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Gliomatosis cerebri: clinical characteristics, management, and outcomes

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Abstract

Gliomatosis cerebri is a rare diffusely infiltrating primary neoplastic glial process of the brain. Our objective is to review clinical presentation, management, and outcome in a large single institution series of gliomatosis cerebri patients. 54 consecutive gliomatosis cerebri cases presenting to Mayo Clinic Rochester between 1991 and 2008 were retrospectively reviewed. Inclusion criteria included involvement of at least three cerebral lobes, lack of a single discrete mass and pathological confirmation of diffuse glioma. Median overall survival (OS) was 18.5 months. Age, gender, presenting symptoms, and contrast enhancement did not correlate significantly with survival, though there was a trend toward decreased overall survival in patients above the median age of 46 years. Karnofsky performance score <70 was associated with poor OS (median 9.5 vs. 20.5 months, p = 0.02). Higher histologic grade was associated with poor progression-free survival (PFS; median for WHO grades II, III, and IV: 21.5, 6.5, and 4 months; p = 0.03) and OS (median 34, 15.5, and 8.5 months; p < 0.05). Radiation therapy was strongly associated with better prognosis (PFS 16.5 vs. 4.5 months, p < 0.01; OS 27.5 vs. 6.5, p < 0.01), but chemotherapy was not. Gliomatosis cerebri patients have a poor prognosis. Lower KPS upon presentation and higher histologic grade predict decreased survival. Surgery’s role is limited beyond biopsy for diagnostic purposes. Radiotherapy appears beneficial, although selection bias could be present in this retrospective study. Chemotherapy’s value is not as clear but this must be interpreted with caution given variable treatment regimens in this series.

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Acknowledgments

This data was presented in part at the Congress of Neurological Surgeons meeting in San Francisco, October 2010. Funding for CG was obtained through the Pathology Core of the Mayo Clinic Brain Cancer SPORE (NCI 1P50CA108961-06).

Conflict of interest

The authors have no conflicts of interest to disclose.

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Authors and Affiliations

  1. Department of Neurologic Surgery, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Selby Chen, Shota Tanaka & Ian F. Parney

  2. Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Caterina Giannini & Daniel H. Lachance

  3. Department of Radiology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Jonathan Morris

  4. Department of Radiation Oncology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Elizabeth S. Yan

  5. Department of Oncology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Jan Buckner

  6. Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Daniel H. Lachance

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  1. Selby Chen
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Correspondence to Selby Chen.

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Chen, S., Tanaka, S., Giannini, C. et al. Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol 112, 267–275 (2013). https://doi.org/10.1007/s11060-013-1058-x

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