Skip to main content

Retrospective analysis of the clinical and radiological features of 94 consecutive DIPGs patients to investigate the factors determining the development of hydrocephalus and its impact on clinical status and survival

Child's Nervous System volume 36, pages 2701–2705 (2020)Cite this article

Abstract

Purpose

There is no consensus in the literature about the impact of hydrocephalus on clinical course and overall survival of diffuse intrinsic pontine gliomas (DIPG) patients as well as about its specific treatment. Authors reviewed a series of DIPG patients to investigate factors related to the onset of hydrocephalus, its treatment, and its impact on clinical course and prognosis.

Methods

A retrospective observational study was performed enrolling pediatric patients affected by DIPG from 2008 to 2018. Clinical and radiological charts were reviewed to find patients’ demographic, pathologic and radiologic features in hydrocephalic and non-hydrocephalic patients. In the hydrocephalus cohort, treatment strategy and its effectiveness and complications were analyzed.

Results

Ninety-four pediatric patients were enrolled in the study. Patients who developed hydrocephalus showed significantly lesser maximum axial tumor areas than patients without hydrocephalus (respectively 6.5 cm2 vs 16.45 cm2, p < 0.005). Hydrocephalus developed in 33 patients (35%) with an onset interval of 5.24 ± 1.21 months (range 3.2–7.3). The majority of hydrocephalic patients (28 cases, 90%) were treated by ventriculoperitoneal shunt, the remaining 3 patients being treated by endoscopic third ventriculostomy. Mean overall survival was 16.6 months ± 20 months without significative difference between the groups.

Conclusion

The onset of hydrocephalus occurs in the first moths of the disease story and found a negative correlation with tumor maximal axial diameter. Early treatment of hydrocephalus presents a very low complications rate with satisfying clinical outcome, as it allows the patients to continue the neurooncological therapies being a part of the treatment armamentarium instead of a palliative solution.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

Fig. 1
Fig. 2
Fig. 3

References

  1. Choux M (1999) Pediatric neurosurgery, 1. Publ. Churchill Livingstone, London

    Google Scholar 

  2. Khatua S, Moore KR, Vats TS, Kestle JRW (2011) Diffuse intrinsic pontine glioma—current status and future strategies. Childs Nerv Syst 27:1391–1397. https://doi.org/10.1007/s00381-011-1468-z

    Article  PubMed  Google Scholar 

  3. Hargrave D, Bartels U, Bouffet E (2006) Diffuse brainstem glioma in children: critical review of clinical trials. Lancet Oncol 7:241–248. https://doi.org/10.1016/S1470-2045(06)70615-5

    Article  PubMed  Google Scholar 

  4. Jansen MH, Veldhuijzen van Zanten SE, Sanchez Aliaga E, Heymans MW, Warmuth-Metz M, Hargrave D, van der Hoeven E, Gidding CE, de Bont ES, Eshghi OS, Reddingius R, Peeters CM, Schouten-van Meeteren A, Gooskens RH, Granzen B, Paardekooper GM, Janssens GO, Noske DP, Barkhof F, Kramm CM, Vandertop WP, Kaspers GJ, van Vuurden D (2015) Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria. Neuro-Oncol 17:160–166. https://doi.org/10.1093/neuonc/nou104

    Article  PubMed  Google Scholar 

  5. Freeman CR, Farmer J-P (1998) Pediatric brain stem gliomas: a review. Int J Radiat Oncol 40:265–271. https://doi.org/10.1016/S0360-3016(97)00572-5

    Article  CAS  Google Scholar 

  6. Jansen MHA, van Vuurden DG, Vandertop WP, Kaspers GJL (2012) Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology. Cancer Treat Rev 38:27–35. https://doi.org/10.1016/j.ctrv.2011.06.007

    Article  PubMed  CAS  Google Scholar 

  7. Amano T, Inamura T, Nakamizo A, Inoha S, Wu CM, Ikezaki K (2002) Case management of hydrocephalus associated with the progression of childhood brain stem gliomas. Childs Nerv Syst 18:599–604. https://doi.org/10.1007/s00381-002-0637-5

    Article  PubMed  Google Scholar 

  8. Roujeau T, Di Rocco F, Dufour C et al (2011) Shall we treat hydrocephalus associated to brain stem glioma in children? Childs Nerv Syst 27:1735–1739. https://doi.org/10.1007/s00381-011-1538-2

    Article  PubMed  Google Scholar 

  9. Cohen BH (2006) Tumors of the pediatric central nervous system. Neuro-Oncol 8:284–285. https://doi.org/10.1215/15228517-2006-009

    Article  Google Scholar 

  10. Veldhuijzen van Zanten SEM, Baugh J, Chaney B, de Jongh D, Sanchez Aliaga E, Barkhof F, Noltes J, de Wolf R, van Dijk J, Cannarozzo A, Damen-Korbijn CM, Lieverst JA, Colditz N, Hoffmann M, Warmuth-Metz M, Bison B, Jones DTW, Sturm D, Gielen GH, Jones C, Hulleman E, Calmon R, Castel D, Varlet P, Giraud G, Slavc I, van Gool S, Jacobs S, Jadrijevic-Cvrlje F, Sumerauer D, Nysom K, Pentikainen V, Kivivuori SM, Leblond P, Entz-Werle N, von Bueren A, Kattamis A, Hargrave DR, Hauser P, Garami M, Thorarinsdottir HK, Pears J, Gandola L, Rutkauskiene G, Janssens GO, Torsvik IK, Perek-Polnik M, Gil-da-Costa MJ, Zheludkova O, Shats L, Deak L, Kitanovski L, Cruz O, Morales la Madrid A, Holm S, Gerber N, Kebudi R, Grundy R, Lopez-Aguilar E, Zapata-Tarres M, Emmerik J, Hayden T, Bailey S, Biassoni V, Massimino M, Grill J, Vandertop WP, Kaspers GJL, Fouladi M, Kramm CM, van Vuurden D, members of the SIOPE DIPG Network (2017) Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease. J Neuro-Oncol 132:255–266. https://doi.org/10.1007/s11060-016-2363-y

    Article  Google Scholar 

  11. Veldhuijzen van Zanten SEM, Lane A, Heymans MW, Baugh J, Chaney B, Hoffman LM, Doughman R, Jansen MHA, Sanchez E, Vandertop WP, Kaspers GJL, van Vuurden D, Fouladi M, Jones BV, Leach J (2017) External validation of the diffuse intrinsic pontine glioma survival prediction model: a collaborative report from the International DIPG Registry and the SIOPE DIPG Registry. J Neuro-Oncol 134:231–240. https://doi.org/10.1007/s11060-017-2514-9

    Article  Google Scholar 

  12. Fischbein NJ, Prados MD, Wara W, Russo C, Edwards MS, Barkovich AJ (1996) Radiologic classification of brain stem tumors: correlation of magnetic resonance imaging appearance with clinical outcome. Pediatr Neurosurg 24:9–23. https://doi.org/10.1159/000121010

    Article  PubMed  CAS  Google Scholar 

  13. Löbel U, Hwang S, Edwards A, Li Y, Li X, Broniscer A, Patay Z (2016) Discrepant longitudinal volumetric and metabolic evolution of diffuse intrinsic pontine gliomas during treatment: implications for current response assessment strategies. Neuroradiology 58:1027–1034. https://doi.org/10.1007/s00234-016-1724-8

    Article  PubMed  PubMed Central  Google Scholar 

  14. Steffen-Smith EA, Sarlls JE, Pierpaoli C et al (2014) Diffusion tensor histogram analysis of pediatric diffuse intrinsic pontine glioma. Biomed Res Int 2014:1–9. https://doi.org/10.1155/2014/647356

    Article  Google Scholar 

  15. Khatua S, Hou P, Bodiwala R, Wolff J, Hamilton J, Patil S, Zaky W, Mahajan A, Ketonen L (2014) Preliminary experience with diffusion tensor imaging before and after re-irradiation treatments in children with progressive diffuse pontine glioma. Childs Nerv Syst 30:925–930. https://doi.org/10.1007/s00381-013-2350-y

    Article  PubMed  Google Scholar 

  16. Poussaint TY, Vajapeyam S, Ricci KI, Panigrahy A, Kocak M, Kun LE, Boyett JM, Pollack IF, Fouladi M (2016) Apparent diffusion coefficient histogram metrics correlate with survival in diffuse intrinsic pontine glioma: a report from the pediatric brain tumor consortium. Neuro-Oncol 18:725–734. https://doi.org/10.1093/neuonc/nov256

    Article  PubMed  CAS  Google Scholar 

  17. Guida L, Roux F-E, Massimino M et al (2019) Safety and efficacy of endoscopic third ventriculostomy in diffuse intrinsic pontine glioma related hydrocephalus: a systematic review. World Neurosurg 124:29–35. https://doi.org/10.1016/j.wneu.2018.12.096

    Article  Google Scholar 

Download references

Author information

Affiliations

  1. Neurosurgery, School of Medicine, Ospedale San Gerardo, Università degli Studi di Milano Bicocca, Monza, Italy

    Carlo Giussani, Lelio Guida, Andrea Trezza & Erik Sganzerla

  2. Pediatric Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Veronica Biassoni, Elisabetta Schiavello & Maura Massimino

  3. Neurosurgery, School of Medicine, Fondazione IRCCS Ca’ Granda, Università degli studi di Milano, Milan, Italy

    Giorgio Carrabba

Authors
  1. Carlo Giussani
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lelio Guida
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Veronica Biassoni
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Elisabetta Schiavello
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Giorgio Carrabba
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Andrea Trezza
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Erik Sganzerla
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Maura Massimino
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Carlo Giussani.

Ethics declarations

The medical ethical committee approved the study (as amendment to clinical protocol INT 153/15).

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Giussani, C., Guida, L., Biassoni, V. et al. Retrospective analysis of the clinical and radiological features of 94 consecutive DIPGs patients to investigate the factors determining the development of hydrocephalus and its impact on clinical status and survival. Childs Nerv Syst 36, 2701–2705 (2020). https://doi.org/10.1007/s00381-020-04589-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-020-04589-4

Keywords

  • Diffuse intrinsic pontine glioma
  • Hydrocephalus
  • Endoscopic third ventriculostomy
  • Ventriculo peritoneal shunt