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.