Rochat Per, Johannesen Helle Hjorth, Gjerris Flemming
University Clinic of Neurosurgery, Neuroscience Centre, Rigshospitalet, Department of Radiology, Copenhagen University Hospital, Copenhagen, Denmark.
J Neurosurg. 2004 Feb;100(2 Suppl Pediatrics):179-82. doi: 10.3171/ped.2004.100.2.0179.
Meningiomas in children are rare, infrequently described in the literature, and often associated with neurofibromatosis Type 2 (NF2). The authors report a series of 22 children treated for an intracranial meningioma in Denmark between 1935 and 1984.
Of 1542 cases of pediatric intracranial tumors in children younger than 15 years of age, 22 harbored meningiomas. Three children suffered from NF. The male/female ratio was 8:14. The mean age at the time of diagnosis was 5 years for boys and 11.5 years for girls. At the time of diagnosis all tumors were large. All patients underwent surgery. In 20 cases, the final histological diagnoses were low-grade and in two cases high-grade tumors. The follow-up period ranged from I to 45 years (mean 16 years). Two patients were lost to follow up. Four of seven boys and three of 13 girls survived. Five of 13 children in whom the tumor was completely removed survived, whereas two of seven in whom the lesion was partially removed survived. The mean survival time in children who died during follow up was 10 years. Two children with anaplastic meningioma remain alive.
The long-term prognosis for surgically treated children with intracranial meningiomas was worse than expected. Some reasons for this may have been the late diagnosis and related large size of the tumor during a period of limited diagnostic capacity prior to the computerized tomography and magnetic resonance imaging eras, and the association of NF2 with multiple tumors of the central nervous system. Complete resection is not always possible and should be performed as an image-guided operation.
儿童脑膜瘤较为罕见,文献中对此描述不多,且常与2型神经纤维瘤病(NF2)相关。作者报告了1935年至1984年间在丹麦接受治疗的一系列22例儿童颅内脑膜瘤病例。
在1542例15岁以下儿童的颅内肿瘤病例中,22例患有脑膜瘤。3名儿童患有NF。男女比例为8:14。男孩诊断时的平均年龄为5岁,女孩为11.5岁。诊断时所有肿瘤都很大。所有患者均接受了手术。20例最终组织学诊断为低级别肿瘤,2例为高级别肿瘤。随访期为1至45年(平均16年)。2例患者失访。7名男孩中有4名存活,13名女孩中有3名存活。肿瘤完全切除的13名儿童中有5名存活,而病变部分切除的7名儿童中有2名存活。随访期间死亡的儿童平均存活时间为10年。2例间变性脑膜瘤患儿仍存活。
手术治疗的儿童颅内脑膜瘤的长期预后比预期差。造成这种情况的一些原因可能是在计算机断层扫描和磁共振成像时代之前诊断能力有限的时期,诊断延迟以及肿瘤相关的大尺寸,以及NF2与中枢神经系统多发肿瘤的关联。完全切除并不总是可行的,应在影像引导下进行手术。