Department of Medical Imaging, Perth Children's Hospital, Nedlands, Perth, WA, 6009, Australia.
PathWest Neuropathology, Royal Perth Hospital, Perth, WA, 6000, Australia.
Childs Nerv Syst. 2023 Apr;39(4):857-861. doi: 10.1007/s00381-023-05836-0. Epub 2023 Jan 20.
Although the posterior fossa is a common location for paediatric brain tumours [1], diffuse glioma isolated to the cerebellum is an extremely rare imaging entity. Only two cases of isolated diffuse paediatric cerebellar glioma have been reported in the English language to the best of our knowledge [2, 3], and only one of these cases had a similar imaging phenotype to our cases [3]. Although somewhat similar to Lhermitte-Duclos (dysplastic gangliocytoma of the cerebellum), the appearances are distinct from other neoplastic entities of the paediatric posterior fossa. Clinical presentation and neurological examination findings are vital however to help differentiate other diffuse pathologies involving the cerebellum such as rhombencephalitis. Presented here are two diffuse cerebellar gliomas in children under the age of 3 with near identical imaging phenotypes demonstrating differing histological and molecular genetic profiles.
尽管后颅窝是小儿脑瘤的常见部位[1]，但孤立性小脑弥漫性胶质瘤在影像学上极为罕见。据我们所知，英文文献中仅报道过两例孤立性小儿小脑弥漫性胶质瘤[2,3]，而且只有一例与我们的病例具有相似的影像学表现[3]。尽管与 Lhermitte-Duclos 病（小脑发育不良性神经节细胞瘤）有些相似，但这些表现与小儿后颅窝的其他肿瘤实体明显不同。然而，临床表现和神经系统检查结果对于帮助鉴别累及小脑的其他弥漫性病变（如脑脊髓炎）非常重要。本文介绍了 2 例年龄均小于 3 岁的小儿弥漫性小脑胶质瘤，其影像学表现几乎完全相同，但组织学和分子遗传学特征不同。
