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Oncologist. 2019 Mar;24(3):402-413. doi: 10.1634/theoncologist.2018-0086. Epub 2018 Aug 10.

Wide Range of Clinical Outcomes in Patients with Gliomatosis Cerebri Growth Pattern: A Clinical, Radiographic, and Histopathologic Study.

Author information

1
Stephen E. and Catherine Pappas Center for Neuro-Oncology, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA ily@partners.org jdietrich1@partners.org.
2
Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA.
3
Department of Medicine, Yale New Haven Hospital, New Haven, Connecticut, USA.
4
Department of Biostatistics, Harvard School of Public Health, Boston, Massachusetts, USA.
5
Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts, USA.
6
Scintillon Institute, San Diego, California, USA.
7
Stephen E. and Catherine Pappas Center for Neuro-Oncology, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.
8
Department of Neurosurgery, Massachusetts General Hospital, Boston, Massachusetts, USA.

Abstract

BACKGROUND:

The 2016 World Health Organization Classification of Central Nervous System Tumors categorizes gliomatosis cerebri growth pattern (GC) as a subgroup of diffuse infiltrating gliomas, defined by extent of brain involvement on magnetic resonance imaging (MRI). Clinical and radiographic features in GC patients are highly heterogeneous; however, prognosis has historically been considered poor.

SUBJECTS, MATERIALS, AND METHODS:

We performed a retrospective search for patients at our institution meeting radiographic criteria of primary, type I GC (defined as diffuse tumor infiltration without associated tumor mass and contrast enhancement on MRI) and analyzed their clinical, imaging, and histopathologic features.

RESULTS:

A total of 34 patients met radiographic criteria of primary, type I GC, and 33 had a confirmed histologic diagnosis of an infiltrating glial neoplasm. Age >47 years at diagnosis was associated with worse overall survival (OS) compared with age ≤47 years (hazard ratio [HR] 1.04, 95% confidence interval [CI] 1.01-1.07, p = .003). Patients with grade 2 tumors demonstrated a trend for improved OS compared with those with grade 3 tumors (HR 2.65, 95% CI 0.99-7.08, p = .051). Except for brainstem involvement, extent or location of radiographic involvement did not detectably affect clinical outcome. IDH mutation status identified a subgroup of GC patients with particularly long survival up to 25 years and was associated with longer time to progression (HR 4.81, 95% CI 0.99-23.47, p = .052).

CONCLUSION:

Patients with primary, type I GC do not uniformly carry a poor prognosis, even in the presence of widespread radiographic involvement. Consistent with other reports, IDH mutation status may identify patients with improved clinical outcome. Molecular characterization, rather than MRI features, may be most valuable for prognostication and management of GC patients.

IMPLICATIONS FOR PRACTICE:

Patients with gliomatosis cerebri growth pattern (GC) constitute a challenge to clinicians, given their wide range of clinical, histologic, and radiographic presentation, heterogeneous outcome patterns, and the lack of consensus on a standardized treatment approach. This study highlights that radiographic extent of disease-albeit category-defining-does not detectably influence survival and that IDH mutations may impact clinical outcome. Practicing oncologists should be aware that select GC patients may demonstrate exceptionally favorable survival times and prognosticate patients based on molecular markers, rather than imaging features alone.

KEYWORDS:

Diffuse infiltrating gliomas; Gliomatosis cerebri; Isocitrate dehydrogenase; Magnetic resonance imaging; Molecular markers

PMID:
30097523
PMCID:
PMC6519760
[Available on 2020-03-01]
DOI:
10.1634/theoncologist.2018-0086

Conflict of interest statement

Disclosures of potential conflicts of interest may be found at the end of this article.

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