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Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases

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Abstract

Gliomatosis cerebri (GC) is a rare fatal glial neoplasm of the central nervous system. Neuroimaging, histological, and clinical outcome data were reviewed of 17 consecutive patients, 8 males and 9 females aged 15–68 years (median, 37 years), treated for GC between April 1992 and October 2007. All patients received cranial irradiation to include the hyperintense area on T2-weighted magnetic resonance imaging. The total dose of the radiotherapy was 50–72 Gy (median, 60). Intravenous nimustine hydrochloride was administered in all patients, combined with temozolomide in four patients. The median survival time was 23.3 months, with a median follow-up of 23.3 months. Kaplan–Meier analysis demonstrated the overall survival rate which was 70.6% for 1 year, 23.5% for 3 years, and 17.7% for 5 years. Spinal enhanced lesions and nodular malignant transformation to glioblastoma were observed during follow-up in two patients each. Poor survival showed correlation with higher Ki-67 labeling index, higher choline/N-acetylaspartate ratio on magnetic resonance spectroscopy, tumor volumes, lower Karnofsky performance status on admission, cognitive/behavioral deterioration, poor response to the initial radiochemotherapy, and emergence of paraventricular enhanced lesions during the clinical course. The prognosis for patients with GC is unfavorable, but radiochemotherapy may prolong survival.

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Authors and Affiliations

  1. Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan

    Tomoo Inoue, Toshihiro Kumabe, Masayuki Kanamori, Yukihiko Sonoda & Teiji Tominaga

  2. Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

    Mika Watanabe

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  1. Tomoo Inoue
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  2. Toshihiro Kumabe
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  3. Masayuki Kanamori
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  4. Yukihiko Sonoda
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Correspondence to Toshihiro Kumabe.

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Kazuhiro Hongo, Matsumoto, Japan

Inoue et al. should be congratulated on their nicely designed and deeply analyzed work. They reported on prognostic factors for patients with gliomatosis cerebri by analyzing their 17 consecutive cases, and showed that poor survival was correlated with higher Ki-67 labeling index, higher choline/N-acetylaspartate ratio, tumor volume, lower Karnofsky performance status on admission, cognitive/behavioral deterioration, poor response to the initial radiochemotherapy, and emergence of paraventricular enhanced lesions. They concluded that the prognosis for patients with gliomatosis cerebri was unfavorable: the median survival time was 23.3 months, the overall survival rate was 17.7% in 5 years, but, they also concluded that radiochemotherapy may prolong survival.

This paper is valuable in that 17 cases were consecutive ones, all cases were histologically verified, and adequately analyzed.

As the surgical management has apparently limitations because of the extension of the lesion into three or more lobes, radiation and/or chemotherapy should be expected to have an important role. The authors have shown that radiochemotherapy may prolong survival, even larger prospective studies are essential to establish more reliable treatment strategy.

Michel Mittelbronn, Frankfurt, Germany

In their current manuscript, Inoue et al. provide an overview about 17 consecutive gliomatosis cerebri patients diagnosed within a time period of 15 years. A special focus of this study was put on patient outcome in association with several clinico-radiological and pathological features. Their findings are consistent with the current knowledge from the literature that among other factors especially younger patient age, better Karnofsky performance status, lower histological grade of the gliomatosis as well as lower MIB-1 proliferation index were associated with a better prognosis [1, 2]. Due to its often large extention in at least three different lobes of the brain, gliomatosis cerebri is not easily accessible for neurosurgical interventions. Therefore, diagnosis of gliomatosis cerebri is usually performed by neuropathological analysis of small biopsies in conjunction with neuroradiological information. Until recently, the first-line treatment strategy consisted of radiotherapy since it has been shown in several studies that CNS irradiation is a positive prognostic factor for gliomatosis cerebri patients [3]. One of the most important considerations of Inoue et al. is their recommendation of the additional use of chemotherapeutic agents in gliomatosis cerebri cases. In their present study, Inoue et al. administered ACNU in all patients and combined it with temozolomide in the most recent patients. A very recent study of Kong et al. also revealed that additional chemotherapeutic treatment with temozolomide or nitrosurea-based substances almost doubled overall survival and tripled time to progression in gliomatosis cerebri patients in comparison to a group of patients only receiving radiotherapy [4]. A better outcome after chemotherapeutic interventions seems to be closely related to a 1p/19q codeletion in gliomatosis cerebri patients while the impact of MGMT promotor methylation is still unclear [5]. However, a median overall survival of only 24.2 months in the patient group receiving both radio- and chemotherapy still points to a very dismal prognosis of gliomatosis cerebri [4]. There are almost no targeted therapy-based approaches for the treatment of gliomatosis cerebri, a fact which might be mainly related to the still enigmatic origin of this entity. The most frequent genetic alteration in gliomatosis cerebri is a mutation in exon 7 of the TP53 gene pointing to an involvement of TP53 in the tumorigenesis of gliomatosis cerebri [6]. Other genetic defects, such as a loss of chromosomes 13q or 10q and gains of 7q, have been established as independent significant predictors of shorter survival [7]. Showing similar genetic alteration as detected in common astrocytomas or oligodendrogliomas, gliomatosis cerebri can also be regarded as a subtype of the group of diffusely infiltrating gliomas [5, 8]. The very low incidence and prevalence rates of gliomatosis cerebri demand larger multi-center studies to better determine potential origins and both diagnostic and therapeutic settings.

References

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2. Taillibert S, Chodkiewicz C, Laigle-Donadey F, Napolitano M, Cartalat-Carel S, Sanson M (2006) Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature. J Neurooncol. 76(2):201–5.

3. Perkins GH, Schomer DF, Fuller GN, Allen PK, Maor MH (2003) Gliomatosis cerebri: improved outcome with radiotherapy. Int J Radiat Oncol Biol Phys. 56(4):1137–1146.

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7. Ware ML, Hirose Y, Scheithauer BW, Yeh RF, Mayo MC, Smith JS, Chang S, Cha S, Tihan T, Feuerstein BG (2007) Genetic aberrations in gliomatosis cerebri. Neurosurgery. 60(1):150–8.

8. Braeuninger S, Schneider-Stock R, Kirches E, Powers JM, Korones DN, Mawrin C (2007) Evaluation of molecular genetic alterations associated with tumor progression in a case of gliomatosis cerebri. J Neurooncol. 82(1):23–7. Epub 2006 Sep 6.

Ghazaleh Tabatabai, Michael Weller, Zurich, Switzerland

Gliomatosis cerebri is defined by the World Health Organization as a diffusely infiltrating glioma involving at least three lobes of the brain. Inoue and colleagues present a retrospective analysis of 17 cases of gliomatosis cerebri and focus on clinical presentation, course of disease, imaging and histological parameters, aiming at defining prognostic factors. Treatment of all patients included radiotherapy and nimustine hydrochloride; 4 patients received temozolomide in addition. Of note, patients were treated over a time period of 15 years where general management standards may have been changed. The following parameters were associated with a poor outcome: high Ki67 labeling index, higher choline/N-acetyl aspartate ratio on magnetic resonance imaging spectroscopy, paraventricular lesions, and poor Karnofsky performance status at presentation. It should be taken into account, however, that this series included only 17 patients and thus statistically powered results allowing reliable conclusions cannot be expected. Nevertheless, since gliomatosis cerebri is a rare disease, this study is an interesting contribution to the field. The parameters presented here need to be further investigated in larger, prospectively studied clinical trial populations to confirm their predictive and/or prognostic value.

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Inoue, T., Kumabe, T., Kanamori, M. et al. Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases. Neurosurg Rev 34, 197–208 (2011). https://doi.org/10.1007/s10143-010-0306-1

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