Kim Joo Whan, Phi Ji Hoon, Kim Seung-Ki, Lee Joo Ho, Park Sung-Hye, Won Jae-Kyung, Choi Jung Yoon, Kang Hyoung Jin, Park Chul-Kee
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, 03080, Seoul, Republic of Korea.
Department of Radiation Oncology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Childs Nerv Syst. 2023 Mar;39(3):583-591. doi: 10.1007/s00381-023-05839-x. Epub 2023 Jan 20.
Pilocytic astrocytoma is a slow-growing tumor that predominantly develops in children, but has a broad age spectrum. A notable characteristic of pilocytic astrocytoma is that the tumor arises in diverse locations and the clinical course is not always benign. Therefore, it is necessary to elucidate the clinical spectrum of the disease and analyze the relevant prognostic factors.
Demographic and treatment-related factors were retrospectively reviewed in a cohort of 254 patients with histologically confirmed pilocytic astrocytoma. Clinical features were compared between the pediatric group (N = 208; age < 18 years) and the adult group (N = 46; age ≥ 18 years). Cox regression analysis was performed to identify relevant prognostic factors.
There was no difference in progression-free survival (PFS) between the pediatric and adult groups (p = 0.36); however, patients under 8 years of age exhibited worse PFS (p < 0.01). Leptomeningeal seeding at diagnosis and pilomyxoid histology was observed only in pediatric patients. In the pediatric group, nine patients experienced recurrence after complete resection. Increasing age (hazard ratio (HR) = 0.89, p < 0.01) and adjuvant therapy (HR = 0.32, p < 0.01) were protective factors against tumor progression. In the adult group, no progression occurred after complete resection. Age and adjuvant therapy were not significant factors in the adult group.
Pilocytic astrocytoma presents with a diverse clinical spectrum. Complete resection is of utmost importance, and appropriate adjuvant treatment is recommended if complete resection cannot be achieved. Children with younger age are associated with more aggressive tumors, and recurrence may occur even after complete resection.
毛细胞型星形细胞瘤是一种生长缓慢的肿瘤，主要发生于儿童，但发病年龄范围较广。毛细胞型星形细胞瘤的一个显著特点是肿瘤发生部位多样，临床病程并非总是良性。因此，有必要阐明该疾病的临床谱并分析相关预后因素。
回顾性分析254例经组织学确诊的毛细胞型星形细胞瘤患者的人口统计学和治疗相关因素。比较儿童组（N = 208；年龄＜18岁）和成人组（N = 46；年龄≥18岁）的临床特征。进行Cox回归分析以确定相关预后因素。
儿童组和成人组的无进展生存期（PFS）无差异（p = 0.36）；然而，8岁以下患者的PFS较差（p＜0.01）。仅在儿童患者中观察到诊断时软脑膜播散和毛黏液样组织学特征。在儿童组中，9例患者在完全切除后复发。年龄增加（风险比（HR）= 0.89，p＜0.01）和辅助治疗（HR = 0.32，p＜0.01）是肿瘤进展的保护因素。在成人组中，完全切除后无进展发生。年龄和辅助治疗在成人组中不是显著因素。
毛细胞型星形细胞瘤临床表现多样。完全切除至关重要，如果无法实现完全切除，建议进行适当的辅助治疗。年龄较小的儿童肿瘤侵袭性更强，即使完全切除后也可能复发。
