A single-center experience of central nervous system tumors in children under three years old

Abstract

Purpose: This study aims to summarize the characteristics of children under three years old (≤3 years) with central nervous system (CNS) tumors and to investigate the factors that influence their overall survival (OS) time.

Methods: We treated 171 pediatric patients (≤3 years) with CNS tumors at Yuquan Hospital of Tsinghua University from January 2016 to June 2023. Of these, 162 cases were successfully followed up. Kaplan-Meier survival analysis and Cox regression were utilized to evaluate factors potentially influencing OS of malignancies.

Results: There was a male predominance among the patients. The three most common tumors were embryonal tumors, gliomas, and craniopharyngiomas. Gross total resection (GTR) was achieved in select cases. Patients with high-grade malignancies were advised to undergo chemotherapy and/or radiotherapy after surgery. Optic gliomas and diffuse midline gliomas were partially resected and treated with adjuvant treatments. The median survival time of low-grade malignant tumors was 41.5 months, while that of high-grade malignant tumors was 15 months. Kaplan-Meier survival analysis identified the factors potentially influencing OS of malignancies: extent of resection, CNS WHO grade, grade of malignancies, and Ki-67 labeling index (Ki-67 LI). Subsequent multivariate analysis highlighted the interactive factor (extent of resection × CNS WHO grade) along with Ki-67 LI, as the most significant variables. Factors such as sex, age, tumor location, and onset-to-treatment time appeared not to affect OS.

Conclusions: GTR remains the cornerstone of treatment for children (≤3 years) with CNS tumors, except for optic glioma, diffuse midline glioma, and germinoma. The interactive factor (extent of resection × CNS WHO grade) and Ki-67 LI are the most significant factors affecting OS. The implementation of preoperative neoadjuvant chemotherapy and early postoperative chemotherapy may enhance prognosis.

Keywords: central nervous system; chemotherapy; child; neurosurgery; pediatrics; tumor.

Figure 1

The pie chart of all types of children (≤3 years) with CNS tumors and details of the top 6 malignancies in our data.

Figure 2

The typical preoperative and follow-up MRI images of several patients. a and b: CNS germinoma metastasis, before and after “chemotherapy and RT”; c and d: CNS germinoma in basal ganglia, before and after “chemotherapy and RT”; e and f: pinealoblastoma, before and after “operation + chemotherapy and RT”; g and h: high-grade malignant astrocytoma, before and after “operation + chemotherapy and RT”; i and j: ependymoma, before and after “operation + RT”; k and l: medulloblastoma, before and after “operation + chemotherapy and RT”; m and n: craniopharygioma, before and after “operation”;.

Figure 3

The Kaplan–Meier plots on four significant factors affecting survival time of children (≤3 years) with CNS tumors: figure a for extent of resection: gross total resection, subtotal resection, partial resection, p = 0.002; figure b for CNS WHO grade: WHO Ⅰ, WHO Ⅱ, WHO Ⅲ, WHO Ⅳ, p < 0.0001; figure c for grade of malignancies: low-grade, high-grade, p < 0.0001; figure d for Ki-67 LI: x < 5, 5 ≤ x < 10, 10 ≤ x < 30, x ≥ 30, p < 0.0001. The Kaplan–Meier plots on four not significant factors affecting survival time of children (≤3 years) with CNS tumors: figure e for age (year): x ≤ 1, 2 < x ≤ 3, 1 < x ≤ 2, 2 < x ≤ 3, p = 0.932; figure f for sex: male, female, p = 0.921; figure g for onset-to-treatment time (month): x < 1, 1 ≤ x < 3, 3 ≤ x < 6, 6 ≤ x < 12, x ≥ 12, p = 0.381; figure h for location: supratentorial; subtentorial; p = 0.144.