Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Feb 13;11(4):559.
doi: 10.3390/healthcare11040559.

Emerging Therapeutic Strategies for Diffuse Intrinsic Pontine Glioma: A Systematic Review

Affiliations
Review

Emerging Therapeutic Strategies for Diffuse Intrinsic Pontine Glioma: A Systematic Review

Shahrukh Farrukh et al. Healthcare (Basel). .

Abstract

Background: Of all central nervous systems tumors, 10-20% are located in the brainstem; diffuse intrinsic pontine glioma (DIPG) is diagnosed in 80% of them. With over five decades of clinical trial testing, there are no established therapeutic options for DIPG. This research article aims to collate recent clinical trial data and provide a landscape for the most promising therapies that have emerged in the past five years.

Methods: PubMed/MEDLINE, Web of Science, Scopus, and Cochrane were systematically searched using the following keywords: Diffuse intrinsic pontine glioma, Pontine, Glioma, Treatment, Therapy, Therapeutics, curative, and/or Management. Both adult and pediatric patients with newly diagnosed or progressive DIPG were considered in the clinical trial setting. The risk of bias was assessed using the ROBINS-I tool.

Results: A total of 22 trials were included reporting the efficacy and safety outcomes among patients. First, five trials reported outcomes of blood-brain barrier bypass via single or repeated-dose intra-arterial therapy or convection-enhanced delivery. Second, external beam radiation regimens were assessed for safety and efficacy in three trials. Third, four trials administered intravenous treatment without using chemotherapeutic regimens. Fourth, eight trials reported the combinations of one or more chemotherapeutic agents. Fifth, immunotherapy was reported in two trials in an adjuvant monotherapy in the post-radiotherapy setting.

Conclusion: This research article captures a clinical picture of the last five years of the direction toward which DIPG research is heading. The article finds that re-irradiation may prolong survival in patients with progressive DIPG; it also instills that insofar palliative radiotherapy has been a key prognostic choice.

Keywords: CNS; advances; diffuse intrinsic pontine glioma; palliative; quality of life; therapies; tumor.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
PRISMA flowchart depicting the study selection process.
Figure 2
Figure 2
Summary plot and traffic light plot depicting risk of bias among the included studies [13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34].

References

    1. Linabery A.M., Ross J.A. Trends in childhood cancer incidence in the US (1992–2004) Cancer Interdiscip. Int. J. Am. Cancer Soc. 2008;112:416–432. - PubMed
    1. Ostrom Q.T., Cioffi G., Waite K., Kruchko C., Barnholtz-Sloan J.S. CBTRUS statistical report: Primary brain and other central nervous system tumors diagnosed in the United States in 2014–2018. Neuro Oncol. 2021;23:iii1–iii105. doi: 10.1093/neuonc/noab200. - DOI - PMC - PubMed
    1. Ostrom Q.T., de Blank P.M., Kruchko C., Petersen C.M., Liao P., Finlay J.L., Stearns D.S., Wolff J.E., Wolinsky Y., Letterio J.J. Alex’s Lemonade Stand Foundation infant and childhood primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol. 2015;16:x1–x36. doi: 10.1093/neuonc/nou327. - DOI - PMC - PubMed
    1. Li G., Mitra S.S., Monje M., Henrich K.N., Bangs C.D., Nitta R.T., Wong A.J. Expression of epidermal growth factor variant III (EGFRvIII) in pediatric diffuse intrinsic pontine gliomas. J. Neurooncol. 2012;108:395–402. doi: 10.1007/s11060-012-0842-3. - DOI - PMC - PubMed
    1. Rashed W.M., Maher E., Adel M., Saber O., Zaghloul M.S. Pediatric diffuse intrinsic pontine glioma: Where do we stand? Cancer Metastasis Rev. 2019;38:759–770. doi: 10.1007/s10555-019-09824-2. - DOI - PubMed

LinkOut - more resources