Ganglioglioma of conus medullaris in a patient of neurofibromatosis type 1: A novel association?

Sundar, I Vijay; Jaiswal, Manish; Purohit, Devendra; Mittal, Radhey Shyam

doi:10.4103/1793-5482.145088

Cited by 3 publications

(16 citation statements)

References 12 publications

(23 reference statements)

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“…[ 4 ] The most common location in the spinal cord is the cervical and the thoracic portions, covering 4–8 vertebral segments. [ 10 , 19 ] The mean lag time between onset of symptoms and confirming diagnosis of the tumor is 12 months. [ 10 , 19 ] There are no unique radiological features for gangliogliomas, however, MRI is very useful for diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…[ 20 ] Finally, in the fourth case, the authors described a ganglioglioma of the conus medullaris in a 16-year-old male (India, 2016). [ 19 ] The present report is the fifth documented case of the association of a spinal ganglioglioma in a patient with NF1 [ Table 1 ]. The aim of this paper is to add data to the literature regarding this rare association.…”

Section: Introductionmentioning

confidence: 97%

“…[ 14 , 21 ] The occurrence in the spinal cord is 1.1% of all spinal neoplasms. [ 19 ] The first complete pathological description of these tumors was made in 1930 by Courville. [ 10 , 19 ] They are generally more frequent in children, corresponding to 4% of CNS tumors in the pediatric population.…”

Section: Introductionmentioning

confidence: 99%

“…[ 19 ] The first complete pathological description of these tumors was made in 1930 by Courville. [ 10 , 19 ] They are generally more frequent in children, corresponding to 4% of CNS tumors in the pediatric population. [ 8 ] Seizure is the most common symptom.…”

Section: Introductionmentioning

confidence: 99%

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Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report

Cruz

Ferreira-Pinto

Brito

et al. 2021

Surgical Neurology International

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Background: Gangliogliomas are rare tumors of the central nervous system. They are usually located intracranially and rarely in the spinal cord. There is no clear correlation between this tumor and neurofibromatosis type 1 (NF1) with only four cases described. The aim of this article is to describe one more case and add data to the literature regarding this rare association. Case Description: An 8-year-old boy with NF1 presented progressive asymmetrical paraparesis (Grade 4 medical research council scale on the right leg and Grade 3 on the left leg). The cervicothoracic spinal magnetic resonance imaging demonstrated an intramedullary lesion from C4 to T4 vertebrae. The patient underwent a microsurgical resection. A partial resection was performed due to a drop in the motor evoked potential signal amplitude during dissection. Pathology report revealed a ganglioglioma (World Health Organization Grade I). Postoperatively, the patient evolved with worsening of the paraparesis. A few weeks later, he has improved his preoperative functional neurological state (better strength and gait). Adjuvant radiotherapy was not used. The patient is being followed up at the neurosurgery outpatient clinic. Conclusion: This is another case of spinal ganglioglioma associated with NF1. The tumor must be included in the differential diagnosis of patients with NF1 and spinal lesions. Complete microsurgical resection remains the standard treatment for spinal gangliogliomas, however, in this specific case, it was decided to leave a portion of the tumor to prevent neurological damage. The prognosis and treatment of this condition associated with NF1 remains to be determined.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report

Cruz

Ferreira-Pinto

Brito

et al. 2021

Surgical Neurology International

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“…Sunder et al reported a case of conus ganglioglioma who had associated neurofibromatosis type 1. 5 Sandeep et al could find 15 reported cases of ganglioglioma of the conus medullaris and added one of their own. 6 Usually the patients present with pain, paraparesis, gait ataxia, sensory disturbances, and bowel or bladder dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Ganglioglioma of Conus Medullaris—A Rare Case

Kumar

Misra

Kumar

et al. 2019

IJNS

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Gangliogliomas favor the temporal lobe. They are rarely reported in the spinal cord. Ganglioglioma of the conus medullaris is very rare. An 11-year-old boy presented with progressive weakness of bilateral lower limbs. Clinical examination and radiologic investigations revealed a lesion in the conus medullaris with an exophytic component involving the cauda equina roots. The lesion was excised near totally. Its histopathologic examination revealed it to be a ganglioglioma. Gangliogliomas of the conus medullaris are rare lesions. Subtotal or near-total excision with preservation of the function should be the aim of the surgical intervention. Because preoperative function largely dictates the postoperative course, it is advisable to intervene early at the first hint of neurologic compromise.

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Multiple Intraspinal Gangliogliomas in a Child With Neurofibromatosis Type 1: Case Report and Literature Review

Morales

Viskochil²,

Hofmann

et al. 2021

Journal of Pediatric Hematology/Oncology

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Neurofibromatosis type 1 (NF1)-associated primary intramedullary spinal cord ganglioglioma has only rarely been reported. Because of frequent nonresectability, they pose significant management challenges despite clinical indolence. This report describes a 4-year-old girl with NF1 who was found to have multiple discrete, infiltrative intramedullary cord masses, and biopsy demonstrated World Health Organization grade I ganglioglioma. Panel-based next-generation sequencing showed her previously identified germline NF1 mutation and a second somatic NF1 mutation. This represents the first report of multiple primary intramedullary gangliogliomas in a child with NF1 and demonstrates how biopsy with panel-based next-generation sequencing provides potential targets for MAPK/MEK/BRAF pathway inhibitor therapy.

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Ganglioglioma of conus medullaris in a patient of neurofibromatosis type 1: A novel association?

Cited by 3 publications

References 12 publications

Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report

Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report

Ganglioglioma of Conus Medullaris—A Rare Case

Multiple Intraspinal Gangliogliomas in a Child With Neurofibromatosis Type 1: Case Report and Literature Review

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