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Research ArticlePediatrics

A Diagnostic Algorithm for Posterior Fossa Tumors in Children: A Validation Study

C.A.P.F. Alves, U. Löbel, J.S. Martin-Saavedra, S. Toescu, M.H. Tsunemi, S.R. Teixeira, K. Mankad, D. Hargrave, T.S. Jacques, C. da Costa Leite, F.G. Gonçalves, A. Vossough and F. D'Arco
American Journal of Neuroradiology May 2021, 42 (5) 961-968; DOI: https://doi.org/10.3174/ajnr.A7057
C.A.P.F. Alves
aFrom the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
gDepartment of Radiology (C.d.C.L.), Hospital das Clinicas, Faculdade de Medicina de Sao Paulo, Sao Paulo
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  • ORCID record for C.A.P.F. Alves
U. Löbel
bRadiology Department (U.L., K.M., F.D.), UCL Great Ormond Street Hospital for Children, London, UK
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J.S. Martin-Saavedra
aFrom the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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S. Toescu
cDepartment of Neurosurgery (S.T.), UCL Great Ormond Street Hospital for Children, London, UK
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M.H. Tsunemi
dDepartment of Biostatistics (M.H.T.), Instituto de Biociências, São Paulo State University, São Paul, Brazil
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S.R. Teixeira
aFrom the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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K. Mankad
bRadiology Department (U.L., K.M., F.D.), UCL Great Ormond Street Hospital for Children, London, UK
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D. Hargrave
ePediatric Oncology Unit (D.H.), UCL Great Ormond Street Institute of Child Health, London, UK
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T.S. Jacques
fDevelopmental Biology and Cancer Programme (T.S.J.), UCL Great Ormond Street Institute of Child Health, University College London, London, UK
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C. da Costa Leite
gDepartment of Radiology (C.d.C.L.), Hospital das Clinicas, Faculdade de Medicina de Sao Paulo, Sao Paulo
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F.G. Gonçalves
aFrom the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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A. Vossough
aFrom the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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F. D'Arco
bRadiology Department (U.L., K.M., F.D.), UCL Great Ormond Street Hospital for Children, London, UK
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  • FIG 1.
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    FIG 1.

    Predesigned radiologic flow chart created according to the literature before diagnostic accuracy analysis. The asterisk indicates brain stem tumors excluded from the analysis. Double asterisks indicate relative to gray matter. Modified with permission from D'Arco et al.11

  • FIG 2.
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    FIG 2.

    Diagnostic accuracy of a predesigned radiologic flow chart to identify different types of cerebellar tumors.

  • FIG 3.
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    FIG 3.

    Diagnostic accuracy of sequence 312 (all types of medulloblastomas) of the predesigned radiologic flow chart to identify different types of medulloblastomas.

  • FIG 4.
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    FIG 4.

    Modified radiologic flow chart (flow chart 2) after diagnostic accuracy analysis. The asterisk indicates brain stem tumors excluded from the analysis. Double asterisks indicate relative to gray matter. Triple asterisks indicates low PPV and sensitivity for any particular molecular/histological group of tumor.

  • FIG 5.
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    FIG 5.

    Differential diagnoses in cases of posterior fossa tumors originating from the cerebellar hemisphere. Axial T2WI (A) and axial ADC map (B) show SHH medulloblastoma (flow chart 2, number 111) in a typical peripheral location within the cerebellar hemisphere due to its origin from ganglionic cell precursors. Note very low ADC values (ie, diffusion restriction). Axial T2WI (C) and axial ADC map (D) show the typical appearance of a pilocytic astrocytoma (flow chart 2, number 123) originating from the cerebellar hemisphere. Note the typical nodule and appearance of cysts and much higher ADC values in comparison with the medulloblastoma.

  • FIG 6.
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    FIG 6.

    Differential diagnoses in posterior fossa tumors involving the foramen of Luschka and cerebellopontine angle. Axial T2WI (A) and ADC map (B) in a child with ependymoma (flow chart 2, number 423). Note the presence of internal vessels (arrow) and intermediate ADC values. Axial T2 (C) and ADC maps (D) in a 2-year-old boy with a AT/RT (new flow chart number 411). Note very low values of ADC, suggesting an embryonal tumor and peripheral cysts.

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    Table 1:

    Statistical analysis of the radiologic flow chart to discriminate different types of cerebellar tumors

    Diagnosis EquivalentFlowchart SequenceSensitivity (%)Specificity (%)PPV (%)NPV (%)Accuracy (%)
    Ependymoma323/42389 (67–99)95 (89–98)71 (49–87)98 (94–100)94 (89–97)
    Pilocytic astrocytoma12388 (76–95)96 (89–99)92 (82–98)93 (85–97)93 (87–96)
    AT/RT41150 (21–79)99 (96–100)86 (42–100)96 (91–98)99 (96–100)
    AT/RT411/31175 (43–95)98 (94–100)75 (43–95)98 (94–100)91 (96–98)
    Medulloblastoma SHH111/112/31371 (42–92)93 (88–97)53 (29–76)97 (92–99)72 (64–79)
    Medulloblastoma WNT41214 (0–58)97 (93–99)20 (1–72)96 (91–98)88 (93–97)
    • View popup
    Table 2:

    Likelihood ratio analysis of the radiologic flow chart to discriminate different types of cerebellar tumors

    DiagnosisFlow Chart SequenceLR+ (95% CI)LR– (95% CI)
    AT/RT411/31134 (10.6–109)a0.26 (0.1–0.7)
    Ependymoma323/42316.5 (7.9–35)0.11 (0.03–0.4)a
    Medulloblastoma SHH111/112/31310.6 (5.2–21.7)0.3 (0.1–0.7)
    Medulloblastoma group 3 or 431210.07 (5.3–19.3)0.23 (0.12–0.45)a
    Pilocytic astrocytoma12320 (7.7–52.8)0.13 (0.13–0.26)a
    • ↵a Clinically applicable confidence intervals.

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American Journal of Neuroradiology: 42 (5)
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C.A.P.F. Alves, U. Löbel, J.S. Martin-Saavedra, S. Toescu, M.H. Tsunemi, S.R. Teixeira, K. Mankad, D. Hargrave, T.S. Jacques, C. da Costa Leite, F.G. Gonçalves, A. Vossough, F. D'Arco
A Diagnostic Algorithm for Posterior Fossa Tumors in Children: A Validation Study
American Journal of Neuroradiology May 2021, 42 (5) 961-968; DOI: 10.3174/ajnr.A7057

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A Diagnostic Algorithm for Posterior Fossa Tumors in Children: A Validation Study
C.A.P.F. Alves, U. Löbel, J.S. Martin-Saavedra, S. Toescu, M.H. Tsunemi, S.R. Teixeira, K. Mankad, D. Hargrave, T.S. Jacques, C. da Costa Leite, F.G. Gonçalves, A. Vossough, F. D'Arco
American Journal of Neuroradiology May 2021, 42 (5) 961-968; DOI: 10.3174/ajnr.A7057
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