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A long duration of the prediagnostic symptomatic interval is not associated with an unfavourable prognosis in childhood medulloblastoma


Gerber, Nicolas U; von Hoff, Katja; von Bueren, André O; Treulieb, Wiebke; Deinlein, Frank; Benesch, Martin; Zwiener, Isabella; Soerensen, Niels; Warmuth-Metz, Monika; Pietsch, Torsten; Mittler, Uwe; Kuehl, Joachim; Kortmann, Rolf-Dieter; Grotzer, Michael A; Rutkowski, Stefan (2011). A long duration of the prediagnostic symptomatic interval is not associated with an unfavourable prognosis in childhood medulloblastoma. European Journal of Cancer, 48(13):2028-2036.

Abstract

BACKGROUND: Due to the lacking specificity of symptoms making a correct diagnosis can be a challenge in children with medulloblastoma. This can lead to prediagnostic symptomatic intervals (PSIs) of several weeks to months. It is unknown whether the length of the PSI is associated with an inferior survival outcome in this population. METHODS: To study the association of PSI with disease stage at diagnosis, tumour control and survival in children with medulloblastoma, prospectively collected data on PSI, clinical, and biological features were analysed in 224 patients diagnosed at the age of 3-18years and treated within the prospective randomised multicentre trial HIT'91. RESULTS: Patients with lower-stage disease tended towards a longer median PSI than those with higher-stage disease (M0 stage, 2.0months; M1 stage, 2.0months; M2/M3 stage, 1month; p=0.094. M0/1 stage versus M2/3 stage; p=0.025). The patient group with the longest PSI had the best survival outcome (PSI ⩾4.0months: 10-year overall survival rate (OS), 71%; PSI <4.0months, 10-year OS, 61%; p=0.056). Age at diagnosis was positively correlated with PSI (p=0.027). No associations were found between PSI and sex histological subtype, presence of postoperative residual tumour, or c-myc and TrkC mRNA expression. CONCLUSION: Contrary to a common belief that a longer PSI may adversely affect prognosis, a longer PSI was associated with a trend towards lower metastatic stage and better survival probabilities. Nevertheless these findings do not obviate the importance of a timely diagnosis in paediatric patients with medulloblastoma.

BACKGROUND: Due to the lacking specificity of symptoms making a correct diagnosis can be a challenge in children with medulloblastoma. This can lead to prediagnostic symptomatic intervals (PSIs) of several weeks to months. It is unknown whether the length of the PSI is associated with an inferior survival outcome in this population. METHODS: To study the association of PSI with disease stage at diagnosis, tumour control and survival in children with medulloblastoma, prospectively collected data on PSI, clinical, and biological features were analysed in 224 patients diagnosed at the age of 3-18years and treated within the prospective randomised multicentre trial HIT'91. RESULTS: Patients with lower-stage disease tended towards a longer median PSI than those with higher-stage disease (M0 stage, 2.0months; M1 stage, 2.0months; M2/M3 stage, 1month; p=0.094. M0/1 stage versus M2/3 stage; p=0.025). The patient group with the longest PSI had the best survival outcome (PSI ⩾4.0months: 10-year overall survival rate (OS), 71%; PSI <4.0months, 10-year OS, 61%; p=0.056). Age at diagnosis was positively correlated with PSI (p=0.027). No associations were found between PSI and sex histological subtype, presence of postoperative residual tumour, or c-myc and TrkC mRNA expression. CONCLUSION: Contrary to a common belief that a longer PSI may adversely affect prognosis, a longer PSI was associated with a trend towards lower metastatic stage and better survival probabilities. Nevertheless these findings do not obviate the importance of a timely diagnosis in paediatric patients with medulloblastoma.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:6 December 2011
Deposited On:15 Mar 2013 07:32
Last Modified:05 Apr 2016 15:22
Publisher:Elsevier
ISSN:0959-8049
Publisher DOI:https://doi.org/10.1016/j.ejca.2011.11.012
PubMed ID:22153558
Permanent URL: https://doi.org/10.5167/uzh-55051

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