Adachi Masayo, Nakamura Michio, Shinozaki Natsuki, Miyazaki Tadashi
Department of Neurosurgery, Narita Red Cross Hospital.
No Shinkei Geka. 2017 May;45(5):397-404. doi: 10.11477/mf.1436203521.
We report on a case of subependymal giant cell astrocytoma(SEGA)in a patient with tuberous sclerosis(TSC)that presented with intratumoral hemorrhage and acute hydrocephalus. Initial treatment was external ventricular drainage to control the intracranial pressure;however, the tumor increased in size due to recurrent hemorrhage. Subsequently, the tumor was successfully removed via the transcortical-transventricular approach without neurological deterioration. Although intratumoral hemorrhage is extremely rare in patients with SEGA, subsequent acute hydrocephalus resulting from obstruction of the foramen of Monro will be fatal if prompt surgical treatment is not available. Careful and periodical radiographic examination of the central nervous system will be mandatory in patients with TSC, especially in those who have subependymal nodules(SEN)or SEGA around the foramen of Monro. Radical surgical removal should be considered before they become symptomatic.
我们报告一例患有结节性硬化症(TSC)的患者发生室管膜下巨细胞星形细胞瘤(SEGA),该患者出现肿瘤内出血和急性脑积水。初始治疗为进行脑室外引流以控制颅内压;然而,由于反复出血,肿瘤体积增大。随后,通过经皮质 - 经脑室入路成功切除肿瘤,且未出现神经功能恶化。虽然SEGA患者中肿瘤内出血极为罕见,但如果不及时进行手术治疗,因Monro孔阻塞导致的后续急性脑积水将是致命的。对于TSC患者,尤其是那些在Monro孔周围有室管膜下结节(SEN)或SEGA的患者,必须进行仔细且定期的中枢神经系统影像学检查。在出现症状之前应考虑进行根治性手术切除。