摘要：

目的 探讨婴幼儿脑肿瘤的特殊临床表现、病理类型以及分布特点。方法 选择2011年1月至2018年12月行手术治疗的儿童（< 18岁）脑肿瘤患者，分为婴幼儿组（0~3岁）与大龄儿童组（4~18岁），归纳分析两组患者肿瘤病理类型、好发部位、临床表现等特点。结果 垂体瘤仅发生于4~18岁大龄儿童（P=0.001）；而室管膜瘤（P=0.001）、脉络丛乳头状瘤/癌（P=0.000）、髓母细胞瘤以外的其他胚胎性肿瘤（P=0.013）则好发于0~3岁婴幼儿，且大多位于幕上（P=0.023）。715例中共371例详细记录肿瘤大小，组间差异具有统计学意义（P=0.000）；肿瘤大小 < 30 mm（χ²=33.939，P=0.000）、30~60 mm（χ²=12.483，P=0.000）和61~100 mm（χ²=23.158，P=0.000）患儿，与 > 100 mm患儿之间差异有统计学意义，且肿瘤大小 > 100 mm患儿中以婴幼儿所占比例最高。低级别脑肿瘤（WHOⅠ~Ⅱ级）患儿总生存期高于高级别脑肿瘤（WHOⅢ~Ⅳ级）患儿（P=0.000）。结论 婴幼儿脑肿瘤具有不同于大龄儿童群体的特殊病理类型和临床特点。婴幼儿低级别胶质瘤预后良好，呈慢病特点；恶性脑肿瘤包括少见的各种胚胎性肿瘤，恶性程度高，易复发和发生神经系统多处种植转移，患儿无法接受放射治疗，而化疗药物的选择和疗效欠佳，预后极差。提高婴幼儿脑肿瘤患儿的生活质量和预后，需要更广泛的多学科合作。

关键词: 脑肿瘤, 婴儿, 病理学, 预后

Abstract:

Objective To investigate the special clinical manifestations, pathological spectrum and locations of infant and young children (0-3 years old) brain tumors that differed from those of the older children population (4-18 years old). Methods Children (< 18 years old) with brain tumors who underwent surgical treatment in our department from January 2011 to December 2018 were collected and were divided into 2 groups:0-3 years old and 4-18 years old according to their age, and tumors, pathological spectrum, locations, clinical manifestations were summarized and compared between 2 groups. Results Among the 100 cases of infant and young children brain tumors, the most common histopathological type was low-grade glioma, especially the pilocytic astrocytoma. Significantly, the incidence of ependymoma (P=0.001) and choroid plexus papilloma/cancer (P=0.000) was much higher than the older children brain tumors population, being the second and third most common brain tumor pathologies of the infant and young children patients. Embryonal neoplasms other than medulloblastoma were higher in this population than the older one (4-18 years old) as well (P=0.013). The proportion of malignant tumors was higher on supratentorial location (P=0.023). Huge tumor growth was more often seen in infant and younger children (P=0.000), tumor diameter < 30 mm (χ²=33.939, P=0.000), 30-60 mm (χ²=12.483, P=0.000), and 61-100 mm (χ²=23.158, P=0.000) had statistic difference with tumors > 100 mm in diameter. The overall survival (OS) rate in low-grade (WHOⅠ-Ⅱ) tumor group was significantly higher than that in high-grade (WHOⅢ-Ⅳ) tumor group (P=0.000). Conclusions Brain tumors in infants and young children had special pathological spectrum and clinical manifestations being different from the older children tumor population. The prognosis of low-grade glioma in infants and young children was good and had the characteristics of chronic disease. Malignant brain tumors, including rare embryonal tumors, were prone to relapse and multiple metastases across the central nervous system. The young children couldn't suffer radiotherapy, and at the mean time, the choice and effect of chemotherapy were quite limited. To improve the quality of life and the prognosis of infants and young children with brain tumors required more extensive multi-disciplinary cooperation.

Key words: Brain neoplasms, Infant, Pathology, Prognosis