Hou Lewis C, Bababeygy Simon R, Sarkissian Vahe, Fisher Paul G, Vogel Hannes, Barnes Patrick, Huhn Stephen L
Department of Neurosurgery, Stanford University School of Medicine, Stanford, Calif. 94305-5327, USA.
Pediatr Neurosurg. 2008;44(4):304-12. doi: 10.1159/000134922. Epub 2008 May 27.
Congenital glioblastoma multiforme is a rare primary brain tumor that has a unique biology distinct from pediatric and adult variants. In this report, we present a case of congenital glioblastoma with complicated management course. A literature review of previously reported cases is included to illustrate the epidemiology and natural history of this disease. A 9-month-old male infant developed acute lethargy, hemiparesis and unilaterally dilated pupil. Imaging studies revealed a large hemispheric tumor, resulting in significant midline shift suggestive of impending herniation. Emergent tumor cystic fluid drainage was performed at initial presentation. A frontotemporoparietal craniotomy was performed on the following day to attempt a gross total resection. Adjuvant chemotherapy consisting of oral temozolomide was administered. The patient eventually succumbed 4 months later due to aggressive tumor progression. Congenital glioblastoma should be included in the differential diagnosis of infants with large intracranial tumors. Although surgical intervention may increase survival, the overall outcome remains poor despite maximal multimodal treatment.
先天性多形性胶质母细胞瘤是一种罕见的原发性脑肿瘤，具有与儿童及成人型不同的独特生物学特性。在本报告中，我们呈现了一例先天性胶质母细胞瘤，其治疗过程复杂。我们还纳入了对既往报道病例的文献综述，以阐明该疾病的流行病学及自然史。一名9个月大的男婴出现急性嗜睡、偏瘫及单侧瞳孔散大。影像学检查显示一个巨大的半球形肿瘤，导致明显的中线移位，提示即将发生脑疝。初次就诊时即进行了紧急肿瘤囊液引流。次日行额颞顶开颅术，试图进行肿瘤全切除。给予口服替莫唑胺辅助化疗。该患者最终因肿瘤进展迅速于4个月后死亡。先天性胶质母细胞瘤应纳入颅内巨大肿瘤婴儿的鉴别诊断。尽管手术干预可能会提高生存率，但即便进行了最大程度的多模式治疗，总体预后仍然很差。
