Does diagnostic delay result in decreased survival in paediatric brain tumours?

Kukal, Karel; Dobrovoljac, Milana; Boltshauser, Eugen; Ammann, Roland A.; Grotzer, Michael A.

doi:10.1007/s00431-008-0755-5

Cited by 71 publications

(84 citation statements)

References 24 publications

(34 reference statements)

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“…Finally, we found the same demographic and clinical characteristics as in other series of medulloblastomas in children [2,17,33]. Other studies have reported a median prediagnostic interval of 5 (Switzerland) [25], 8 (North America) [17,30], and 22 weeks (India) [27]. The median interval observed in our study (9 weeks) is close to that observed in North America.…”

Section: Discussionsupporting

confidence: 92%

“…Existing studies of the time to diagnosis of brain tumours in children and their determinants are of limited value for understanding the specific issues of medulloblastomas, for they group together several types of tumours, different in histology and site [3,8,11,16,25,30,35] and include a fairly small number of medulloblastoma [8,11,16,25,30,35], or are based on recruitment from a single hospital and thus subject to substantial selection bias [8,11,16,17,25,35]. Because the exact causes of diagnosis delays in medulloblastoma in children remain poorly understood, it is difficult to propose guidelines to reduce them [41].…”

Section: Introductionmentioning

confidence: 99%

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Interval between onset of symptoms and diagnosis of medulloblastoma in children: distribution and determinants in a population-based study

et al. 2011

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Hospital-based studies have reported long delays in the diagnosis of paediatric brain tumours. Our objective was to describe the duration between onset of symptoms and diagnosis of medulloblastoma in children and study their clinical determinants in a population-based study. This retrospective cohort study included all paediatric medulloblastoma from a region of France from 1990 to 2005. The median interval from symptom onset until diagnosis for these 166 patients was 65 days and did not decrease during the study period. The most frequent manifestations were: vomiting (88%), headaches (79%), psychomotor regression (60% of children under 3 years), psychological symptoms (27%), strabismus (26%), and asthenia (25%). For one third of the children under 3 years, the diagnosis was made only after life-threatening signs of intracranial hypertension appeared. The prediagnosis interval was significantly longer (median 91 vs. 60 days, p = 0.001) in children with psychological symptoms (27%). Causes for intervals that exceeded the median (65 days) included inconsistent (25%) or late (36%) combination of headaches and vomiting, a period of spontaneous symptom remission (14%-20%), no (24%) or late (57%) neurological signs, psychological symptoms (35%), and a normal neurological examination (27%). Time to medulloblastoma diagnosis in children remains fairly long, despite advances in imaging. Primary-care physicians must be suspicious not only of suggestive neurological signs, but also of non-specific symptoms that persist or are multiple. A meticulous neurological examination and cerebral imaging for such patients might facilitate earlier diagnosis.

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Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Interval between onset of symptoms and diagnosis of medulloblastoma in children: distribution and determinants in a population-based study

et al. 2011

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“…3 This can lead to prediagnostic symptomatic intervals (PSI) ('diagnostic time lags') in the range of several weeks to months. [4][5][6][7][8][9][10][11] The assumption that a longer PSI may result in a more advanced disease stage and/or have an adverse impact on tumor control, survival and/or neurological/neuropsychological/quality of survival outcome may lead to selfreproaches from parents and physicians and can result in accusations of medical malpractice. [12][13][14] However, available information on the correctness of this assumption is insufficient, as studies on PSI in children with brain tumors suffer from low patient numbers, retrospective design, heterogeneity of patient, disease, and treatment characteristics, and lacking outcome data.…”

Section: -2mentioning

confidence: 99%

“…[12][13][14] However, available information on the correctness of this assumption is insufficient, as studies on PSI in children with brain tumors suffer from low patient numbers, retrospective design, heterogeneity of patient, disease, and treatment characteristics, and lacking outcome data. [4][5][6][7][8][9][10][11][15][16][17][18][19][20][21][22][23][24] To study the association of the PSI with disease stage at diagnosis as well as tumor control and survival outcome, we analyzed prospectively collected data on 224 homogeneously treated patients. 3.…”

Section: -2mentioning

confidence: 99%

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

Gerber

Hoff

Bueren

et al. 2012

European Journal of Cancer

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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. 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 pediatric patients with medulloblastoma.3

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“…Accordingly, only the length of the prediagnostic symptomatic interval (PSI) can provide some indirect evidence regarding the kinetics of tumour growth: Not only are differences documented between different types of brain tumours, with malignant tumours (e.g. MBs) having shorter PSIs than low-grade tumours [4,10,12,17,19], but considerable variability is seen within the patient group with MB [2,8]: In a large prospective series, we found PSIs ranging from 0 to 24 months, with a median of 2 months. Interestingly, the group of patients with the longest PSIs had lower stage disease at diagnosis and a better overall survival probability than that with shorter PSIs [6].…”

Section: Discussionmentioning

confidence: 99%

Natural history of a medulloblastoma: 30 months of wait and see in a child with a cerebellar incidentaloma

et al. 2013

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INTRODUCTION:With the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking, and little is known about their optimal management. CASE REPORT: We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 14 months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. DISCUSSION: To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic changes. DOI: https://doi.org/10.1007/s00381-013-2077-9Posted at the Zurich Open Repository and Archive, University of Zurich ZORA URL: https://doi.org/10.5167/uzh-81611 Accepted Version Originally published at: Zeilhofer, Ulrike B; Scheer, Ianina; Warmuth-Metz, Monika; Rushing, Elisabeth J; Pietsch, Torsten; Boltshauser, Eugen; Grotzer, Michael A; Gerber, Nicolas U (2013). Natural history of a medulloblastoma: 30 months of wait and see in a child with a cerebellar incidentaloma. Child's Nervous System, 29 (7) ABSTRACTWith the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking and little is known about their optimal management. We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 4 months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic changes.

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Does diagnostic delay result in decreased survival in paediatric brain tumours?

Cited by 71 publications

References 24 publications

Interval between onset of symptoms and diagnosis of medulloblastoma in children: distribution and determinants in a population-based study

Interval between onset of symptoms and diagnosis of medulloblastoma in children: distribution and determinants in a population-based study

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

Natural history of a medulloblastoma: 30 months of wait and see in a child with a cerebellar incidentaloma

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