8 Citations (Scopus)

Abstract

Adult brainstem gliomas are difficult to classify based on radiologic and histologic features. A K27M mutation in histone 3 has been described to identify high-grade midline gliomas associated with a particularly unfavorable prognosis. While initially considered a pediatric entity, it is now known that H3K27M-mutant brainstem gliomas occur in all age groups, but they are less well understood in adults. We studied clinical, radiologic, and pathologic features of 25 brainstem gliomas diagnosed at our institution between 1994 and 2017 in subjects at least 18 years old. Seven tumors (28%) were positive for the H3K27M mutation, and their median overall survival was significantly shorter than in the H3-wildtype group (p=0.004). Although the mutation was invariably associated with a poor prognosis, our study also illustrates the radiologic and pathologic heterogeneity in this molecular tumor subtype. The results showed that H3K27M-mutant status and clinically aggressive course cannot be ruled out based on low-grade histology on the initial biopsy, exophytic growth, only focal or minimal enhancement or an extrapontine location, such as midbrain or medulla. These results favor an integrated approach employing a combination of clinical, radiologic, histologic features as well as H3K27M immunohistochemistry for the diagnostic subclassification of adult brainstem gliomas.

Original languageEnglish (US)
Pages (from-to)302-311
Number of pages10
JournalJournal of Neuropathology and Experimental Neurology
Volume77
Issue number4
DOIs
StatePublished - Apr 1 2018

Fingerprint

Radiology
Glioma
Brain Stem
Pathology
Mutation
Mesencephalon
Histones
Neoplasms
Histology
Age Groups
Immunohistochemistry
Pediatrics
Biopsy
Survival
Growth

Keywords

  • Adult
  • Brainstem
  • Exophytic
  • Glioma
  • H3K27M mutation
  • Midline
  • Survival

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

@article{7e9e789d847b421ca91167e3ea7ce2ec,
title = "Adult brainstem gliomas with H3K27M mutation: Radiology, pathology, and prognosis",
abstract = "Adult brainstem gliomas are difficult to classify based on radiologic and histologic features. A K27M mutation in histone 3 has been described to identify high-grade midline gliomas associated with a particularly unfavorable prognosis. While initially considered a pediatric entity, it is now known that H3K27M-mutant brainstem gliomas occur in all age groups, but they are less well understood in adults. We studied clinical, radiologic, and pathologic features of 25 brainstem gliomas diagnosed at our institution between 1994 and 2017 in subjects at least 18 years old. Seven tumors (28{\%}) were positive for the H3K27M mutation, and their median overall survival was significantly shorter than in the H3-wildtype group (p=0.004). Although the mutation was invariably associated with a poor prognosis, our study also illustrates the radiologic and pathologic heterogeneity in this molecular tumor subtype. The results showed that H3K27M-mutant status and clinically aggressive course cannot be ruled out based on low-grade histology on the initial biopsy, exophytic growth, only focal or minimal enhancement or an extrapontine location, such as midbrain or medulla. These results favor an integrated approach employing a combination of clinical, radiologic, histologic features as well as H3K27M immunohistochemistry for the diagnostic subclassification of adult brainstem gliomas.",
keywords = "Adult, Brainstem, Exophytic, Glioma, H3K27M mutation, Midline, Survival",
author = "Daoud, {Elena V.} and Veena Rajaram and Chunyu Cai and Oberle, {Robert J.} and Martin, {Gregory R.} and Raisanen, {Jack M.} and White, {Charles L.} and Chan Foong and Mickey, {Bruce E.} and Edward Pan and Hatanpaa, {Kimmo J.}",
year = "2018",
month = "4",
day = "1",
doi = "10.1093/jnen/nly006",
language = "English (US)",
volume = "77",
pages = "302--311",
journal = "American Journal of Psychotherapy",
issn = "0002-9564",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Adult brainstem gliomas with H3K27M mutation

T2 - Radiology, pathology, and prognosis

AU - Daoud, Elena V.

AU - Rajaram, Veena

AU - Cai, Chunyu

AU - Oberle, Robert J.

AU - Martin, Gregory R.

AU - Raisanen, Jack M.

AU - White, Charles L.

AU - Foong, Chan

AU - Mickey, Bruce E.

AU - Pan, Edward

AU - Hatanpaa, Kimmo J.

PY - 2018/4/1

Y1 - 2018/4/1

N2 - Adult brainstem gliomas are difficult to classify based on radiologic and histologic features. A K27M mutation in histone 3 has been described to identify high-grade midline gliomas associated with a particularly unfavorable prognosis. While initially considered a pediatric entity, it is now known that H3K27M-mutant brainstem gliomas occur in all age groups, but they are less well understood in adults. We studied clinical, radiologic, and pathologic features of 25 brainstem gliomas diagnosed at our institution between 1994 and 2017 in subjects at least 18 years old. Seven tumors (28%) were positive for the H3K27M mutation, and their median overall survival was significantly shorter than in the H3-wildtype group (p=0.004). Although the mutation was invariably associated with a poor prognosis, our study also illustrates the radiologic and pathologic heterogeneity in this molecular tumor subtype. The results showed that H3K27M-mutant status and clinically aggressive course cannot be ruled out based on low-grade histology on the initial biopsy, exophytic growth, only focal or minimal enhancement or an extrapontine location, such as midbrain or medulla. These results favor an integrated approach employing a combination of clinical, radiologic, histologic features as well as H3K27M immunohistochemistry for the diagnostic subclassification of adult brainstem gliomas.

AB - Adult brainstem gliomas are difficult to classify based on radiologic and histologic features. A K27M mutation in histone 3 has been described to identify high-grade midline gliomas associated with a particularly unfavorable prognosis. While initially considered a pediatric entity, it is now known that H3K27M-mutant brainstem gliomas occur in all age groups, but they are less well understood in adults. We studied clinical, radiologic, and pathologic features of 25 brainstem gliomas diagnosed at our institution between 1994 and 2017 in subjects at least 18 years old. Seven tumors (28%) were positive for the H3K27M mutation, and their median overall survival was significantly shorter than in the H3-wildtype group (p=0.004). Although the mutation was invariably associated with a poor prognosis, our study also illustrates the radiologic and pathologic heterogeneity in this molecular tumor subtype. The results showed that H3K27M-mutant status and clinically aggressive course cannot be ruled out based on low-grade histology on the initial biopsy, exophytic growth, only focal or minimal enhancement or an extrapontine location, such as midbrain or medulla. These results favor an integrated approach employing a combination of clinical, radiologic, histologic features as well as H3K27M immunohistochemistry for the diagnostic subclassification of adult brainstem gliomas.

KW - Adult

KW - Brainstem

KW - Exophytic

KW - Glioma

KW - H3K27M mutation

KW - Midline

KW - Survival

UR - http://www.scopus.com/inward/record.url?scp=85045064745&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85045064745&partnerID=8YFLogxK

U2 - 10.1093/jnen/nly006

DO - 10.1093/jnen/nly006

M3 - Article

C2 - 29444279

AN - SCOPUS:85045064745

VL - 77

SP - 302

EP - 311

JO - American Journal of Psychotherapy

JF - American Journal of Psychotherapy

SN - 0002-9564

IS - 4

ER -