Long Time to Diagnosis of Medulloblastoma in Children Is Not Associated with Decreased Survival or with Worse Neurological Outcome

International audienceBACKGROUND: The long time to diagnosis of medulloblastoma, one of the most frequent brain tumors in children, is the source of painful remorse and sometimes lawsuits. We analyzed its consequences for tumor stage, survival, and sequelae. PATIENTS AND METHODS: This retrospective population-based cohort study included all cases of pediatric medulloblastoma from a region of France between 1990 and 2005. We collected the demographic, clinical, and tumor data and analyzed the relations between the interval from symptom onset until diagnosis, initial disease stage, survival, and neuropsychological and neurological outcome. RESULTS: The median interval from symptom onset until diagnosis for the 166 cases was 65 days (interquartile range 31-121, range 3-457). A long interval (defined as longer than the median) was associated with a lower frequency of metastasis in the univariate and multivariate analyses and with a larger tumor volume, desmoplastic histology, and longer survival in the univariate analysis, but not after adjustment for confounding factors. The time to diagnosis was significantly associated with IQ score among survivors. No significant relation was found between the time to diagnosis and neurological disability. In the 62 patients with metastases, a long prediagnosis interval was associated with a higher T stage, infiltration of the fourth ventricle floor, and incomplete surgical resection; it nonetheless did not influence survival significantly in this subgroup. CONCLUSIONS: We found complex and often inverse relations between time to diagnosis of medulloblastoma in children and initial severity factors, survival, and neuropsychological and neurological outcome. This interval appears due more to the nature of the tumor and its progression than to parental or medical factors. These conclusions should be taken into account in the information provided to parents and in expert assessments produced for malpractice claims

Clinical and biological specificity of beta-thalassemia intermedia: a case report

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IQ score according to time to diagnosis (p<0.05, see text).

<p>IQ score according to time to diagnosis (p<0.05, see text).</p

Relation between time to diagnosis (TtD) and local extension.

*<p>Degree of significance of nonparametric test (Mann-Whitney or Kruskal-Wallis) of the distribution of time to diagnosis.</p>‡<p>Local extension stages according to Chang-Harisiadis classification <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0033415#pone.0033415-Chang1" target="_blank">[33]</a>, <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0033415#pone.0033415-Harisiadis1" target="_blank">[34]</a>.</p

Relation between time to diagnosis (TtD) and clinical and tumor characteristics.

§<p>Degree of significance of nonparametric test (Mann Whitney or Kruskal-Wallis) of the distribution of time to diagnosis.</p>¤<p>Odd Ratio [95% confidence interval].</p>±<p>Degree of significance of the chi-2 test or Fisher's exact test.</p>¤¤<p>adjusted Odds Ratio [95% confidence interval].</p>‡<p>Degree of significance of the coefficient of the logistic regression test.</p>*<p>One patient died within 24 h of surgery, before spinal MRI, and was excluded from the analysis.</p>**<p>The data concerning psychological signs have been previously published <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0033415#pone.0033415-Brasme1" target="_blank">[25]</a>.</p

Distribution of time to diagnosis according to the presence of metastatic disease (<i>p</i><10⁻⁴).

<p>Distribution of time to diagnosis according to the presence of metastatic disease (<i>p</i><10⁻⁴).</p

Survival according to age, tumor characteristics, and time to diagnosis (TtD).

*<p>Degree of significance of the Logrank test.</p>‡<p>Cox model with adjustment for the following covariables: TtD less than or more than the median of 65 days, age older or younger than 5 years, desmoplastic or not histology, metastatic or localized tumor.</p>§<p>One patient died within 24 h of surgery, before spinal MRI, and was thus excluded from the analysis.</p