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Title: The analysis of approach to and results of treatment in congenitaltumors of central nervous system


Introduction Tumors of the central nervous system are the most commonpediatric solid tumors. They are less common in the first year of lifebut rank second from infantile malignancies. CNS tumors differ inhistological features, location, dynamics of development and clinicalpresentation. In spite of disproportionately large size they arerecognized late which is the result of the capability of infant scull anddeveloping brain. Among clinical symptoms usually dominatenonspecific symptoms of increasing intracranial pressure.Neuroectodermal tumors are the most common in neonatal andinfantile period. Congenital tumors of central nervous system generatedifficulties in therapeutic management and have a poor prognosis.Aim of the studyThe aim of the study is to analyze diagnostic and therapeuticprocedures and outcome in tumors of CNS, to establish factors whichhave an impact on outcome and to attempt to draw up a proceduralalgorithm especially in terms of ethics in cases with fatal prognosis.Material and methodsFor the purpose of the study a group of 42 patients presenting to theDepartment of Neurosurgery at Children’s University Hospital inKrakow during the years 1998-2012 due to congenital CNS tumorsaccording to Ellams’ classification was enrolled. The examined grouprepresented 8,89% of all children operated on due to newly recognizedCNS tumor over that period of time. The retrospective ; review wasperformed in 28 cases based on medical records and history obtainedfrom parents. . There was also analysis of 14 new cases of childrenwho had been admitted to the Department of Neurosurgery due tocongenital CNS tumors. The material consists of sixteen girls (38,1%)and twenty-six boys (61,9%).Each case was analyzed according to symptoms and age of their onsetand diagnosis, duration of symptoms and signs, tumor location,histology of neoplasm, methods of treatment and outcome.Functional outcome was evaluating according to Glasgow OutcomeScale (GOS) and Lansky Performance Scale all together and after 1and 5 years from diagnosis., Results All included patients presented symptoms with onset up to the endof the 1st year of age. Two patients were diagnosed during prenatalperiod. After birth the largest group constitute infants up to the end of6 weeks of age according to Ellams’ classification.Median time of duration of symptoms was 4 weeks. Because ofdifferences in duration of symptoms in some cases diagnosis wasmade after 12 months of age if symptoms appeared up to 12 months oflife. Median age of recognition was 32 weeks.The most common presenting sings in children up to 6 weeks of agewere macrocrania and those of increased intracranial pressure whereasin older children focal symptoms were more common. Nearly 60% ofall tumors were located in supratentorial compartment.Ultra ; sonography was the initial imagine study in 45%.The differences in time of the age of symptoms between high gradeand low grade tumors was statistically significant with prevalence ofhigh grade tumors during the first 6 months of life and low grade inthe period 6–12 months.Total resection was achieved in more than half of patients (51,3%)who underwent surgical intervention. Adjuvant treatment wasperformed in 38,1% of patients. Mortality was 40,5%. Taking intoconsideration differences in tumors location and histopathologic typethere was statistically significant higher mortality and survival in thecases located infratentorially and high grade tumors.The analysis of functional outcome after 1 and 5 years from diagnosisassessed with Lansky Performance Scale showed statisticallysignificant deterioration after 5 years that affected mainly patients whoreceived low score after 1 year, with high grade tumors located inposterior fossa and incompletely resected.ConclusionsCongenital CNS tumors have poor prognosis comparing to thosefind in other children both in respect of mortality and quality of life.The outcome of congenital CNS tumors depends the most on theextend of surgical resection, histopathological type and location of thetumor.The management in cases with fatal prognosis should includepossibility of ending the therapy in every stage of treatment from thetime of prenatal diagnosi ; s until finish therapeutic possibilities.

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2 - studia doktoranckie

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Wydział Lekarski


Kwiatkowski, Stanisław

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tylko w bibliotece

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Mar 17, 2023

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Mar 12, 2014

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ZB-120306 Mar 17, 2023


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