Clinical Analysis Of Primary Pediatric Central Nervous System Tumors

Part One Primary pediatric central nervous system tumors Statistic study of 763 cases in a single institutiontionObjective:To investigate the epidemiology of primary pediatric central nervous system tumors based on a series of the past 10 years pediatric neurosurgical patients in Shanghai Hua Shan Hospital affiliated to Fu Dan UniversityMethods:Retrospective study was performed on children under 18 years old with central nervous system tumors during 1995 and 2004.Patients' age and gender distribution,location of the tumors,and WHO classification and grading of the tumors were analyzed.Results:Altogether 763 cases were included.The mean age was 12.7%and the male to female ratio was 1.56:1.Among them,intracranial tumors took 93.4%(713/763),whereas spinal tumors accounts for 6.6%(50/763).In intracranial tumors,62.4%(476/763) of them was supratentorial including pineal-quardrigeminal tumors,which was predominant to infratentorial tumors(30.5%,233/763).In this series of patients,688 cases had pathological diagnosis.The most common 5 categories of tumors were:astrocytic tumors(25.7%,177/688),craniopharyngioma (12.8%,88/688),medulloblastoma(9.3%,64/688),germ cell tumors (8.9%,61/688),and pituitary adenoma(7.5%,52/688).Conclusion:As the main neurological medical center of southern part of China,the statistics of Hua Shan hospital could be representative.The epidemiology of pediatric central nervous system tumors has its own specialty.Therefore,it is crucial to deal with primary pediatric central nervous system tumors according to children's characteristics. Part Two Diagnosis,treatment and long-term therapeutic effect of pediatric pilocytic astrocytomaObjective:To investigate the clinical classification,the therapeutic features and long-term survival of pediatric pilocytic astrocytoma.Methods:Retrospective analysis on pediatric patients under 18 years old with pilocytic astrocytoma confirmed by pathology during 1995 and 2005 was made on clinical manifestation,radiological imaging,therapeutic features and long-term prognosis according to their different locations.Results:Altogether 61 patients were included.The mean age was 11.7±4.6 years old and the male to female ratio was 1:1.2.Tumors occurred mostly in the cerebellum(29 cases,47.5%),secondly in optic pathway-hypothalamus-Ⅲventricle(15 cases,24.6%),then in thalamus-basal ganglia(7 cases,11.5%),hemisphere(5 cases,8.2%), brain stem(3 cases,4.9%) and spinal cord(2 cases,3.3%).The main radiological feature of the tumors located in cerebellum and hemisphere was a cystic mass with an enhancing nodular and ringlike cystic wall.The tumor located in optic pathway-hypothalamus-Ⅲventricle appeared in the form of homogenously enhanced solid lesion.The tumor located in brain stem came in the form of nonenhanced solid lesion.All the patients underwent operations.The rate of total resection of the tumors located in cerebellum and hemisphere was 94.1%.As for the tumors located in optic pathway-hypothalamus-Ⅲventricle,only 2 cases(13.3%) achieved total resection.Radiotherapy was given in 15 patients(68.1%) who had subtotal or partial resection and the chemotherapy was given in 2 patients (9.1%).51 patients(83.6%) were successfully followed up.The period was 1～10 years.No recurrence occurred to patients with total resection. The 5-year survival rate of the tumor located in optic pathway-hypothalamus-Ⅲventricle was 77.8%.All the three patients with tumor located in brain stem still survived and had no recurrence.Conclusions:The prognosis of pilocytic astrocytoma correlates closely with the tumor location.Tumors located in cerebellum and hemisphere can be cured after total resection.While most of the tumors located in optic pathway-hypothalamus-Ⅲventricle and brain stem are not able to be totally removed.But 78.6%of them have been in static status for a long period of time.The main purpose for the surgery of pilocytic astrocytoma in these regions is to get a confirmed pathological diagnosis and to make the CSF pathway unblocked.