Jooma R, Hayward R D, Grant D N
Neurosurgery. 1984 Jan;14(1):31-41. doi: 10.1227/00006123-198401000-00008.
One hundred infants with intracranial tumors symptomatic during the 1st year of life were studied. They differed from older children in having a higher percentage of supratentorial tumors and in the fact that 90% of the tumors were of neuroectodermal origin. Vomiting, alteration of psychomotor development, and macrocrania were the most common presenting features. The "diencephalic syndrome" was seen in 5 infants, and subarachnoid hemorrhage due to tumor was diagnosed in 4. Computed tomography as the primary investigation is increasing the number of neoplasms diagnosed in this age group, although review of the skull roentgenograms in the series disclosed an abnormality in 92%. Eighty of the tumors were verified, 68 by a cranial operation and the rest at autopsy. Of the verified neoplasms, 20% were medulloblastomas, 12.5% were choroid plexus papillomas, and 10% were cerebellar astrocytomas. The cumulative average survival was 27 months but, for those who underwent a tumor operation, the average survival was 37 months. The operative mortality was 30%. Thirty-nine patients were irradiated, and this subset had a 5-year survival rate of 43%. The morbidity was high irrespective of radiotherapy; 60% of those who survived 1 year were moderately or severely disabled. Those infants receiving more than 5000 rads of whole brain radiation tended to have greater deficits in the long term. When analyzed separately, patients treated after 1970 had greatly improved mortality and morbidity rates.
对100例在1岁以内出现症状的颅内肿瘤婴儿进行了研究。他们与大龄儿童不同,幕上肿瘤的比例更高,且90%的肿瘤起源于神经外胚层。呕吐、精神运动发育改变和巨头症是最常见的临床表现。5例婴儿出现了“间脑综合征”,4例诊断为肿瘤所致的蛛网膜下腔出血。计算机断层扫描作为主要检查手段,使该年龄组诊断出的肿瘤数量有所增加,尽管该系列病例的颅骨X线片复查显示92%存在异常。80例肿瘤得到确诊,68例通过开颅手术确诊,其余在尸检时确诊。在确诊的肿瘤中,20%为髓母细胞瘤,12.5%为脉络丛乳头状瘤,10%为小脑星形细胞瘤。累积平均生存期为27个月,但接受肿瘤手术的患者平均生存期为37个月。手术死亡率为30%。39例患者接受了放疗,该亚组的5年生存率为43%。无论是否接受放疗,发病率都很高;存活1年的患者中有60%有中度或重度残疾。接受超过5000拉德全脑放疗的婴儿长期来看往往有更严重的缺陷。单独分析时,1970年以后接受治疗的患者死亡率和发病率有了显著改善。