Pontine tumor in a neonate: case report and analysis of the current literature

Restricted access

Tumors of the central nervous system represent the largest group of solid tumors found in pediatric patients. Pilocytic astrocytoma is the most common pediatric glioma, mostly located in the posterior fossa. The majority of brainstem tumors, however, are classified as highly aggressive diffuse intrinsic pontine gliomas (DIPGs) and their prognosis is dismal.

The authors report on the case of a neonate in whom MRI and neuropathological assessment were used to diagnose DIPG. Before initiation of the planned chemotherapy, the tumor regressed spontaneously, and the newborn exhibited a normal neurological development. Meanwhile, Illumina Human Methylation450 BeadChip analysis reclassified the tumor as pilocytic astrocytoma of the posterior fossa.

In conclusion, the authors advocate not initiating immediate intensive therapy in newborns with brain tumors, even with classical appearance of a DIPG; rather, they would like to encourage a biopsy to define the best individual therapeutic approach and avoid ineffective chemotherapy.

ABBREVIATIONS DIPG = diffuse intrinsic pontine glioma; PA = pilocytic astrocytoma.

Article Information

Correspondence Konrad Bochennek: Goethe University, Frankfurt, Germany. konrad.bochennek@kgu.de.

INCLUDE WHEN CITING Published online February 15, 2019; DOI: 10.3171/2018.10.PEDS18215.

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

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Tumor regression—serial MRI sagittal images. A: Newborn at 2 days. T2-weighted sagittal image. The tumor (white vertical arrow) is hyperintense on T2w and involves most part of the pons with encasement of the basilar artery (white horizontal arrow). B: Newborn at 2 days. T1-weighted postcontrast sagittal image shows no significant enhancement (white vertical arrow). C: T2-weighted sagittal image shows clear improvement with decrease in tumor (white vertical arrow) size 7 weeks after birth. The basilar artery is now well seen (white horizontal arrow). D: T2-weighted sagittal image 4 months after birth reveals a continuous decrease in the size of the tumor compatible with a spontaneous remission. Note: after biopsy defect growth with persisting residual T2 hyperintensity. E: T2-weighted sagittal image 2 years and 3 months after birth with continuous decrease in the size of the tumor.

  • View in gallery

    Tumor regression—serial MRI axial images. A: Newborn at 2 days. T2-weighted axial image. The tumor is hyperintense on T2w and involves most part of the pons with encasement of the basilar artery. B: Newborn at 7 weeks after birth. T2-weighted axial image shows some regression of the tumor. C: T2-weighted axial image shows clear improvement with decrease in tumor 4 month after birth. The basilar artery is now well seen. D: T2-weighted axial image 2 years and 3 months after birth reveals a continuous decrease in the size of the tumor compatible with a spontaneous remission.

  • View in gallery

    Histological and immunohistochemical assessment of the tumor tissue. A: H&E staining of the tumor tissue revealed a focally moderately cell dense and pleomorphic glial tumor with small to medium-sized hyperchromatic nuclei and multipolar eosinophilic cellular protrusions. There were no hints of necrosis or vascular proliferations. B: Immunohistochemistry showing the proliferation marker Ki67 revealing a considerably elevated proliferative activity. C: Many neurofilament-positive axons (black arrows) are intermingled with tumor cells demonstrating a rather diffuse infiltration pattern of the tumor. While most tumor cell nuclei are positive for trimethylated histone H3K27 (H3K27me3) (D), the staining for its mutated counterpart H3K27 M (E) shows negative results indicating a wildtype H3K27 at the respective position and speaking against a DIPG, which would frequently be positive for this mutation.

References

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 82 82 82
Full Text Views 15 15 15
PDF Downloads 29 29 29
EPUB Downloads 0 0 0

PubMed

Google Scholar