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Published on: January 9, 2019
Indolent course of brainstem tumors with K27M-H3.3 mutation
Lorena V Baroni1,2, Palma Solano-Paez1, Liana Nobre1
1Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
Abstract:
Diffuse intrinsic pontine glioma (DIPG) is characterized by a short history of brainstem symptoms and well-known magnetic resonance imaging features with a fatal outcome. However, we report three unusual cases of brainstem tumors with an initial indolent and protracted course, which subsequently developed the classical imaging and clinical features of DIPG. Our findings support this notion that K27M is an early event in development and suggest that the emergence of additional events resulted in rapid progression after a long period of latency. Identification of such markers of aggressive behavior in the context of an indolent course is needed for better characterization and treatment management.
Insights
Diffuse intrinsic pontine glioma (DIPG) typically progresses rapidly. However, three unusual cases showed a slow onset before developing classical DIPG features, suggesting K27M mutations may occur early.
Area of Science:
- Pediatric neuro-oncology
- Brainstem tumor research
- Cancer genetics
Background:
- Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive brainstem tumor with a poor prognosis.
- DIPG typically presents with rapid onset of neurological symptoms and characteristic MRI findings.
Observation:
- Three pediatric patients presented with brainstem tumors exhibiting an unusually indolent and protracted clinical course.
- These tumors later transformed, displaying the classical imaging and clinical features of DIPG.
Findings:
- The K27M mutation, a known driver in DIPG, is hypothesized to be an early event in tumor development.
- Subsequent acquisition of additional genetic events likely triggers rapid progression after a prolonged latency period.
Implications:
- This challenges the traditional view of DIPG's rapid progression, highlighting potential for delayed onset.
- Identifying markers for aggressive behavior in indolent brainstem tumors is crucial for accurate diagnosis and therapeutic strategies.
