[Malignant tumors of the cerebral hemisphere in children. A series of 64 cases].
[Malignant tumors of the cerebral hemisphere in children. A series of 64 cases].
复制标题
[儿童大脑半球恶性肿瘤。
DOI：
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复制
发表时间：
1993
期刊：
影响因子：
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通讯作者：
J. Hirsch
中科院分区：
文献类型：
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作者：
E. Hoppe;J. Hirsch
背景
儿童大脑半球肿瘤约占所有中枢神经系统肿瘤的15%。其中30%~ 40%为恶性，预后差。
患者和方法
本文分析了1970 ~ 1989年收治的64例（男30例，女34例）15岁以下儿童大脑半球恶性肿瘤的临床资料。星形细胞瘤27例，室管膜瘤20例，少突胶质母细胞瘤7例，肉瘤6例，神经母细胞瘤2例，髓上皮瘤1例，畸胎瘤1例。肿瘤位于额叶18例，颞叶9例，顶叶8例，枕叶2例。27例肿瘤侵犯2个或多个肺叶。肿瘤切除58例（完全切除36例）。42名儿童接受了放射治疗，其中18名还接受了化疗。
结果
20%的病例从首次临床表现到确诊的时间超过6个月。56%的患者在发病时出现颅内压升高的症状，73%的患者在入院时出现颅内压升高的症状。只有30%的病例出现惊厥。9名儿童在术后第一个月死亡。5年的精算生存率为39 +/- 13%，10年为29 +/- 13%。室管膜瘤的这一比率（5年时为48 +/- 23%，10年时为41 +/- 23%）优于星形细胞瘤（25 +/- 18%和13 +/- 16%）。27例星形细胞瘤中有3例发生在6至7年前因急性淋巴细胞白血病接受治疗的儿童身上。一名婴儿在星形细胞瘤手术和化疗后2年发生急性淋巴细胞白血病，一名患者在5个月大时接受视网膜母细胞瘤治疗，13年后发生星形细胞瘤。
结论
颅内压增高是大脑半球恶性肿瘤较惊厥更常见的首发症状。首次表现与诊断之间的时间间隔较长，提示20%的病例为良性肿瘤变性。第二个恶性肿瘤的发展在儿童中并不例外。室管膜瘤的预后比星形细胞瘤好，这表明无论室管膜瘤的级别如何，都应该切除和放疗。
BACKGROUND
Cerebral hemisphere tumors in children represent about 15% of all tumors of central nervous system. 30 to 40% of them are malignant and have a poor prognosis.
PATIENTS AND METHODS
The files of 64 children (30 boys and 34 girls, less than 15 years old) treated between 1970 and 1989 for malignant cerebral hemisphere tumor were analysed. There were 27 astrocytomas, 20 ependymomas, 7 oligodendroblastomas, 6 sarcomas, 2 neuroblastomas, 1 medulloepithelioma and 1 teratoma. The tumor was located in the frontal area in 18 patients; it was temporal in 9, parietal in 8 and occipital in 2 patients. The tumor infiltrated 2 or several lobes in 27 children. The tumor was excised in 58 patients (completely in 36). Radiotherapy was given in 42 children; 18 of them also received chemotherapy.
RESULTS
The time between the first clinical manifestation and diagnosis was longer than 6 months in 20% of cases. Symptoms of increased intracranial pressure were present at onset of the disease in 56% of patients and at admission in 73%. Convulsions revealed the tumor in only 30% of cases. Nine children died during the first post-operative month. The actuarial survival rate was 39 +/- 13% at 5 years and 29 +/- 13% at 10 years. This rate was better for ependymomas (48 +/- 23% at 5 years and 41 +/- 23% at 10 years) than for astrocytomas (25 +/- 18% and 13 +/- 16%). Three of the 27 astrocytomas developed in children who had been treated 6 to 7 years earlier for acute lymphoblastic leukemia. One infant developed acute lymphoblastic leukemia 2 years after surgery and chemotherapy for astrocytoma and one patient, treated at 5 months of age for retinoblastoma, developed an astrocytoma 13 years later.
CONCLUSIONS
Increased intracranial pressure is more frequently the first symptom than are convulsions in malignant cerebral hemisphere tumors. The long interval between the first manifestations and diagnosis suggests degeneration of benign tumors in 20% of cases. The development of a second malignant neoplasm is not exceptional in children. The better prognosis for ependymomas than for astrocytomas indicates that cerebral ependymomas should be excised and irradiated regardless of their grade.