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Diffuse Intrinsic Pontine Glioma (DIPG)

What is Diffuse Intrinsic Pontine Glioma?

Diffuse intrinsic pontine glioma (DIPG) is a malignant tumor of the glial tissue of the nervous system that originates in the largest part of the brainstem. It is found almost exclusively in children, with diagnosis usually between the ages of 6-7 years.1,2,3 Diffuse intrinsic pontine glioma accounts for 10-15% of all brain tumors in children.4,5

More than half of patients experience symptoms such as unpaired eye movement, double vision, slurred speech, difficulty swallowing, trouble maintaining balance, or a drooping of one part of the face.6 Most patients survive less than 1 year, with less than 10% of patients surviving beyond 2 years after diagnosis.7,8

How Common is DIPG?

Diffuse intrinsic pontine glioma is an ultra-rare pediatric disease with a slightly higher frequency in males.9 Incidence of this type of tumor is 1 to 2 cases per 100,000 people.1

How is PTC Working to Treat DIPG?

PTC596 is an orally bioavailable small molecule tubulin inhibitor that arrests tumor cells, including cancer stem cells, in the G2/M cell cycle phase, through the action of inhibiting tubulin polymerization.10

PTC is currently recruiting for a phase 1b clinical trial

References

[1] Srikanthan D, et al. Chin Neurosurg J 2021;7:6

[2] Chamberlain MC. J Neuro-Oncol 1993;15(2):133–139.

[3] Littman P, et al. Cancer 1980;45:2787–2792.

[4] Minturn JE, et al. Curr Treat Options Neurol 2013;15:316–327.

[5] Freeman CR, et al. Int J Radiat Oncol Biol Phys 1998;40:265–271.

[6] Donaldson SS, et al. Advances toward an understanding of brainstem gliomas. J Clin Oncol. 2006;24(8):1266-72

[7] Fisher PG, et al. A clinicopathologic reappraisal of brain stem tumor classification. Identification of pilocystic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer. 2000 Oct 01;89(7):1569-76.

[8] Rashed WM, et al. Pediatric diffuse intrinsic pontine glioma: where do we stand? Cancer Metastasis Rev. 2019;38(4):759-770.

[9] Chen J, et al. Identification of new therapeutic targets and natural compounds against diffuse intrinsic pontine glioma (DIPG). Bioorg Chem. 2020;99:103847

[10] Jernigan F, et al. Preclinical and Early Clinical Development of PTC596, a Novel Small-Molecule Tubulin-Binding Agent. Molecular Cancer Therapeutics. 2021;20:1846-1857. Published OnlineFirst July 26, 2021; DOI: 10.1158/1535-7163.MCT-20-0774

How Can You Stay Informed About DIPG?

If you wish to speak to us regarding our therapeutic approach, please reach out.