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Predictors of mortality and tumor recurrence in desmoplastic infantile ganglioglioma and astrocytoma—and individual participant data meta-analysis (IPDMA)

  • Clinical Study
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Abstract

Purpose

Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are classified together as grade I neuronal and mixed neuronal-glial tumor of the central nervous system by the World Health Organization (WHO). These tumors are rare and have not been well characterized in terms of clinical outcomes. We aimed to identify clinical predictors of mortality and tumor recurrence/progression by performing an individual patient data meta-analysis (IPDMA) of the literature.

Methods

A systematic literature review from 1970 to 2020 was performed, and individualized clinical data for patients diagnosed with DIA/DIG were extracted. Aggregated data were excluded from collection. Outcome measures of interest were mortality and tumor recurrence/progression, as well as time-to-event (TTE) for each of these. Participants without information on these outcome measures were excluded. Cox regression survival analyses were performed to determine predictors of mortality and tumor recurrence / progression.

Results

We identified 98 articles and extracted individual patient data from 188 patients. The cohort consisted of 58.9% males with a median age of 7 months. The majority (68.1%) were DIGs, while 24.5% were DIAs and 7.5% were non-specific desmoplastic infantile tumors; DIAs presented more commonly in deep locations (p = 0.001), with leptomeningeal metastasis (p = 0.001), and was associated with decreased probability of gross total resection (GTR; p = 0.001). Gender, age, and tumor pathology were not statistically significant predictors of either mortality or tumor recurrence/progression. On multivariate survival analysis, GTR was a predictor of survival (HR = 0.058; p = 0.007) while leptomeningeal metastasis at presentation was a predictor of mortality (HR = 3.27; p = 0.025). Deep tumor location (HR = 2.93; p = 0.001) and chemotherapy administration (HR = 2.02; p = 0.017) were associated with tumor recurrence/progression.

Conclusion

Our IPDMA of DIA/DIG cases reported in the literature revealed that GTR was a predictor of survival while leptomeningeal metastasis at presentation was associated with mortality. Deep tumor location and chemotherapy were associated with tumor recurrence / progression.

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Data availability

The authors will provide data upon request.

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Funding

This study received no funding. MZS is supported by an NIH training grant: R25NS079198.

Author information

Authors and Affiliations

  1. Division of Neurosurgery, Brain Institute, Nicklaus Children’s Hospital, Miami, FL, USA

    Shelly Wang

  2. Department of Neurosurgery, University of Miami, Miami, FL, USA

    Shelly Wang

  3. Department of Neurosurgery, University of California Los Angeles, Los Angeles, CA, USA

    Matthew Z. Sun, Aria Fallah & Anthony C. Wang

  4. Department of Neurosurgery, University of Louisville, Louisville, KY, USA

    I. Joshua Abecassis

  5. Department of Surgery, Université de Montréal, Montreal, QC, Canada

    Alexander G. Weil

  6. Division of Pediatric Neurosurgery, Sick Kids Toronto, University of Toronto, Toronto, ON, Canada

    George M. Ibrahim

  7. Department of Neurological Surgery, University of Washington and Seattle Children’s Hospital, Seattle, WA, USA

    Chibawanye Ene, Richard G. Ellenbogen & Jeffrey G. Ojemann

  8. Division of Hematology Oncology, Department of Pediatrics, University of Washington and Seattle Children’s Hospital, Seattle, WA, USA

    Sarah E. S. Leary, James M. Olson & J. Russell Geyer

  9. Department of Anatomic Pathology, Seattle Children’s Hospital, University of Washington and Laboratories, Seattle, WA, USA

    Bonnie L. Cole

  10. Department of Laboratory Medicine, University of Washington and Seattle Children’s Hospital, Seattle, WA, USA

    Christina M. Lockwood

Authors
  1. Shelly Wang
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  2. Matthew Z. Sun
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  3. I. Joshua Abecassis
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  4. Alexander G. Weil
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  11. James M. Olson
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  15. Anthony C. Wang
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Contributions

SW, AF and ACW designed the systematic review; SW, MZS, and ACW performed the literature review and created the IPDMA; SW, AGW, GMI and ACW designed and performed the analysis the data; SW, MZS, and ACW drafted and revised the manuscript; IJA, AF, CE, SESL, BLC, CML, JMO, JRG, RGE, JGO, and ACW critically reviewed the literature, supervised the scientific analysis, and evaluated the significance of the findings.

Corresponding author

Correspondence to Anthony C. Wang.

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The authors report no conflicts of interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Informed consent was not needed due to study design requiring no collection of data other than from the published literature.

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Wang, S., Sun, M.Z., Abecassis, I.J. et al. Predictors of mortality and tumor recurrence in desmoplastic infantile ganglioglioma and astrocytoma—and individual participant data meta-analysis (IPDMA). J Neurooncol 155, 155–163 (2021). https://doi.org/10.1007/s11060-021-03860-1

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  • DOI: https://doi.org/10.1007/s11060-021-03860-1

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