Noguchi N, Kanno H, Sakata K, Tokoro K, Yamamoto I
Department of Neurosurgery, School of Medicine, Yokohama City University.
No Shinkei Geka. 1997 Nov;25(11):1017-9.
We presented a case of tuberous sclerosis (TS) associated with subependymal giant cell astrocytoma (SEGC) and renal angiomyolipoma (RAML). A 19-year-old female, who had been diagnosed as TS since she was 3 months old, was admitted with complaints of headache, vomiting, and abdominal pain. At 10 years of age, a ventricular tumor was shown on CT, and at 16 years of age a ventricular peritoneal shunt was placed for obstructive hydrocephalus. On admission, an abdominal CT showed bilateral renal huge multicystic tumors with hemorrhage, which were diagnosed as RAMLs. CT and MRI showed an intraventricular tumor near the foramen of Monro, and this tumor was removed through a transcortical approach. The pathological diagnosis was SEGA. Large sized RAMLs were identified by CT. Although TS is often associated with additional tumors in the brain, heart, kidney, and other organs, the combination of SEGA and RAML is quite rare. If ventricular peritoneal shunt is placed in a TS patient, risk of shunt malfunction should be taken into account.
我们报告了一例结节性硬化症（TS）合并室管膜下巨细胞星形细胞瘤（SEGA）和肾血管平滑肌脂肪瘤（RAML）的病例。一名19岁女性，自3个月大起就被诊断为TS，因头痛、呕吐和腹痛入院。10岁时，CT显示有一个脑室肿瘤，16岁时因梗阻性脑积水进行了脑室腹腔分流术。入院时，腹部CT显示双侧肾脏有巨大的多囊性肿瘤并伴有出血，诊断为RAML。CT和MRI显示在孟氏孔附近有一个脑室内肿瘤，该肿瘤通过经皮质入路切除。病理诊断为SEGA。CT发现有大尺寸的RAML。虽然TS常与脑、心脏、肾脏和其他器官的额外肿瘤相关，但SEGA和RAML的组合相当罕见。如果在TS患者中进行脑室腹腔分流术，应考虑分流故障的风险。
