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Posterior fossa immature teratoma in an infant with trisomy 21: A case report and review of the literature

机译:婴儿21号三体症后窝未成熟畸胎瘤:一例报道并文献复习

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Background: Intracranial teratoma associated with Down syndrome is rare. With only three previously reported cases, our case is the first one presenting an immature component. Case Description: A 2-month-old boy with trisomy 21 presented with lethargy and head enlargement. A magnetic resonance imaging (MRI) study showed an obstructive hydrocephalus with 0.5 cm posterior fossa tumor compressing the cerebellum. The tumor revealed a mixed intensity on T1- and T2-weighted MRI images and was surrounded by peritumoral cysts. It was heterogeneously enhancing and showed multinodular mass. The tumor was gross totally removed via suboccipital craniotomy and histologically diagnosed as immature teratoma. Four cycles of chemotherapy consisting of cisplatin and etoposide followed the surgery. The radiotherapy was withheld due to infancy. Recurrent lesions in the tumor bed were noted 10 months later. They were removed in the second surgery and histologically identified as mature teratoma. Conclusion: Maturation of immature teratoma may be a result of natural conversion of multipotent embryonal cells into mature tissues and following chemotherapy.
机译:背景:与唐氏综合症相关的颅内畸胎瘤很少。由于只有三个先前报告的病例,我们的病例是第一个呈现不成熟成分的病例。病例描述:一个2个月大的21三三体男孩,表现出嗜睡和头部扩大。磁共振成像(MRI)研究显示阻塞性脑积水,后窝窝0.5 cm肿瘤压迫小脑。肿瘤在T1和T2加权MRI图像上显示混合强度,并被肿瘤周围的囊肿包围。异质性增强并显示多结节性肿块。通过枕下开颅手术将肿瘤完全切除,并在组织学上诊断为未成熟畸胎瘤。手术后进行了四个周期的化疗,包括顺铂和依托泊苷。由于婴儿期而停止放疗。 10个月后注意到肿瘤床上的复发性病变。他们在第二次手术中被切除,并在组织学上被鉴定为成熟的畸胎瘤。结论:未成熟畸胎瘤成熟可能是多能胚胎细胞自然转化为成熟组织并进行化学疗法的结果。

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