Gliomatosis cerebri: clinical characteristics, management, and outcomes
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CLINICAL STUDY
Gliomatosis cerebri: clinical characteristics, management, and outcomes Selby Chen • Shota Tanaka • Caterina Giannini • Jonathan Morris • Elizabeth S. Yan • Jan Buckner Daniel H. Lachance • Ian F. Parney
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Received: 25 October 2012 / Accepted: 14 January 2013 / Published online: 23 January 2013 Ó Springer Science+Business Media New York 2013
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 Electronic supplementary material The online version of this article (doi:10.1007/s11060-013-1058-x) contains supplementary material, which is available to authorized users. S. Chen (&) S. Tanaka I. F. Parney Department of Neurologic Surgery, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA e-mail: [email protected] C. Giannini D. H. Lachance Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA J. Morris Department of Radiology, Mayo Clinic College of Medicine, Rochester, MN, USA E. S. Yan Department of Radiation Oncology, Mayo Clinic College of Medicine, Rochester, MN, USA J. Buckner Department of Oncology, Mayo Clinic College of Medicine, Rochester, MN, USA D. H. Lachance Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA
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. Keywords Gliomatosis cerebri Malignant glioma Surgery Radiation Chemotherapy
Introduction Gliomatosis cerebri is a rare diffuse neoplastic process of the brain that
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