Butterfly glioblastoma: Clinical characteristics, treatment strategies and outcomes in a population-based cohort

Abstract

Background Butterfly glioblastoma is a rare subgroup of glioblastoma with a bihemispheric tumor crossing the corpus callosum, and is associated with a dismal prognosis. Prognostic factors are previously sparsely described and optimal treatment remain uncertain. We aimed to analyse clinical characteristics, treatment strategies and outcome from butterfly glioblastoma in a real-world setting. Methods This retrospective population-based cohort study included patients diagnosed with butterfly glioblastoma in Western Norway between 01/01/2007 and 31/12/2014. We enrolled patients with histologically confirmed glioblastoma and patients with a diagnosis based on typical MRI pattern. Clinical data was extracted from electronic medical records. Molecular and MRI volumetric analyses were retrospectively performed. Survival analyses were performed using Kaplan-Meier method and Cox proportional hazards regression models. Results Among 381 patients diagnosed with glioblastoma, 33 patients (8.7%) met the butterfly glioblastoma criteria. Median overall survival was 5.5 months (95% CI 3.1-7.9) and three-year survival was 9.1%. Hypofractionated radiation therapy with or without temozolomide was the most frequently used treatment strategy, given to 16 of the 27 (59.3%) patients receiving radiation therapy. Best supportive care was associated with poorer survival compared to multimodal treatment (adjusted hazard ratio 5.11 (95%CI 1.09-23.89)). Conclusion Outcome from butterfly glioblastoma was dismal, with a median overall survival of less than six months. However, long-term survival was comparable to that observed in non-butterfly glioblastoma, and multimodal treatment was associated with longer survival. This suggests that patients with butterfly glioblastoma may benefit from a more aggressive treatment approach despite the overall poor prognosis.

The impact of surgery on butterfly gliomas - A real-life study of biopsy vs. surgical resection

“…Despite technological advances, established and described techniques for resection of these tumors, and evidence favorable to this strategy, surgery is still little used in the primary treatment of BG [4,9,11]. This is possibly due to the poor results reported in the past and the lack of know-how and expertise of neurosurgeons in this type of intervention.…”

“…Gliomas are one of the most common primary neoplasm of the central nervous system (CNS) (25,5% of cases) and the most common malignant brain tumor in the adult population (80,8% of cases) [1]. A growth pattern involving both radial hemispheres and the corpus callosum, termed butter y glioma (bG), is usually associated with poor prognosis, short survival, di cult management, cognitive impairment, and functional rapid movement [2][3][4][5]. Despite the histology most commonly seen to be glioblastoma (GB), this growth pattern can also be seen in low-grade gliomas and in lymphoproliferative disorders.…”

“…The role of cytoreduction as a primary treatment strategy for bB is still controversial, particularly in cases of butter y Glioblastomas (bGB) [2][3][4][5][6][7][8][9][10][11]. The involvement of eloquent areas and long bers of critical association intervention, limit its approval and signi cantly increase health morbidity and mortality [12].…”

Bilateral Laser Interstitial Thermal Therapy for Butterfly Gliomas Compared With Needle Biopsy: A Preliminary Survival Study

“…The subset of bGBM has been proven to have worse outcomes as compared with other GBMs but can be improved with cytoreduction. 1,[20][21][22] A review of National Cancer Database by Baoro et al showed that surgical resection improved the OS to 11.5 months as compared with 6.5 months with a biopsy alone. 21 However, a meta-analysis by Chawla et al 22 noted a significantly high rate of complications with surgery as opposed to biopsy (28% vs 13%; P < .01).…”

Glioblastoma initiation, migration, and cell types are regulated by core bHLH transcription factors ASCL1 and OLIG2

“…Glioblastoma (GBM) is the most common and malignant form of glioma, with a median survival below 18 months and a five-year survival rate of less than 10% 1 . A major challenge in the treatment of GBM is that by the time of diagnosis, highly infiltrative tumor cells have already migrated across long distances on major white matter tracts and/or the microvasculature to invade surrounding brain regions 2 . Thus, despite the use of fluorescence guided surgery for maximal resection of tumor tissues followed by concurrent chemotherapy with temozolomide (TMZ) and conformal radiation [3][4][5] , recurrence is unavoidable since elimination of all residual cells is not possible.…”