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To describe the natural history of spinal gangliogliomas (GG) in order to determine the most appropriate neuro-oncological management. A Medline search for relevant publications up to July 2023 using the key phrase "ganglioglioma spinal" and "ganglioglioma posterior fossa" led to the retrieval of 178 studies. This corpus provided the basis for the present review. As an initial selection step, the following inclusion criteria were adopted: (i) series and case reports on spinal GG; (ii) clinical outcomes were reported specifically for GG; (iii) GG was the only pathological diagnosis for the evaluation of the tumor; (iv) papers written only in English was evaluated; and (v) papers describing each case in the series were included. The World Health Organization (WHO) 2021 grading criteria for gangliogliomas were applied. A total of 107 tumors were evaluated (63 from male patients and 44 from female patients; 1.43 male/1.0 female ratio, mean age 18.34 ± 15.84 years). The most common site was the cervical spine, accounting for 43 cases (40.18%); GTR was performed in 35 cases (32.71%) and STR in 71 cases (66.35%), while this information was not reported in 1 case (0.94%). 8 deaths were reported (7.47%) involving 2 males (25%) and 6 females (75%) aged 4-78 years (mean 34.27 ± 18.22) years. GGs located on the spine displayed the same gender ratio as these tumors in general. The most frequent symptom was pain and motor impairment, while the most prevalent location was the cervical spinal cord. GTR of the tumor posed a challenge for neurosurgeons, due to the difficulty of resecting the lesion without damaging the spinal eloquent area, explaining the lower rate of cure for this tumor type.

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INTRODUCTION: Brain tumors are a frequent cause of epilepsy in the pediatric population. The last International League Against Epilepsy (ILAE) classification of focal cortical dysplasias (FCDs) includes a subgroup consisting of tumors with surrounding dysplastic abnormalities (FCD type IIIb). Although its pathogenesis is still unclear, it has several clinical and therapeutic (surgical) implications. BACKGROUND: A number of benign tumors (such as dysembryoplastic neuroepithelial tumors and gangliogliomas) frequently present with medically refractory epilepsy associated with cortical dysplasia. In such cases, planning of surgical resection needs to take into consideration not only the tumor but also the whole area of epileptogenicity. The use of intraoperative electrocorticography recordings is reported to result in better postoperative outcomes, since they help delineate the abnormal cerebral cortex that needs to be resected to provide seizure freedom to patients. Clinical, radiological, and pathological features are also discussed herein.

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METHOD: Forty-two patients with low-grade brain tumor and refractory epilepsy were studied. The mean age was 22.3 years. They were divided into two groups: Group A, patients with ganglioglioma (n=19) and group B, patients with other low-grade tumors (n=23) (14 astrocytoma, 6 oligodendroglioma, 2 dysembryoplastic neuroepithelial tumor, and 1 xanthoastrocytoma). RESULTS: Age at seizures onset was 7 years or less in 73% of the patients in group A and in 30.4% of the patients in group B (p=0.045). Complex partial occurred frequently in group A and B (94.7% versus 82%, respectively). Seizures frequency was higher in group B (p=0.002). Computerized tomography (CT) was normal in 36.8% of group A patients and abnormal in all group B patients. Magnetic resonance imaging (MRI) was abnormal in all patients. Surgical removal was complete in 89.5% of the patients in group A and in 78.2% of the patients in group B. CONCLUSION: The association of refractory epilepsy and complex partial seizures, at a relatively low frequency, in young patients potentially normal CT and a MRI hypointense temporal lobe lesion in T1-weighed slices were habitual image findings in ganglioglioma, rather than other low-grade tumor.

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METHOD: Forty-two patients with low-grade brain tumor and refractory epilepsy were studied. The mean age was 22.3 years. They were divided into two groups: Group A, patients with ganglioglioma (n=19) and group B, patients with other low-grade tumors (n=23) (14 astrocytoma, 6 oligodendroglioma, 2 dysembryoplastic neuroepithelial tumor, and 1 xanthoastrocytoma). RESULTS: Age at seizureÆs onset was 7 years or less in 73 percent of the patients in group A and in 30.4 percent of the patients in group B (p=0.045). Complex partial occurred frequently in group A and B (94.7 percent versus 82 percent, respectively). SeizureÆs frequency was higher in group B (p=0.002).Computerized tomography (CT) was normal in 36.8 percent of group A patients and abnormal in all group B patients. Magnetic resonance imaging (MRI) was abnormal in all patients. Surgical removal was complete in 89.5 percent of the patients in group A and in 78.2 percent of the patients in group B. CONCLUSION: The association of refractory epilepsy and complex partial seizures, at a relatively low frequency, in young patients potentially normal CT and a MRI hypointense temporal lobe lesion in T1-weighed slices were habitual image findings in ganglioglioma, rather than other low-grade tumor.

MÉTODO: Foram estudados 42 pacientes com tumor cerebral primário de baixo grau e epilepsia refratária. A idade média foi 22,3 anos. Eles foram divididos em dois grupos: no grupo A os pacientes com ganglioglioma (n=19) e no grupo B os pacientes com outros tumores primários de crescimento lento (n=23) (14 astrocitomas, 6 oligodendrogliomas, 2 tumores desembrioblástico neuroepitelial e um xantoastrocitoma). RESULTADOS: A idade de início das crises convulsivas foi 7 anos ou menos em 73 por cento dos pacientes no grupo A e 30,4 por cento dos pacientes no grupo B (p=0,045). A crise convulsiva do tipo parcial complexa foi a mais identificada nos grupos A e B (94,7 por cento versus 82 por cento, respectivamente). A freqüência de crise foi mais alta no grupo B (p=0,002). A tomografia computadorizada foi normal em 36,8 por cento dos pacientes no grupo A e anormal em todos no grupo B. A ressonância magnética foi anormal em todos os pacientes. A remoção cirúrgica foi completa em 89.5 por cento dos pacientes no grupo A e 78,2 por cento no grupo B. CONCLUSÃO: A associação de epilepsia refratária e crise parcial complexa, principalmente quando a freqüência não é muito alta, em pacientes jovens, mesmo com tomografia computadorizada normal e alteração hipointensa na seqüência de T1 da ressonância magnética é sugestiva de ganglioglioma mais que outros tipos de tumor cerebral primário de baixo grau.

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We report a case of a young female patient with a rare and recently described form of brain tumor. This patient had a history of headache, hemiparesis and motor simple partial seizures. Her investigation revealed a brain tumor involving the left frontal and parietal lobes. The radiological images showed a cystic mass with multiple nodular masses and a rim of contrast enhancement extending from the right parietal cortex to the ipsilateral ventricle and corpus callosum. The patient underwent gross resection of the lesion and the histological analysis of the surgical specimen revealed a pseudopapillary structure formed by delicate vessels intermixed with a fibrillary pattern and bordered by intense astrocytic reaction with Rosenthal fibers. These features correspond to the recently described mixed neuronal-glial neoplasm, the papillary glioneuronal tumor. The patient has been followed for five years since the surgical treatment, without evidence of tumor recurrence, confirming the indolent behavior of this type of tumor.

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Relatamos o caso de uma jovem paciente com um tumor cerebral raro e recentemente descrito. Essa paciente apresentou-se com queixas de cefaléia, hemiparesia e crises epiléticas parciais simples. Sua investigação revelou um tumor cerebral envolvendo os lobos frontal e parietal esquerdos. As imagens radiológicas mostraram uma massa cística com massas nodulares múltiplas e reforço anelar de contraste estendendo do córtex parietal direito aos ventrículo lateral e corpo caloso ipsilaterais. A paciente se submeteu à ressecção do tumor e a análise histológica da lesão revelou uma estrutura pseudopapilar formada por vasos delicados entremeados com um padrão fibrilar e emoldurada por intensa reação astrocítica com fibras de Rosenthal. Essas características correspondem a uma forma de neoplasia mista glial-neuronal recentemente descrita, o tumor papilar glioneuronal. A paciente está em seguimento desde a cirurgia sem evidência de recorrência tumoral, confirmando o comportamento benigno desse tipo de tumor.

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Os gangliogliomas são neoplasias mistas, compostas de elementos gliais e neuronais, extremamente raros na região da glândula pineal. Na presente revisão da literatura foram encontrados oito casos publicados. Apresentamos o caso de paciente de 14 anos, masculino, com ganglioglioma da região da pineal, tratado cirurgicamente, com exérese total da lesão por via suboccipital transtentorial. O estudo histológico mostrou tratar-se de ganglioglioma grau I, confirmado por imuno-histoquímica. Conclui-se que tais tumores são raros e que se deve optar pela cirurgia, objetivando a exérese total. Quando isso não for possível, ou no caso de recorrência, o paciente deve ser acompanhado clínica e radiograficamente, considerando-se a radioterapia como tratamento complementar.

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