Gamma Knife Radiosurgery for Hypothalamic Hamartoma: A Multi-Institutional Retrospective Study on Safety, Efficacy, and Complication Profile.

IF 3.9 2区 医学 Q1 CLINICAL NEUROLOGY
Neurosurgery Pub Date : 2025-02-01 Epub Date: 2024-07-11 DOI:10.1227/neu.0000000000003110
Manjul Tripathi, Jason P Sheehan, Ajay Niranjan, Lydia Ren, Stylianos Pikis, L Dade Lunsford, Selcuk Peker, Yavuz Samanci, Anne Marie Langlois, David Mathieu, Cheng Chia Lee, Huai Che Yang, Hansen Deng, Ashutosh Rai, Narendra Kumar, Jitendra Kuma Sahu, Naveen Sankhyan, Harsh Deora
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引用次数: 0

Abstract

Background and objectives: Gamma knife radiosurgery (GKRS) is a safe and effective treatment option for hypothalamic hamartomas (HH), but there is no consensus opinion on its timing, dosage, and follow-up. The aim of this study was to define the safety, efficacy, outcome, and complication profile of GKRS in this patient population.

Methods: This retrospective multicentric study involved 39 patients with the mean age of 16 ± 14.84 years. Early seizures resulted in an earlier age of diagnosis in 97% of patients. At baseline, no endocrine abnormalities were seen in 75% of patients while 18.9% showed precocious puberty (PP). The median target volume was 0.55 cc (0.1-10.00 cc), and a median margin dose of 16 Gy (8.1-20.0 Gy) was delivered in a single session. All patients were evaluated for clinical, endocrinological, and radiological outcomes.

Results: The median follow-up was 5 (0.1-15) years. The median target volume of the cohort was 0.55 (0.35-1.77) cc. The largest HH was of 10 cc. 24/39 (61.5%) were small HH (Regis I-III). At presentation, 94.8% patients suffered from seizures (87.18% with gelastic seizures). 7/39 patients (17.9%) were presented with both PP and epilepsy. Only one (2.6%) patient presented with PP alone. 29 patients had more than 3-year follow-up. All received ≥16 Gy targeting complete HH. 28% of patients showed regression in HH volume. Patients with Regis grade I-III and longer follow-up (>75 months) showed gradual improvement in seizures. 16/29 patients (55.2%) achieved good seizure control (Engel I/II) while 13 (44.8%) were in Engel III/IV status. Nine patients needed adjuvant treatment because of poor seizure control. Eight patients suffered from transient increase in seizures. One patient developed poikilothermia, and 2 patients developed new onset hormonal deficiency.

Conclusion: GKRS is a safe and effective modality for treatment of HH with significant improvement in seizure control with minimal disruption of endocrine profile. It provides an excellent safety, efficacy, and complication profile, especially for small HH. Latency of results and its adjuvant nature remain the areas of research and breakthroughs among contemporary treatment options.

伽玛刀放射手术治疗下丘脑 Hamartoma:一项关于安全性、有效性和并发症概况的多机构回顾性研究。
背景和目的:伽玛刀放射外科手术(GKRS)是治疗下丘脑仓鼠神经瘤(HH)的一种安全有效的方法,但对于其时机、剂量和随访还没有达成共识。本研究的目的是确定 GKRS 在这一患者群体中的安全性、疗效、结果和并发症情况:这项多中心回顾性研究涉及 39 名患者,平均年龄为 16 ± 14.84 岁。97%的患者因癫痫发作较早而被确诊。基线时,75%的患者未发现内分泌异常,18.9%的患者出现性早熟(PP)。中位靶体积为 0.55 cc(0.1-10.00 cc),单次治疗的中位边缘剂量为 16 Gy(8.1-20.0 Gy)。对所有患者的临床、内分泌和放射学结果进行了评估:中位随访时间为5(0.1-15)年。中位靶体积为 0.55 (0.35-1.77) cc。24/39(61.5%)例为小HH(Regis I-III)。94.8%的患者在就诊时有癫痫发作(87.18%为凝胶状发作)。7/39 的患者(17.9%)同时患有 PP 和癫痫。只有一名患者(2.6%)仅伴有 PP。29 名患者的随访时间超过 3 年。所有患者都接受了针对完全HH的≥16 Gy治疗。28%的患者的HH体积有所缩小。Regis分级为I-III级且随访时间较长(>75个月)的患者癫痫发作情况逐渐改善。16/29例患者(55.2%)的癫痫发作控制良好(恩格尔I/II级),13例(44.8%)处于恩格尔III/IV级状态。九名患者因癫痫发作控制不佳而需要辅助治疗。八名患者的癫痫发作一过性增加。1名患者出现了poikilothermia,2名患者出现了新的激素缺乏症:GKRS是治疗HH的一种安全有效的方法,可显著改善癫痫发作控制,对内分泌的影响极小。它具有极佳的安全性、有效性和并发症情况,尤其适用于小型 HH。疗效的潜伏期及其辅助性质仍是当代治疗方案中需要研究和突破的领域。
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来源期刊
Neurosurgery
Neurosurgery 医学-临床神经学
CiteScore
8.20
自引率
6.20%
发文量
898
审稿时长
2-4 weeks
期刊介绍: Neurosurgery, the official journal of the Congress of Neurological Surgeons, publishes research on clinical and experimental neurosurgery covering the very latest developments in science, technology, and medicine. For professionals aware of the rapid pace of developments in the field, this journal is nothing short of indispensable as the most complete window on the contemporary field of neurosurgery. Neurosurgery is the fastest-growing journal in the field, with a worldwide reputation for reliable coverage delivered with a fresh and dynamic outlook.
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