Ganglioneuroblastoma of the cerebellum: neuroimaging and pathological features of a case

Rationale:Intracranial ganglioneuroblastoma represents a rare subtype of primitive neuroectodermal tumor. Here, we report a hippocampal ganglioneuroblastoma and a literature review of cerebral anglioneuroblastoma is carried out.Patient concerns:We report a 16-year-old male patient presenting with absence seizure and high-infiltration hippocampal ganglioneuroblastoma.Interventions:Magnetic resonance imaging (MRI) indicates a space-occupying lesion with a well-defined margin in the right temporal lobe and hippocampus. However, hyper-signal on flair and diffusion-weighted imaging (DWI) with a low apparent diffusion coefficient (ADC) value is detected, which prompts high tumoral invasiveness.Interventions:A total resection of tumor and subsequent chemotherapy combing with radiotherapy is performed.Outcomes:For a follow-up period of 60 months, no evidence of recurrence and further seizures are detected.Lessons:High-infiltration hippocampal ganglioneuroblastoma is a rare event. MRI examination often showed features of low-grade gliomas, while hyper-signal lesion on DWI with a low ADC value can be detected. Complete resection combined with fractionated radiotherapy and chemotherapy was the optimal treatment for cerebral ganglioneuroblastoma.

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“…It was reported that the longest asymptomatic period of the patients with intracranial GNB is 60 months following the above treatment. [ 11 ]…”

Section: Discussionmentioning

confidence: 99%

Adult hippocampal ganglioneuroblastoma

Yao

Chen²,

Shang-Guan³

et al. 2017

Medicine

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“…This explains that patients with this subtype are likely to be offered surgery and benefit from surgical excision with overall better survival. Ganglioneuroblastoma typically has a high invasive behaviour but slower multiplication rate and the asymptomatic period of up to 60 months has been reported after surgical excision [35,36].…”

Section: Management Optionsmentioning

confidence: 99%

Primary Central Nervous System Neuroblastoma: An Enigmatic Entity

Mishra¹,

Agrawal²

2021

Pheochromocytoma, Paraganglioma and Neuroblastoma

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Neuroblastoma is one of the most common solid tumour in the paediatric age group. Central nervous system (CNS) involvement in neuroblastoma is commonly due to metastasis from the extracranial primary. Primary CNS Neuroblastoma (PCNS-NB) is a rare entity and highlights errors in development of neural crest cells and CNS. A lot has been published since the first description of PCNS-NB four decades ago. Over the years, neuroscientists, geneticists, and clinicians have improved the understanding of PCNS-NB. PCNS-NB is an enigmatic entity with variable presentation, epidemiology, clinical features and outcomes. Recent update in knowledge is seen in 2016 WHO classification of CNS tumours with reclassification of CNS neuroblastoma. It further subclassified different histological variants of PCNS-NB and its molecular correlates. Most common histological subtype of PCNS-NB is neuroblastoma followed by ganglioneuroblastoma. Studies support the view that younger age group, less number of lesions, ganglioneuroblastoma histology subtype and surgical management are good prognostic indicators. This chapter provides an updated overview of epidemiology, clinical features, histological and molecular diagnosis, and outcomes of PCNS-NB in addition to the role of adjuvant therapy.

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“…Upon reviewing the literature for pediatric cases of primary intracranial ganglioneuroblastomas, we identified 10 patients reported in the English and French literature [2][3][4][5][6][7]. Reported intracranial tumors most commonly arise in the parietal lobe and cerebellum with symptoms of increased intracranial pressure; only one case involved the occipital lobe [2].…”

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confidence: 99%

“…With this treatment combination, the patient had a favorable clinical course without tumor recurrence for more than 60 months. Survivals were reported in three of the 10 previously reported patients at 7 months, 26 months, and 36 months [5][6][7]. The range of survival after initial symptoms was 0.7-36 months (median 11 months), which is substantially less than the 60% 3 year survival in pediatric patients with cerebral neuroblastomas [8].…”

mentioning

confidence: 99%