Jurkiewicz Elżbieta, Brożyna Agnieszka, Grajkowska Wiesława, Bekiesińska-Figatowska Monika, Daszkiewicz Paweł, Nowak Katarzyna, Malczyk Katarzyna, Walecki Jerzy, Perek Danuta, Syczewska Małgorzata
Department of Diagnostic Imaging, MR Unit, The Children's Memorial Health Institute, Warsaw, Poland.
Childs Nerv Syst. 2012 Aug;28(8):1193-201. doi: 10.1007/s00381-012-1798-5. Epub 2012 May 31.
Central nervous system tumors diagnosed before the end of the first year of life differ from those found in older children and in adults. The differences include mode of clinical presentation, anatomical distribution, histopathological diagnoses, response to therapy, and outcome.
The material consists of 56 children (23 girls and 33 boys), aged at recognition 32 Hbd-12 months. We reviewed charts and MR exams according to age of the onset of symptoms, location of tumors, treatment, histopathology, and outcomes. Data of the outcome were analyzed using Kaplan-Meier plots and chi-square test.
Eleven cases were recognized before 6 weeks of life, 24 before the age of 6 months, and 21 were diagnosed up to the end of 1 year of age. Thirty-eight tumors were located in the supratentorial compartment; 18 were infratentorial. Median age of tumors' recognition was 5.2 months; 4.3 months for supratentorial and 7.2 months for infratentorial tumors. We found 18 glial cell tumors (high and low grade), 15 embryonal tumors, and 12 choroid plexus tumors.
The outcome of congenital CNS tumors depends on the size, location, time of diagnosis, histological type of the tumor, and therapeutic option. Neurosurgical procedures are necessary in most cases. Despite the notable advances in therapy, the outcome remains poor.
出生后第一年末之前诊断出的中枢神经系统肿瘤与大龄儿童及成人的中枢神经系统肿瘤有所不同。这些差异包括临床表现方式、解剖分布、组织病理学诊断、对治疗的反应以及预后。
研究材料包括56名儿童(23名女孩和33名男孩),确诊时年龄为32天至12个月。我们根据症状出现的年龄、肿瘤位置、治疗、组织病理学及预后对病历和磁共振成像检查进行了回顾。使用Kaplan-Meier曲线和卡方检验对预后数据进行了分析。
11例在出生6周前确诊,24例在6个月前确诊,21例在1岁末前确诊。38个肿瘤位于幕上腔;18个位于幕下。肿瘤确诊的中位年龄为5.2个月;幕上肿瘤为4.3个月,幕下肿瘤为7.2个月。我们发现18例胶质细胞瘤(高分级和低分级)、15例胚胎性肿瘤和12例脉络丛肿瘤。
先天性中枢神经系统肿瘤的预后取决于肿瘤的大小、位置、诊断时间、组织学类型及治疗选择。大多数情况下需要进行神经外科手术。尽管治疗取得了显著进展,但预后仍然很差。