Effect of surgery and chemotherapy on long-term survival in infants with congenital glioblastoma: an integrated survival analysis

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  • 1 Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota;
  • 2 Department of Neurological Surgery, University of Miami Miller School of Medicine, Nicklaus Children’s Hospital, Miami, Florida;
  • 3 Department of Neurosurgery, University of Oklahoma, Oklahoma City, Oklahoma; and
  • 4 Department of Pediatric Oncology, Mayo Clinic Children’s Center, Rochester, Minnesota
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OBJECTIVE

Glioblastoma (GBM) during infancy is rare, and the clinical outcomes of congenital GBM are not well understood. Correspondingly, the aim of this study was to present a long-term survivor case from the authors’ institution, and establish an integrated cohort of cases across the published literature to better understand the clinical course of this disease in this setting.

METHODS

The authors report the outcomes of an institutional case of congenital GBM diagnosed within the first 3 months of life, and performed a comprehensive literature search for published cases from 2000 onward for an integrated survival analysis. All cases were integrated into 1 cohort, and Kaplan-Meier estimations, Fisher’s exact test, and logistic regression were used to interrogate the data.

RESULTS

The integrated cohort of 40 congenital GBM cases consisted of 23 (58%) females and 17 (42%) males born at a median gestational age of 38 weeks (range 22–40 weeks). Estimates of overall survival (OS) at 1 month was 67%, at 1 year it was 59%, and at 10 years it was 45%, with statistically superior outcomes for subgroups in which patients survived to be treated by resection and chemotherapy. In the overall cohort, multivariable analysis confirmed resection (p < 0.01) and chemotherapy (p < 0.01) as independent predictors of superior OS. Gestational age > 38 weeks (p < 0.01), Apgar scores ≥ 7 at 5 minutes (p < 0.01), absence of prenatal hydrocephalus (p < 0.01), and vaginal delivery (p < 0.01) were associated with greater odds of surgical diagnosis versus autopsy diagnosis.

CONCLUSIONS

Congenital GBM can deviate from the expected poor prognosis of adult GBM in terms of OS. Both resection and chemotherapy confer statistically superior prognostic advantages in those patients who survive within the immediate postnatal period, and should be first-line considerations in the initial management of this rare disease.

ABBREVIATIONS EOR = extent of resection; GBM = glioblastoma; GTR = gross-total resection; IHC = immunohistochemical; OS = overall survival; STR = subtotal resection.

Supplementary Materials

    • Supplemental Table 1 (PDF 224 KB)

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Contributor Notes

Correspondence David J. Daniels: Mayo Clinic, Rochester, MN. daniels.david@mayo.edu.

INCLUDE WHEN CITING Published online August 14, 2020; DOI: 10.3171/2020.5.PEDS20226.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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