Butterfly glioma

 

Butterfly glioma is a high grade astrocytoma, usually a glioblastoma (WHO grade IV), which crosses the midline via the corpus callosum. Other white matter commissures are also occasionally involved. The term butterfly refers to the symmetric wing like extensions across the midline.

Epidemiology

Most frequently butterfly gliomas occur in the frontal lobes, crossing via the genu of the corpus callosum, however posterior butterflies are also encountered.

Differential diagnosis

Primary central nervous system lymphoma: especially in AIDS patients

cerebral toxoplasmosis: especially in AIDS patients

tumefactive demyelination

cerebral metastases (rare)

occasionally a leptomeningeal process which fills the quadrigeminal and ambient cisterns can cause confusion.

A meningioma can mimic butterfly glioma when it arises from the falx cerebri and crosses the midline. Presence of a cerebrospinal fluid intensity cleft between the tumour and adjacent brain cortex is a useful sign to identify the extra-axial location of these lesions and differentiate them from butterfly gliomas 1).

Treatment

Gliomas invading the anterior corpus callosum are commonly deemed unresectable due to an unacceptable risk/benefit ratio, including the risk of abulia.

Burks et al. in a study presents evidence that anterior butterfly gliomas can be safely removed using a novel, attention-task based, awake brain surgery technique that focuses on preserving the anatomical connectivity of the cingulum and relevant aspects of the cingulate gyrus 2).

Current management options include biopsy only, followed by radiation and chemotherapy; surgical decompression followed by radiation and chemotherapy; or biopsy followed by palliative measures (comfort care). Management decisions are subjective, based upon physician experience and/or patient/family preferences in light of the prognosis of this disease.

Outcome

The prognosis of glioblastoma multiforme (GBM) is poor even with aggressive first-line therapy, which includes surgery, radiation therapy, and adjuvant chemotherapy. Although the ideal course of treatment for elderly patients with newly diagnosed GBM is still undecided and requires further studies, the new chemotherapeutic agents administered with or without concomitant radiation therapy have shown promising results. However, in our setting, where resources are limited and newer treatment options are expensive, it is often difficult to deliver the best care to the patient 3).

Case series

2017

Burks et al. reviewed clinical data on all patients undergoing glioma surgery performed by the senior author during a 4-year period at the University of Oklahoma Health Sciences Center. Forty patients were identified who underwent surgery for butterfly gliomas. Each patient was designated as having undergone surgery either with or without the use of awake subcortical mapping and preservation of the cingulum. Data recorded on these patients included the incidence of abulia/akinetic mutism. In the context of the study findings, the authors conducted a detailed anatomical study of the cingulum and its role within the DMN using postmortem fiber tract dissections of 10 cerebral hemispheres and in vivo diffusion tractography of 10 healthy subjects.

Forty patients with butterfly gliomas were treated, 25 (62%) with standard surgical methods and 15 (38%) with awake subcortical mapping and preservation of the cingulum. One patient (1/15, 7%) experienced postoperative abulia following surgery with the cingulum-sparing technique. Greater than 90% resection was achieved in 13/15 (87%) of these patients.

This study presents evidence that anterior butterfly gliomas can be safely removed using a novel, attention-task based, awake brain surgery technique that focuses on preserving the anatomical connectivity of the cingulum and relevant aspects of the cingulate gyrus 4).

2014

Of 336 patients with newly diagnosed GBM who were operated on, 48 (14 %) presented with bGBM, where 29 (60 %) and 19 (40 %) underwent surgical resection and biopsy, respectively. In multivariate analysis, a bGBM was independently associated with poorer survival [HR (95 % CI) 1.848 (1.250-2.685), p < 0.003]. In matched-pair analysis, patients who underwent surgical resection had improved median survival than biopsy patients (7.0 vs. 3.5 months, p = 0.03). In multivariate analysis, increasing percent resection [HR (95 % CI) 0.987 (0.977-0.997), p = 0.01], radiation [HR (95 % CI) 0.431 (0.225-0.812), p = 0.009], and temozolomide [HR (95 % CI) 0.413 (0.212-0. 784), p = 0.007] were each independently associated with prolonged survival among patients with bGBM. This present study shows that while patients with bGBM have poorer prognoses compared to non-bGBM, these patients can also benefit from aggressive treatments including debulking surgery, maximal safe surgical resection, temozolomide chemotherapy, and radiation therapy 5).

2011

Median age was 59 years; 52 % were female; median preoperative Karnofsky performance score (KPS) was 80. Twelve patients underwent biopsy and eleven underwent surgical decompression. The median tumor volume for the biopsy group was 60.6 cm(3) and for the surgically decompressed group 40.5 cm(3). In the biopsy group, five patients received adjuvant therapy but one died prior to its completion; two died prior to the initiation of adjuvant therapy and five were lost to follow up. In the surgical decompression group, seven patients received adjuvant therapy, one died prior to the initiation of adjuvant therapy, two were treated with palliative measures only, and one was lost to follow up. Kaplan-Meier estimates of overall median post surgical-survival of the whole group was 180 days, the biopsy group 48 days, and the surgically decompressed group 265 days (p = 0.14). Our results show that there was a higher median survival in the surgically decompressed group; but a direct correlation could not be established, and that the median KPS did not improve in either group after treatment. A larger multicenter review is required to quantitatively assess the factors, including tumor biomarkers that are associated with patient outcome 6).

Case reports

A 54-year-old man presented with change in behaviour, nocturnal enuresis, abnormal limb movement and headache of one week’s duration. The diagnosis of butterfly glioma (glioblastoma multiforme) was made based on imaging characteristics and was further confirmed by biopsy findings. As the corpus callosum is usually resistant to infiltration by tumours, a mass that involves and crosses the corpus callosum is suggestive of an aggressive neoplasm 7).

1)

Watts J, Box G, Galvin A, et al. Magnetic resonance imaging of meningiomas: a pictorial review. Insights Imaging. 2014;5:113–22.
2) , 4)

Burks JD, Bonney PA, Conner AK, Glenn CA, Briggs RG, Battiste JD, McCoy T, O’Donoghue DL, Wu DH, Sughrue ME. A method for safelyresecting anterior butterfly gliomas: the surgical anatomy of the default mode network and the relevance of its preservation. J Neurosurg. 2017 Jun;126(6):1795-1811. doi: 10.3171/2016.5.JNS153006. Epub 2016 Sep 16. PubMed PMID: 27636183.
3)

Agrawal A. Butterfly glioma of the corpus callosum. J Cancer Res Ther. 2009 Jan-Mar;5(1):43-5. PubMed PMID: 19293489.
5)

Chaichana KL, Jusue-Torres I, Lemos AM, Gokaslan A, Cabrera-Aldana EE, Ashary A, Olivi A, Quinones-Hinojosa A. The butterfly effect on glioblastoma: is volumetric extent of resection more effective than biopsy for these tumors? J Neurooncol. 2014 Dec;120(3):625-34. doi: 10.1007/s11060-014-1597-9. Epub 2014 Sep 6. PubMed PMID: 25193022; PubMed Central PMCID: PMC4313925.
6)

Dziurzynski K, Blas-Boria D, Suki D, Cahill DP, Prabhu SS, Puduvalli V, Levine N. Butterfly glioblastomas: a retrospective review and qualitative assessment of outcomes. J Neurooncol. 2012 Sep;109(3):555-63. doi: 10.1007/s11060-012-0926-0. Epub 2012 Jul 18. PubMed PMID: 22806339; PubMed Central PMCID: PMC3992290.
7)

Krishnan V, Lim TC, Ho FC, Peh WC. Clinics in diagnostic imaging (175). Corpus callosum glioblastoma multiforme (GBM): butterfly glioma. Singapore Med J. 2017 Mar;58(3):121-125. doi: 10.11622/smedj.2017017. PubMed PMID: 28361164; PubMed Central PMCID: PMC5360865.

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