title: Single agent vinorelbine in pediatric patients with progressive optic pathway glioma.
abstract: The management of progressive unresectable low-grade glioma remains controversial. Treatment options have included radiotherapy, and more recently chemotherapy, usually following an initial period of observation. Within this context, we evaluated vinorelbine, a semi-synthetic vinca alkaloid that has shown evidence of activity against glioma. From July 2007 an institutional protocol with vinorelbine (30 mg/m(2) days 0, 8, 22) for a total of 18 cycles, has been conducted at IOP/GRAACC/UNIFESP for children with optic pathway glioma (OPG). The main objectives were clinical and radiological response, as well as toxicity profile. Twenty-three patients with progressive OPG with a mean age of 69 months (4-179) were enrolled. Three patients had a diagnosis of neurofibromatosis type 1. Twenty-two patients were assessable for response with an overall objective response rate of 63 %, with eight patients showing stable disease. The most important toxicity was hematologic (grade III/IV neutropenia) observed in four patients. Gastrointestinal toxicity (grade I/II vomiting) was observed in seven patients and only 1 patient showed grade I peripheral neuropathy. The median progression-free survival (PFS) was 33 months (6.9-69) with a 3 and 5 year PFS of 64 ± 19 and 37 ± 20 %, respectively, for an overall 3 and 5 year-survival of 95 ± 10 %. This study suggests that vinorelbine may be an interesting option for pediatric low-grade gliomas, showing low toxicity profile and providing a good quality of life for patients with such chronic disease.
authors.affiliation: Pediatric Oncology, IOP-GRAACC/Federal University of São Paulo, Rua Botucatu no 743 - Vila Clementino, São Paulo, 04023-062, Brazil, deacappellano@uol.com.br.
authors.forename: Andrea Maria
authors.initials: AM
authors.lastname: Cappellano
authors.forename: Antonio Sergio
authors.initials: AS
authors.lastname: Petrilli
authors.forename: Nasjla Saba
authors.initials: NS
authors.lastname: da Silva
authors.forename: Frederico Adolfo
authors.initials: FA
authors.lastname: Silva
authors.forename: Priscila Mendes
authors.initials: PM
authors.lastname: Paiva
authors.forename: Sergio
authors.initials: S
authors.lastname: Cavalheiro
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
mesh_terms: D000293:Adolescent; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use* / Q000633:toxicity; D001921:Brain / Q000187:drug effects / Q000473:pathology / Q000528:radiation effects; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D020339:Optic Nerve Glioma / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology / Q000532:radiotherapy; D016019:Survival Analysis; D016896:Treatment Outcome; D014747:Vinblastine / Q000031:analogs & derivatives* / Q000627:therapeutic use / Q000633:toxicity; D000077235:Vinorelbine
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D014747:Vinblastine; D000077235:Vinorelbine