Authors: Reineke A Schoot Gitta Bleeker Huib N Caron Berthe L van Eck Hugo A Heij Jan de Kraker Godelieve A Tytgat
Publish Date: 2013/06/06
Volume: 40, Issue: 10, Pages: 1516-1522
Abstract
In patients with localised neuroblastoma without adverse genetic aberrations observational treatment is justified Therapy is required when organ or respiratory functions have become compromised As the outcome is good side effects of treatment should be prevented The aim of this retrospective study was to evaluate response and outcome in patients treated with 131Imetaiodobenzylguanidine MIBG for unresectable localised neuroblastoma with compromised organ functionsPatients with localised neuroblastoma median age 16 years 0–55 years diagnosed between 1989 and 2008 were included in this retrospective study n = 21 Primary tumours were unresectable and there was a compromised organ or respiratory function Diagnosis and staging were performed according to the International Neuroblastoma Staging System Fixed doses of 131IMIBG therapy 50–200 mCi were given The median number of infusions was two range one to seven Response was graded according to the International Neuroblastoma Response CriteriaOf the 21 patients 14 did not need any chemotherapy Patients were treated with 131IMIBG therapy and in most cases with additional surgery and/or chemotherapy Sixteen achieved complete response CR three very good partial response VGPR one partial response PR and one progressive disease PD Two patients died of PD after having achieved CR initially and due to surgical complications a few months after resection Tenyear overall survival and eventfree survival were 905 The median followup was 85 years range 04–196 yearsNeuroblastoma is the most common extracranial solid tumour of childhood Arising from the neural crest primary tumours can be localised in the adrenal glands or anywhere along the sympathetic side chain Important prognostic factors are age stage MYCN amplification MNA loss of heterozygosity of chromosome 1p 1pLOH chromosomal 11q aberration and DNA ploidy 1 Approximately 25 of patients present with localised disease without distant metastasesTreatment modality and intensity of treatment in patients with localised disease stage 1–3 International Neuroblastoma Staging System INSS are based upon risk stratification and defined by age stage and biological features The prognosis is good and the majority of patients can be cured by surgery alone or by a combination of surgery and chemotherapy 2
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