Bridge over troubled waters.

Top Line: What clinical or molecular features suggest improved treatment outcomes for patients with diffuse intrinsic pontine glioma (DIPG)?
The Study: DIPG is depressing. Much of  CNS oncology is seeing a molecular renaissance, but DIPG has been largely left out. While that’s pretty much still true, this study provides us with the largest DIPG molecular data to date. Over 1K cases of DIPG were assembled from multiple international registries, and roughly 30%(!) of patients had biopsies. Most patients (75%) received combined chemoradiation, and the rest primarily received radiation alone. The median overall survival (OS) was 11 months, and rates of OS at one and two years were 42% and 10%. This leaves a small subset of long-term survival (LTS, 24-60 months) and a tiny subset (1-2%) of very LTS (>60 months). LTS was associated with <3 or >10 years of age, a more protracted onset of symptoms (>12-24 weeks) that didn’t include cranial neuropathy, and receipt of chemoradiation. Histone 3 mutations were important, specifically H3.1 K27M that predicted LTS and H3.3 K27M that predicted no LTS.
Bottom Line: Very young or older DIPG patients with protracted symptom onset treated with chemoradiation may have a higher (but still tiny) likelihood of favorable outcome. | Hoffman, J Clin Oncol 2018

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