PDF(Pubmed)
Abstract：
Intracranial immature teratomas are rare, highly malignant, and fast-growing with a poor prognosis. We report the case of an infant with a large immature teratoma in the intracranial compartment. A two-month-old child presented to the emergency room with drowsiness and seizures. CT and cranial MRI showed hydrocephalus with a large expansive process in the right cerebral hemisphere extending to the infratentorial compartment, compressing the cerebellum and brainstem. It was then decided to partially resect the lesion. Postoperatively, due to the aggressiveness of the residual tumor, the patient developed complications (status epilepticus, hyperthermia, and electrolyte disorders) and died. Histopathological and immunohistochemical studies confirmed an immature teratoma. Teratomas are a subtype of germ cell tumors. Immature teratomas contain a population of cells that retain embryonic characteristics and tissues with more primitive components derived from all or some of the three germ layers (ectoderm, mesoderm, and endoderm). The prognosis of immature teratomas is associated with the degree of tumor differentiation, and those composed of undifferentiated embryonic tissues have a poor prognosis. This case report illustrates the rare and severe occurrence of a bulky immature cerebral teratoma in an infant. Unfortunately, despite undergoing a planned partial resection, the infant ended up having complications and died. Therefore, due to the size of the lesion in an infant, these cases are always complex when deciding on a surgical approach.
摘要：
颅内未成熟畸胎瘤很少见,高度恶性,并且快速增长，预后不良。我们报告了一例婴儿在颅内室有一个大的未成熟畸胎瘤。一个两个月大的孩子带着困倦和癫痫发作出现在急诊室。CT和头颅MRI显示脑积水，右脑半球有一个大的扩张过程，延伸到幕下室，压缩小脑和脑干.然后决定部分切除病变。术后,由于残留肿瘤的侵袭性，患者出现并发症（癫痫持续状态，热疗,和电解质紊乱）并死亡。组织病理学和免疫组织化学研究证实未成熟畸胎瘤。畸胎瘤是生殖细胞肿瘤的一种亚型。未成熟的畸胎瘤包含保留胚胎特征的细胞群和组织，其更原始的成分来自三个胚层中的全部或部分（外胚层，中胚层,和内胚层）。未成熟畸胎瘤的预后与肿瘤分化程度有关,那些由未分化的胚胎组织组成的预后较差。此病例报告说明了婴儿中大量未成熟脑畸胎瘤的罕见和严重发生。不幸的是,尽管进行了计划中的部分切除,婴儿最终出现并发症并死亡。因此,由于婴儿病变的大小，在决定手术方法时，这些病例总是很复杂。
