofrio; Manuela Maestrini; Alia Zerbato; Laura Cerchia; Maria Capasso; Mario Capasso; Monica Fedele Journal: Cancers (Basel) Date: 2019-10-11 Impact factor: 6.639