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ganglioglioma

Ganglioglioma

Ganglioglioma is a tumor of neuroepithelial tissue that arises from ganglion cells in the central nervous system.

Gangliogliomas are mixed tumors which contain both glial and neuronal elements.

Gangliogliomas and ganglioneuromas are slow growing benign tumors.

Advances in the immunohistochemical detection of neuron-specific and neuronal-associated antigens have resulted in the discovery of neuronal elements in certain primary human brain tumors. The results have been not only to expand what neuropathologists commonly recognize as gangliogliomas, including the tumors now known as glioneurocytic tumor with neuropil rosettes and papillary ganglioneuroma, but also to expand the spectrum of tumor types to now include tumors such as central neurocytoma, dysembryoplastic neuroepithelial tumor, and desmoplastic infantile ganglioglioma.

see Optic pathway ganglioglioma.

see Spinal cord ganglioglioma.

see Third ventricle ganglioglioma

Epidemiology

Mostly affect children and young adults.

Low-grade gangliogliomas/gangliocytomas (GGs) are rare tumors

Because of their rarity, large-scale, population-based studies focusing on epidemiology and outcomes are lacking.

348 children with low-grade GGs diagnosed from 2004 to 2010, with a median follow-up of 37 months. Tumors were more prevalent in males (n = 208, 59.8%) than females (n = 140, 40.2%) (P < .001). Almost 63% occurred in children >10 years, whereas only 3.5% were found in those <1 year old. Approximately 50% were located in the temporal lobes, and only 3.7% and 3.5% were located in the brainstem and spinal cord, respectively. Surgery was performed on 91.6% of cases, with gross total resection achieved in 68.3%. Radiation was used in 3.2%. Young age (<1 year) and brainstem location were associated with worse overall survival.

Low-grade GGs occur in older children with a male preference 1).

Treatment

Gross total resection is achieved in the majority of cases, and radiation is rarely used.

Prognosis

Although the majority of patients have an excellent prognosis, infants and patients with brainstem tumors have worse survival rates.

Case series

2016

27 patients with drug resistant epilepsy and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92.5% and 90.4% were seizure-free in the first and fifth year after surgery, respectively. Twelve of 16 children were successful in becoming drug-free, with complete withdrawal by 3.2 years. Surgery proved to be potentially curative and safe in these cases, suggesting that the tumor diagnosis and surgery cannot be postponed 2).


Thirty-seven children were identified, with a median age at presentation of 8.2 years and median follow-up of 38.0 months. Eighteen tumors (48.6%) were typical and 19 (51.4%) were atypical. All typical lesions presented with seizures, whereas no atypical lesions did so. Sixteen (88.9%) typical lesions were located in the temporal lobe. In the atypical group, tumor location was variable, including 11 (57.9%) in the brainstem. Death during follow-up was statistically more common in the atypical group (31.6% vs 0%, p = 0.02). Gross-total resection (GTR) was achieved for 15 of 16 typical tumors (93.8%), compared with 3 atypical tumors (15.8%, p < 0.0001). Presentation with seizure or non-brainstem location were each associated with survival (p = 0.02 and 0.004, respectively). The presence of mutation in BRAF exon 15 did not differ between the 2 groups. Pediatric GG with typical imaging features is associated with excellent rates of GTR and overall survival. Atypical GG is commonly encountered, less amenable to GTR, and associated with a worse outcome. This may relate to anatomical or biological characteristics and merits further investigation 3).

1)
Dudley RW, Torok MR, Gallegos DR, Mulcahy-Levy JM, Hoffman LM, Liu AK, Handler MH, Hankinson TC. Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. Neurosurgery. 2015 Mar;76(3):313-20. doi: 10.1227/NEU.0000000000000619. PubMed PMID: 25603107; PubMed Central PMCID: PMC4333003.
2)
Bernardino MR, Funayama C, Hamad AP, Machado H, Sakamoto A, Thome U, Terra VC, Santos AC. Refractory epilepsy in children with brain tumors. The urgency of neurosurgery. Arq Neuropsiquiatr. 2016 Dec;74(12):1008-1013. doi: 10.1590/0004-282×20160157. PubMed PMID: 27992000.
3)
Patibandla MR, Ridder T, Dorris K, Torok MR, Liu AK, Handler MH, Stence NV, Fenton LZ, Hankinson TC. Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course. J Neurosurg Pediatr. 2016 Jan;17(1):41-8. doi: 10.3171/2015.6.PEDS15215. Epub 2015 Oct 2. PubMed PMID: 26431248.
ganglioglioma.txt · Last modified: 2017/06/19 15:12 by administrador