Stevens E Andrew, Stanton Constance A, Nichols Kyle, Ellis Thomas L
Department of Neurosurgery, Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina 27157, USA.
J Neurosurg Pediatr. 2009 Oct;4(4):368-71. doi: 10.3171/2009.5.PEDS0955.
The authors present the case of a rare extraventricular, intraparenchymal choroid plexus carcinoma (CPC). This 6-year-old girl presented to the emergency department with a 1-week history of headaches, nausea, and vomiting. Imaging studies revealed an intraaxial cystic and solid mass located in the right frontal lobe with central nodular enhancement and minimally enhancing cyst walls. Gross-total resection was accomplished via craniotomy without complications. The initial pathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT); however, immunostaining for INI1 protein (using the BAF47/SNF5 antibody) showed retention of nuclear staining in the tumor cells, resulting in a change in the diagnosis to CPC. There was no evidence of recurrence at the last follow-up 2.5 years after treatment, which supports the diagnosis of CPC over AT/RT. This case emphasizes the importance of immunostaining for INI1 protein for distinguishing CPC from AT/RT in cases with atypical or indeterminate features.
作者报告了一例罕见的脑室外、脑实质内脉络丛癌（CPC）病例。这名6岁女孩因头痛、恶心和呕吐1周就诊于急诊科。影像学检查显示，右侧额叶有一个轴内囊实性肿块，中央结节强化，囊壁强化轻微。通过开颅手术实现了肿瘤全切，无并发症。最初的病理诊断为非典型畸胎样/横纹肌样瘤（AT/RT）；然而，INI1蛋白免疫染色（使用BAF47/SNF5抗体）显示肿瘤细胞中核染色保留，从而将诊断改为CPC。治疗后2.5年的最后一次随访时没有复发迹象，这支持将该病例诊断为CPC而非AT/RT。该病例强调了在具有非典型或不确定特征的病例中，INI1蛋白免疫染色对于区分CPC和AT/RT的重要性。
