Lim Jia Xu, Tan Enrica E K, Ng Lee Ping, Seow Wan Tew, Chang Kenneth T E, Wong Ru Xin, Looi Wen Shen, Low David C Y, Low Sharon Y Y
Neurosurgical Service, KK Women's and Children's Hospital, Singapore, Singapore.
Paediatric Haematology/Oncology Service, KK Women's and Children's Hospital, Singapore, Singapore.
Front Surg. 2022 May 2;9:827675. doi: 10.3389/fsurg.2022.827675. eCollection 2022.
The treatment of pediatric optic pathway gliomas (OPG) is challenging. At present, most centers provide individualized treatment to maximize progression free survival (PFS) and minimize morbidity. We aim to report our experience in the management of pediatric OPG, and investigate factors associated with an increased duration of remission after treatment.
This is a single-institution study approved by the hospital ethics board. A retrospective review of consecutive OPGs managed from 2000 to 2020 was performed. Patients were divided into those managed with monomodality treatment (MT) and those who received combined therapy (CT). MT included various forms of surgery, chemotherapy and radiotherapy given alone, while CT involves a combination of surgery and adjuvant chemotherapy and/or radiotherapy.
Twenty-two patients were selected for this study. They had 40 treatment cycles; and a total follow up duration of 194.8 patient-years. Most of them were male (63.6%) and presented with visual deficits (72.7%). The mean age at initial presentation was 65 months and majority (86.4%) had their tumors arising directly from the optic chiasm, with 77.3% with hypothalamic extension. One patient had Neurofibromatosis type I (4.5%). The most common histological diagnosis was pilocytic astrocytoma (90.9%), followed by pilomyxoid astrocytoma (9.1%). The 5- and 10- year PFS were 46.2% and 36.4% respectively, while the 5- and 10-year OS were both 100%. When accounting for treatment type, there were 24 treatment cycles with MT (60.0%) and 16 CT (40.0%). After adjustment, treatments with MT were shown to have a shorter mean duration of remission (MT: 45 ± 49, CT: 84 ± 79 months; = 0.007). Cox regression curve plotted after adjusting for patient's age at treatment demonstrated a significantly longer PFS in the CT group ( = 0.037).
Our results suggest a significant survival benefit of CT over MT for affected patients due to the prolonged the duration of disease remission, for both primary and subsequent treatments. Nonetheless, we acknowledge that our study reflects the outcomes of treatment strategies that have evolved over time. We emphasize the need for collective efforts from a dedicated multidisciplinary team and international collaborations for better disease understanding.
小儿视神经通路胶质瘤（OPG）的治疗具有挑战性。目前，大多数中心提供个体化治疗，以最大限度地提高无进展生存期（PFS）并将发病率降至最低。我们旨在报告我们在小儿OPG管理方面的经验，并调查与治疗后缓解期延长相关的因素。
这是一项经医院伦理委员会批准的单机构研究。对2000年至2020年连续管理的OPG进行了回顾性研究。患者分为接受单一治疗（MT）的患者和接受联合治疗（CT）的患者。MT包括单独进行的各种形式的手术、化疗和放疗，而CT则包括手术与辅助化疗和/或放疗的联合。
本研究选取了22例患者。他们有40个治疗周期；总随访时间为194.8患者年。他们大多数为男性（63.6%），并伴有视力缺陷（72.7%）。初次就诊时的平均年龄为65个月，大多数（86.4%）的肿瘤直接起源于视交叉，77.3%的肿瘤侵犯下丘脑。1例患者患有I型神经纤维瘤病（4.5%）。最常见的组织学诊断是毛细胞型星形细胞瘤（90.9%），其次是毛黏液样星形细胞瘤（9.1%）。5年和10年的PFS分别为46.2%和36.4%，而5年和10年的总生存率均为100%。在考虑治疗类型时，MT有24个治疗周期（60.0%），CT有16个治疗周期（40.0%）。调整后，MT治疗的缓解期平均持续时间较短（MT：45±49个月，CT：84±79个月；P=0.007）。在调整治疗时患者年龄后绘制的Cox回归曲线显示，CT组的PFS明显更长（P=0.037）。
我们的结果表明，对于受影响的患者，由于疾病缓解期延长，CT相对于MT具有显著的生存益处，无论是对于初始治疗还是后续治疗。尽管如此，我们承认我们的研究反映了随着时间推移而演变的治疗策略的结果。我们强调需要一个专门的多学科团队共同努力并开展国际合作，以更好地了解这种疾病。
