Ganglioglioma of the Spinal Cord

Oppenheimer, Daniel C.; Johnson, Mahlon D.; Judkins, Alexander R.

doi:10.4103/2156-7514.166355

Cited by 16 publications

(6 citation statements)

References 9 publications

(12 reference statements)

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“…Patients usually present with a long history of epileptic seizures, that do not typically respond to antiepileptic medicine, especially when the tumor involves the neocortex or temporal lobe. Other uncommonly encountered symptoms range from headache, nausea, visual disturbances, and fatigue – if situated in the hypothalamus or third ventricle [ 34 ] – to progressive myelopathy, motor and sensory defects, gait abnormalities and bowel or bladder dysfunction – if situated in the spinal cord [ 33 , 35 ].…”

Section: ⧉ Discussionmentioning

confidence: 99%

Practical aspects regarding the histopathological grading and anaplastic transformation of gangliogliomas – a literature review

Lisievici

Lisievici²,

Pasov³

et al. 2022

Rom J Morphol Embryol

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Ganglioglioma represents a benign central nervous tumor, occurring predominantly in the pediatric population and affecting the temporal lobe. It is also renowned for its epileptogenic potential. However, to date, there are numerous uncertain features about this tumor, especially about its grading system. In the former World Health Organization (WHO) Classification of central nervous tumors system, gangliogliomas could have been attributed one out of three grades: grade I (benign), grade II (atypical), and grade III (anaplastic). The new classification systems have renounced to atypical ganglioglioma nomenclature, due to the lack of histopathological criteria for this entity. Another controversial aspect of grade I ganglioglioma is its potential to transform into a malignant tumor, namely, most frequently an anaplastic ganglioglioma. Based on our knowledge, there are no literature reviews to date focusing on anaplastic transformation potential. The present paper encompasses all anaplastic transformation of gangliogliomas and has analyzed the time frame between the two events, the age of the patients and its relationship to the complete or subtotal resection and administration of radiotherapy. Thirty-three cases of malignant transformation of ganglioglioma have been reported so far in the literature, with 54.54% of them undergoing progression to anaplastic ganglioglioma and 21.21% to anaplastic ganglioglioma. Median age was 26 years, and the cases were evenly distributed between the two genres. Only 27.27% of all evaluated cases had been administrated adjuvant radiotherapy, and only 44% of the latter have had an incomplete tumoral resection.

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Section: ⧉ Discussionmentioning

confidence: 99%

Practical aspects regarding the histopathological grading and anaplastic transformation of gangliogliomas – a literature review

Lisievici

Lisievici²,

Pasov³

et al. 2022

Rom J Morphol Embryol

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“…The first description of ganglioglioma was detected in 1870 by Loretz and further studied in 1926 by Perkins. Ganglioglioma is a rare tumor of the central nervous system accounting for 1–1.5% of all spinal tumors (4, 40, 41). Gross total resection is the most reliable treatment (10, 42).…”

Section: Discussionmentioning

confidence: 99%

Prolonged Response Induced by Single Agent Vemurafenib in a BRAF V600E Spinal Ganglioglioma: A Case Report and Review of the Literature

et al. 2019

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Spinal ganglioglioma is a rare low-grade, slow-growing tumor of the central nervous system affecting mostly children and young adults. After surgery, some patients show tumor recurrence and/or malignant transformation. Gangliogliomas harbor molecular deficiencies such as mutations in the B-rapidly accelerated fibrosarcoma ( BRAF ) gene, resulting in activation of a downstream signaling pathway and cancer development. Vemurafenib is a BRAF inhibitor used to treat patients with BRAF V600E -mutated cancer. Although a few studies have reported the clinical responses in gangliogliomas, the sequence and duration of treatment have not been established. We describe a case of an adult with a progressive BRAF V600E mutant spinal cord ganglioglioma 9 years after surgery who was treated with vemurafenib. This treatment resulted in a partial response within 2 months, which was sustained for more than a year. The patient then decided to stop treatment because of side effects. Despite this decision, the tumor showed no sign of progression 21 months after treatment discontinuation. This is the first reported case of a response to vemurafenib in an adult with progressive spinal cord BRAF V600E -mutated ganglioglioma which was sustained after treatment discontinuation.

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“…Spinal gangliogliomas are a slow growing glial tumor that accounts for 1% of IMSCTs [ 48 ]. The lesions are rare with approximately only 70 cases reported in literature [ 71 ]. On MRI, gangliogliomas appear as a well circumscribed mass that is hypointense on T1 and hyperintense on T2 with tumoral cysts as a common finding.…”

Section: Intramedullary Tumorsmentioning

confidence: 99%

Characterization and Treatment of Spinal Tumors

2022

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The prevalence of spinal tumors is rare in comparison to brain tumors which encompass most central nervous system tumors. Tumors of the spine can be divided into primary and metastatic tumors with the latter being the most common presentation. Primary tumors are subdivided based on their location on the spinal column and in the spinal cord into intramedullary, intradural extramedullary, and primary bone tumors. Back pain is a common presentation in spine cancer patients; however, other radicular pain may be present. Magnetic resonance imaging (MRI) is the imaging modality of choice for intradural extramedullary and intramedullary tumors. Plain radiographs are used in the initial diagnosis of primary bone tumors while Computed tomography (CT) and MRI may often be necessary for further characterization. Complete surgical resection is the treatment of choice for spinal tumors and may be curative for well circumscribed lesions. However, intralesional resection along with adjuvant radiation and chemotherapy can be indicated for patients that would experience increased morbidity from damage to nearby neurological structures caused by resection with wide margins. Even with the current treatment options, the prognosis for aggressive spinal cancer remains poor. Advances in novel treatments including molecular targeting, immunotherapy and stem cell therapy provide the potential for greater control of malignant and metastatic tumors of the spine.

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Ganglioglioma of the Spinal Cord

Cited by 16 publications

References 9 publications

Practical aspects regarding the histopathological grading and anaplastic transformation of gangliogliomas – a literature review

Practical aspects regarding the histopathological grading and anaplastic transformation of gangliogliomas – a literature review

Prolonged Response Induced by Single Agent Vemurafenib in a BRAF V600E Spinal Ganglioglioma: A Case Report and Review of the Literature

Characterization and Treatment of Spinal Tumors

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