Original ArticleManagement and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis
Introduction
The World Health Organization (WHO) previously defined gliomatosis cerebri (GC) as a diffusely infiltrating glioma with involvement of 3 or more lobes of the cerebrum.1 Histologically, GC can originate from neoplastic astrocytes or oligodendrocytes, and for many years, studies have hypothesized that gliomatosis does not represent a unique disease state.1, 2, 3 In the 2016 update, WHO reclassified GC as a pattern of growth for astrocytomas or oligodendrogliomas rather than a distinct pathologic entity.4 Regardless of classification, patients with glioma involving three or more lobes show notably poor and highly variable clinical courses depending on grade and underlying histology.5, 6, 7, 8, 9
The incidence of GC is low, with reported cases in the hundreds.10 The number of cases of histology-specific subtypes is even lower. In oncology, rare tumors are often grouped based on similar characteristics to gather preliminary data. For instance, WHO grade II astrocytoma and WHO grade II oligodendroglioma are grouped into the category of low-grade glioma in several landmark studies.11, 12 Despite limitations with this method, these efforts have identified valuable characteristics of rare tumors. We adopted this approach in our study and combined astrocytic and oligodendrocytic GC into a single category. We used the Surveillance Epidemiology and End Results (SEER) database (1999–2010) to analyze patterns of diagnosis and management of patients with GC. We explored the method of diagnosis, rate of radiation therapy (RT), and factors predictive of overall survival in this population. Implications of these findings are discussed.
Section snippets
Data and Study Population
The SEER Program is a national registry designed and run by the National Cancer Institute. The database contains incidence and survival data on patients with cancer from 18 population-based cancer registries, representing more than one quarter of the total U.S. population (SEER Research Data 1973–2010). Data for this study were downloaded from the SEER database as ASCII text files. We included patients diagnosed with glioma between 1999 and 2010, because of reliable coding for extent of
Patient and Clinical Characteristics
We identified 111 adult patients with GC in the SEER database. In the same database, we previously identified 21,962 patients with glioblastoma, 2775 patients with AA,16 and 2378 patients with WHO grade II oligodendroglioma (data not published). We therefore estimate the incidence of GC to be approximately 1/25 of AA and oligodendroglioma and 1/200 of glioblastoma. Table 1 shows descriptive statistics of the demographic variables. The median age was 66 years, with a range of 26–98 years. There
Discussion
Our SEER-based analysis estimates the incidence of GC to be ∼1/25 of oligodendroglioma (data not published) and ∼1/200 of glioblastoma,16 two forms of cancer already known for their rarity. The most notable finding of our analysis is that 2 of 5 patients who presented with GC to a SEER institution did not receive tissue confirmation of their diagnosis. Furthermore, 7% of patients who did not have microscopic confirmation of their diagnosis received radiation. This practice pattern contrasts
Conclusions
We found that approximately 40% of SEER patients with GC were not diagnosed based on microscopic confirmation of surgically acquired tissues. We propose that tissue diagnosis is warranted in patients with GC, because genomic analysis of these specimens may provide insights that will contribute to meaningful therapeutic intervention.
References (42)
- et al.
Gliomatosis cerebri: improved outcome with radiotherapy
Int J Radiat Oncol Biol Phys
(2003) - et al.
Superior efficacy of gross total resection in anaplastic astrocytoma patients relative to glioblastoma patients
World Neurosurg
(2016) - et al.
The new WHO classification of brain tumours
Brain Pathol
(1993) - et al.
Gliomatosis cerebri: no evidence for a separate brain tumor entity
Acta Neuropathol
(2016) - et al.
Gliomatosis cerebri: growing evidence for diffuse gliomas with wide invasion
Expert Rev Neurother
(2008) - et al.
The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary
Acta Neuropathol
(2016) - et al.
Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases
Childs Nerv Syst
(2013) - et al.
Correlative analysis of gene expression profile and prognosis in patients with gliomatosis cerebri
Cancer
(2009) - et al.
Pediatric Gliomatosis Cerebri: A Review of 15 Years
J Child Neurol
(2015) - et al.
Genetic aberrations in gliomatosis cerebri
Neurosurgery
(2007)
NOA-05 phase 2 trial of procarbazine and lomustine therapy in gliomatosis cerebri
Ann Neurol
Prognostic factors for survival in adult patients with cerebral low-grade glioma
J Clin Oncol
Prospective randomized trial of low-versus high-dose radiation therapy in adults with supratentorial low-grade glioma: initial report of a North Central Cancer Treatment Group/Radiation Therapy Oncology Group/Eastern Cooperative Oncology Group Study
J Clin Oncol
CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2008–2012
Neuro Oncol
Recursive partitioning analysis of prognostic factors in three Radiation Therapy Oncology Group malignant glioma trials
J Natl Cancer Inst
Gross-total resection outcomes in an elderly population with glioblastoma: a SEER-based analysis
J Neurosurg
Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma
N Engl J Med
Initial chemotherapy in gliomatosis cerebri
Neurology
Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature
J Neurooncol
Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types
Clin Neuropathol
Gray matter involvement predicts chemosensitivity and prognosis in gliomatosis cerebri
Neurology
Cited by (14)
Predictive factors for overall survival in surgical cases of gliomatosis cerebri from the National Cancer Database
2020, Journal of Clinical NeuroscienceCitation Excerpt :As GC is a diffusely infiltrative, poorly delineated process, gross total resection is unfeasible without significant morbidity [15], and only 12% of reported cases in our analysis achieved near/gross total resection. In the literature, the extent of resection (partial vs. near/gross total) does not seem to impact overall survival [4,8,10,11,15,20]. However, one study indicates improved survival in patients with partial resection compared to biopsy [18], another study identifies partial resection as indicative of progression-free survival rather than overall survival [20], and others find that surgical resection, whether partial or subtotal, improves overall survival [11,21].
Extent of resection, molecular signature, and survival in 1p19q-codeleted gliomas
2021, Journal of NeurosurgeryGliomatosis cerebri: A monocentric real-life experience
2021, Journal of Cancer Metastasis and TreatmentImpact of facility type and volume in low-grade glioma Outcomes
2020, Journal of Neurosurgery
Conflict of interest statement: The project described was partially supported by the National Institutes of Health, grant TL1TR001443 (KTC) of CTSA funding. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.