Elsevier

World Neurosurgery

Volume 103, July 2017, Pages 186-193
World Neurosurgery

Original Article
Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis

https://doi.org/10.1016/j.wneu.2017.03.103Get rights and content

Objective

We used the SEER (Surveillance Epidemiology and End Results) database (1999–2010) to analyze the clinical practice patterns and overall survival in patients with gliomatosis cerebri (GC), or glioma involving 3 or more lobes of the cerebrum.

Methods

We identified 111 patients (age ≥18 years) with clinically or microscopically diagnosed GC in the SEER database. Analyses were performed to determine clinical practice patterns for these patients and whether these practices were associated with survival.

Results

Fifty-eight percent of the 111 patients with GC received microscopic confirmation of their diagnosis. Of the remaining patients, 40% were diagnosed via imaging or laboratory tests, and 2% had unknown methods of diagnosis. Seven percent of patients who did not have microscopic confirmation of their diagnosis received radiation therapy. Radiation therapy and surgery were not associated with survival. The only variable significantly associated with overall survival was age at diagnosis. Patients aged 18–50 years showed improved survival relative to patients aged >50 years (median survival, 11 and 6 months, respectively; P = 0.03). For patients aged >50 years, improved overall survival was observed in the post-temozolomide era (2005–2010) relative to those treated in the pre-temozolomide era (1999–2004) (median survival, 9 and 4 months, respectively; P = 0.005).

Conclusions

In the SEER database, ∼40% of the patients with glioma with imaging findings of GC do not receive microscopic confirmation of their diagnosis. We propose that tissue confirmation is warranted in patients with GC, because genomic analysis of these specimens may provide insights that will contribute to meaningful therapeutic intervention.

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.

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    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.

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