Department of Neurosurgery, Rutgers-Robert Wood Johnson Medical School & University Hospital, Rutgers-New Jersey Medical School, New Brunswick, New Jersey, USA,
Department of Neurological Surgery, Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
Pediatr Neurosurg. 2022;57(2):118-126. doi: 10.1159/000521732. Epub 2021 Dec 30.
Optic pathway/hypothalamic gliomas are rare pediatric brain tumors. The management paradigm for these challenging tumors includes chemotherapy, radiotherapy, or surgical resection, but the optimal management strategy remains elusive. Gamma knife radiosurgery (GKRS) has emerged as a promising treatment for such lesions as documented by a small number of cases in the literature.
We present a rare case of hypothalamic glioma in a 13-year-old girl who was referred to our service due to growth of an incidentally diagnosed hypothalamic lesion following head injury at the age of 8 years. The lesion demonstrated hypointensity on T1- and hyperintensity on T2-weighted imaging without contrast enhancement. Given the growth of the lesion on serial imaging, a stereotactic biopsy was performed demonstrating low-grade glioma. The patient underwent GKRS treatment with a marginal dose of 15 Gy at 50% isodose line for a tumor volume of 2.2 mL. Annual radiological surveillance over the next 17 years demonstrated a gradual shrinkage of the lesion until it completely disappeared. The patient is currently a healthy 31-year-old female without any visual, endocrine, or neurocognitive deficits.
The outcome obtained after extended follow-up in our patient highlights the safety and efficacy of GKRS in the management of hypothalamic gliomas in pediatrics, which in turn can avoid potentially serious complications of surgery in this vulnerable patient population, especially in this sensitive location.
视神经通路/下丘脑胶质瘤是罕见的儿童脑肿瘤。这些具有挑战性的肿瘤的治疗模式包括化疗、放疗或手术切除，但最佳管理策略仍难以捉摸。伽玛刀放射外科 (GKRS) 已成为治疗此类病变的一种有前途的方法，这在文献中的少数病例中有记录。
我们报告了一例 13 岁女孩的下丘脑胶质瘤病例，她因 8 岁时头部受伤后意外诊断出的下丘脑病变生长而被转至我们的服务部门。病变在 T1 加权像上表现为低信号，在 T2 加权像上表现为高信号，无对比增强。鉴于病变在连续影像学上的生长，进行了立体定向活检，结果显示为低级别胶质瘤。患者接受了 GKRS 治疗，边缘剂量为 15 Gy，50%等剂量线用于 2.2 毫升的肿瘤体积。在接下来的 17 年中，每年进行影像学监测，显示病变逐渐缩小，直至完全消失。目前，患者是一名健康的 31 岁女性，没有任何视觉、内分泌或神经认知缺陷。
我们患者的长期随访结果突出了 GKRS 在儿科下丘脑胶质瘤治疗中的安全性和有效性，这反过来又可以避免在这个脆弱的患者群体中手术可能带来的严重并发症，尤其是在这个敏感的位置。
