Introduction:
Diffuse intrinsic pontine glioma (DIPG) is an aggressive lethal brainstem tumor that occurs predominantly in the pediatric population, accounting for nearly 80% of tumors in this location1-3. Currently, focal radiation therapy (RT) is the only intervention that temporarily halts tumor progression with clinical and radiologic responses in the majority of cases.4 However, most tumors progress after a few months of upfront irradiation.2,3 Although critical molecular alterations and biologic pathways have been identified in the last decade,5 no effective drugs have been introduced into the clinic. At first progression, focal reirradiation (reRT) seems to offer clinical and radiological benefit to patients, particularly to those with initial and maintained response to irradiation.6 2,7 However, no clear recommendations exist in the context of metastatic progression. Anecdotally, CSI has been used upfront in the treatment of a few metastatic DIPG patients alone or in combination with other treatments.8-10 To our knowledge, there are no reports of its use at first metastatic progression.