Indolent course of brainstem tumors with K27M-H3.3 mutation

Lorena V. Baroni; Palma Solano-Paez; Liana Nobre; Orli Michaeli; Cynthia Hawkins; Suzanne Laughlin; Ute Bartels; Vijay Ramaswamy; Eric Bouffet

doi:10.1002/pbc.28102

Indolent course of brainstem tumors with K27M-H3.3 mutation

Lorena V. Baroni, Palma Solano-Paez, Liana Nobre, Orli Michaeli, Cynthia Hawkins, Suzanne Laughlin, Ute Bartels, Vijay Ramaswamy, Eric Bouffet^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

4 Scopus citations

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.

Original language	English
Article number	e28102
Journal	Pediatric Blood and Cancer
Volume	67
Issue number	3
DOIs	https://doi.org/10.1002/pbc.28102
State	Published - 1 Mar 2020
Externally published	Yes

Funding

Funders	Funder number
Walk Foundation
Terry Fox Foundation

Keywords

DIPG
K27M
brainstem
diffuse intrinsic pontine glioma
indolent course
tumor

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1002/pbc.28102

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title = "Indolent course of brainstem tumors with K27M-H3.3 mutation",

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.",

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AU - Baroni, Lorena V.

AU - Solano-Paez, Palma

AU - Nobre, Liana

AU - Michaeli, Orli

AU - Hawkins, Cynthia

AU - Laughlin, Suzanne

AU - Bartels, Ute

AU - Ramaswamy, Vijay

AU - Bouffet, Eric

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N2 - 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.

AB - 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.

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Indolent course of brainstem tumors with K27M-H3.3 mutation

Abstract

Funding

Keywords

UN SDGs

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