Shamji Mohammed F, Vassilyadi Michael, Lam Cornelius H, Montes José L, Farmer Jean-Pierre
Division of Neurosurgery, The Ottawa Hospital, Ottawa, ON K1Y 4E9, Canada.
Pediatr Neurosurg. 2009;45(5):368-74. doi: 10.1159/000257526. Epub 2009 Nov 11.
Congenital brain tumors in the younger pediatric population are rare lesions that are histologically distinct from those in the older pediatric population. Malignant histology is common, with persistently poor outcomes despite accessible neuroimaging and evolving adjuvant therapy. There remains scant literature about the natural history of these patients because of rarity and varied institutional experiences.
A retrospective review was performed of congenital brain tumor patients surgically treated at the Montreal Children's Hospital (MCH) over a 22-year period. Patients presenting in the first year of life were evaluated for demographic information, presenting symptoms, lesion location, and management. Analysis was by median rank test and chi(2) statistics.
13 cases of congenital brain tumors were identified: 5 supratentorial and 8 infratentorial. Median age (p = 0.93) and gender (p = 0.57) did not differ by location, and predominant histologies were choroid plexus papilloma and primitive neuroectodermal tumor. Seizure activity was exclusive to supratentorial lesions (40%, p = 0.03), with hypotonia observed only among infratentorial lesions (50%, p = 0.02). There was equal incidence of hydrocephalus (69%, p = 0.57) and increasing head circumference (38%, p = 0.27) by lesion location. Supratentorial lesions were treated by total resection (n = 3), subtotal resection (n = 1), and biopsy (n = 1). Infratentorial lesions were treated by total resection (n = 1), subtotal resection (n = 2), biopsy (n = 1), no operation (n = 2), and decompressive laminectomy for two spinal lesions.
Congenital brain tumor patients represent fewer than 2% of patients treated at MCH. An evolving understanding of management objectives for these lesions requires understanding institutional experiences. Patients with supratentorial lesions frequently present with seizures, hydrocephalus, and macrocrania, and more frequently underwent total resection at surgery.
小儿先天性脑肿瘤是罕见病变，其组织学特征与大龄儿童的脑肿瘤不同。恶性组织学类型很常见，尽管有可用的神经影像学检查和不断发展的辅助治疗，但预后仍然很差。由于病例罕见且各机构经验不同，关于这些患者自然病史的文献仍然很少。
对蒙特利尔儿童医院（MCH）在22年期间接受手术治疗的先天性脑肿瘤患者进行回顾性研究。对出生后第一年就诊的患者评估人口统计学信息、症状表现、病变位置及治疗情况。采用中位数秩检验和卡方统计进行分析。
共识别出13例先天性脑肿瘤患者：幕上肿瘤5例，幕下肿瘤8例。幕上和幕下肿瘤患者的中位年龄（p = 0.93）和性别（p = 0.57）无差异，主要组织学类型为脉络丛乳头状瘤和原始神经外胚层肿瘤。癫痫发作仅见于幕上病变（40%，p = 0.03），肌张力减退仅见于幕下病变（50%，p = 0.02）。幕上和幕下病变脑积水发生率相同（69%，p = 0.57），头围增大发生率相近（38%，p = 0.27）。幕上病变的治疗方式包括全切（n = 3）、次全切（n = 1）和活检（n = 1）。幕下病变的治疗方式包括全切（n = 1）、次全切（n = 2）、活检（n = 1）、未手术（n = 2）以及对2例脊髓病变行减压性椎板切除术。
先天性脑肿瘤患者占MCH接受治疗患者的比例不到2%。对这些病变管理目标的认识不断发展，这需要了解各机构的经验。幕上病变患者常表现为癫痫发作、脑积水和巨头症，手术时更常接受全切治疗。
