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Survival and functional outcomes in paediatric thalamic and thalamopeduncular low grade gliomas

  • Original Article -Pediatric Neurosurgery
  • Published:
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Abstract 

Background

Childhood thalamopeduncular gliomas arise at the interface of the thalamus and cerebral peduncle. The optimal treatment is total resection but not at the cost of neurological function. We present long-term clinical and oncological outcomes of maximal safe resection.

Methods

Retrospective review of prospectively collected data: demography, symptomatology, imaging, extent of resection, surgical complications, histology, functional and oncological outcome.

Results

During 16-year period (2005–2020), 21 patients were treated at our institution. These were 13 girls and 8 boys (mean age 7.6 years). Presentation included progressive hemiparesis in 9 patients, raised intracranial pressure in 9 patients and cerebellar symptomatology in 3 patients. The tumour was confined to the thalamus in 6 cases. Extent of resection was judged on postoperative imaging as total (6), near-total (6) and less extensive (9). Surgical complications included progression of baseline neurological status in 6 patients, and 5 of these gradually improved to preoperative status. All tumours were classified as low-grade gliomas. Disease progression was observed in 9 patients (median progression-free survival 7.3 years). At last follow-up (median 6.1 years), all patients were alive, median Lansky score of 90. Seven patients were without evidence of disease, 6 had stable disease, 7 stable following progression and 1 had progressive disease managed expectantly.

Conclusion

Paediatric patients with low-grade thalamopeduncular gliomas have excellent long-term functional and oncological outcomes when gross total resection is not achievable. Surgery should aim at total resection; however, neurological function should not be endangered due to excellent chance for long-term survival.

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Availability of data and materials

Available from the corresponding author upon reasonable request.

Code availability

Not applicable.

Abbreviations

cDNA:

Complementary deoxyribonucleic acid

CST:

Cortico-spinal tract

DNA:

Deoxyribonucleic acid

EOR:

Extent of resection

ETV:

Endoscopic third ventriculostomy

FLAIR:

Fluid-attenuated inversion recovery

GTR:

Gross total resection

LGG:

Low-grade glioma

MCS:

Milan Complexity Scale

MRI:

Magnetic resonance imaging

NTR:

Near total resection

OS:

Overall survival

PCR:

Polymerase chain reaction

PFS:

Progression-free survival

PR:

Partial resection

RNA:

Ribonucleic acid

RT PCR:

Reverse transcriptase polymerase chain reaction

STR:

Subtotal resection

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Funding

This work was supported by the Grant Agency of Charles University, Prague PRIMUS/19/MED/06 and Research Project of the Ministry of Health of the Czech Republic No 00064203.

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Authors and Affiliations

  1. Department of Neurosurgery, Second Faculty of Medicine, Charles University and Motol University Hospital, V Úvalu 84, Prague, Czech Republic

    Vladimír Beneš 3rd, Petr Libý, Jakub Táborský, Jana Blažková Jr, Ondřej Bradáč & Michal Tichý

  2. Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

    Michal Zápotocký, David Sumerauer & Adéla Mišove

  3. Department of Anaesthesiology and Intensive Care Medicine, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

    Jana Blažková Sr

  4. Department of Paediatric Neurology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

    Ivana Perníková

  5. Department of Radiology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

    Martin Kynčl

  6. Department of Pathology and Molecular Medicine, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

    Lenka Krsková, Miroslav Koblížek & Josef Zámečník

  7. Department of Neurosurgery and Neurooncology, First Medical Faculty, Charles University and Military University Hospital, Prague, Czech Republic

    Ondřej Bradáč

Authors
  1. Vladimír Beneš 3rd
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  2. Michal Zápotocký
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Vladimir Beneš 3rd, Michal Zápotocký, Petr Libý, Jakub Táborský, Jana Blažková Jr., Jana Blažková Sr., David Sumerauer, Adéla Mišove, Ivana Perníková, Martin Kynčl, Lenka Krsková, Miroslav Koblížek, Josef Zámečník, Ondřej Bradáč and Michal Tichý. The first draft of the manuscript was written by Vladimír Beneš 3rd and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Vladimír Beneš 3rd.

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Ethics approval

Ethical approval was waived by the local Ethics Committee of Second Faculty of Medicine, Charles University and Motol University Hospital in view of the retrospective nature of the study and all the procedures being performed were part of the routine care.

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The authors declare no competing interests.

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Comments

This manuscript describes a series of 21 children with thalamic and thalamopeduncular low grade gliomas treated between 2005 and 2020. The authors provide a good description of their patients and their surgical technique, emphasising their choice of operative approach, their use of adjunctive methods to maximise surgical safety and the fact that a good long term neurological and oncological outcome does not necessarily require a complete resection of the tumour. The authors have addressed comments from the initial reviewers. Although other similar series have been described, these are rare tumours and a relatively large well-described and well-managed series such as this one is still a useful addition to the literature. Within the current paradigms of chemotherapy and targeted therapy for low grade gliomas, their emphasis on surgical safety rather than complete resection is important. This is not always clarified in surgical series.

Kristian Aquilina

London, UK

QueryPrevious presentation: Accepted in part as an ePoster presentation for EANS 2021 Virtual Congress, October 03–07, 2021

This article is part of the Topical Collection on Pediatric Neurosurgery

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Beneš, V., Zápotocký, M., Libý, P. et al. Survival and functional outcomes in paediatric thalamic and thalamopeduncular low grade gliomas. Acta Neurochir 164, 1459–1472 (2022). https://doi.org/10.1007/s00701-021-05106-5

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