Abstract：
BACKGROUND: Atypical teratoid/rhabdoid tumours (AT/RT) are uncommon but aggressive, malignant tumours in the paediatric age group. Presentation of concomitant supratentorial and infratentorial lesions in an infant is extremely rare. We discuss an infant diagnosed with such lesions. Systematic PubMed search was conducted using keywords \'atypical teratoid /rhabdoid tumor\', \'paediatric\' and \'multifocal\'. Reports were included for patients younger than 18 years with two or more lesions. The search yielded additional five cases and were tabulated. Age, sex, location, treatment given and survival/outcome were noted.
METHODS: A 10-month-old child presented with complaints of drowsiness and intractable vomiting. Imaging showed multifocal supra- and infratentorial lesions with obstructive hydrocephalus. The child underwent ventriculoperitoneal shunt followed by surgical removal of the posterior fossa lesion. Histopathological features were consistent with AT/RT.
CONCLUSIONS: Multifocal AT/RT are very rare. The impact of multifocality in the outcome is not known as very few reports are available. Newer targeted therapies may offer insight in improving outcomes in the future.
METHODS: A 10-month-old child presented with complaints of drowsiness and intractable vomiting. Imaging showed multifocal supra- and infratentorial lesions with obstructive hydrocephalus. The child underwent ventriculoperitoneal shunt followed by surgical removal of the posterior fossa lesion. Histopathological features were consistent with AT/RT.
CONCLUSIONS: Multifocal AT/RT are very rare. The impact of multifocality in the outcome is not known as very few reports are available. Newer targeted therapies may offer insight in improving outcomes in the future.
摘要：
背景：非典型畸胎样/横纹肌样瘤（AT/RT）并不常见，但具有侵袭性，儿科年龄组的恶性肿瘤。婴儿并发的幕上和幕下病变的出现极为罕见。我们讨论被诊断患有这种病变的婴儿。使用关键词“非典型畸胎瘤/横纹肌样瘤”进行系统的PubMed搜索，“儿科”和“多焦”。纳入了18岁以下有两个或两个以上病变的患者的报告。搜索产生了另外5个病例，并将其制成表格。年龄,性别,location,给予治疗并记录生存/结局.
方法：一名10个月大的儿童出现嗜睡和顽固性呕吐。影像学显示多灶性上和下病变伴梗阻性脑积水。该儿童接受了脑室腹膜分流术，然后手术切除了后颅窝病变。组织病理学特征与AT/RT一致。
结论：多焦AT/RT非常罕见。多灶性对结果的影响尚不清楚，因为可用的报告很少。较新的靶向疗法可能会在未来改善结果方面提供见解。
方法：一名10个月大的儿童出现嗜睡和顽固性呕吐。影像学显示多灶性上和下病变伴梗阻性脑积水。该儿童接受了脑室腹膜分流术，然后手术切除了后颅窝病变。组织病理学特征与AT/RT一致。
结论：多焦AT/RT非常罕见。多灶性对结果的影响尚不清楚，因为可用的报告很少。较新的靶向疗法可能会在未来改善结果方面提供见解。
