OBJECTIVE Jugular foramen schwannomas (JFSs) are rarely seen, benign tumors with slow growth. Today, management options for JFSs include observation, surgery, and radiation. However, the optimal treatment strategy remains controversial. Stereotactic radiosurgery serves as a minimally invasive alternative or adjuvant therapeutic regimen of microsurgery. Gamma Knife radiosurgery is suitable for patients with JFS who have small- and medium-sized tumors and normal cranial nerve (CN) function. Hypofractionated stereotactic radiotherapy (HSRT) offers a potential radiobiological advantage and may result in better preservation of normal structures compared to single-fraction stereotactic radiosurgery. The aim of the article was to review the clinical and radiographic outcomes of patients with JFS who were treated using HSRT. METHODS The authors retrospectively analyzed 74 patients with JFS who received HSRT between January 2009 and January 2020 in the authors' center. Among them, 53 patients were newly diagnosed with JFS, 19 patients had a previous history of microsurgical resection, and the other 2 patients underwent CyberKnife because of tumor recurrence after Gamma Knife radiosurgery. A total of 73 patients had preexisting CN symptoms and signs. The median tumor volume was 14.8 cm3 (range 0.5-41.2 cm3), and most of them (70.3%) were ≥ 10 cm3. The radiation dose regimen was prescribed depending on the tumor size, and more fractions were used in larger tumors. The median margin doses prescribed were 18.2 Gy/2 fractions, 21.0 Gy/3 fractions, and 21.6 Gy/4 fractions. RESULTS The median follow-up was 103 months (range 18-158 months). After treatment, 42 (56.8%) patients had tumor regression, 27 (36.5%) patients had stable tumors, and 5 (6.8%) experienced tumor progression. Among them, MRI revealed that 1 patient had a complete response. Three patients received surgery at a median of 25 months because of tumor progression. One patient underwent ventriculoperitoneal shunt insertion for hydrocephalus that developed after HSRT independent of tumor progression. The 5-year progression-free survival rate was 93.2%. Preexisting cranial neuropathies improved in 46 patients, remained stable in 14, and worsened in 14. CONCLUSIONS HSRT proved to be a safe and effective primary or adjuvant treatment strategy for JFSs, although 14 patients (18.9%) experienced some degree of delayed symptomatic deterioration posttreatment. This therapeutic option was demonstrated to provide both excellent tumor control and improvement in CN function.

中文翻译：

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使用射波刀机器人放射外科系统进行大分割立体定向放射治疗颈静脉孔神经鞘瘤的长期临床结果。

目的 颈静脉孔神经鞘瘤（JFS）是一种罕见的良性肿瘤，生长缓慢。如今，JFS 的治疗选择包括观察、手术和放射。然而，最佳治疗策略仍存在争议。立体定向放射外科手术作为显微外科手术的微创替代或辅助治疗方案。伽玛刀放射外科手术适用于中小型肿瘤、脑神经（CN）功能正常的 JFS 患者。与单次立体定向放射外科手术相比，大分割立体定向放射治疗（HSRT）具有潜在的放射生物学优势，并且可以更好地保存正常结构。本文的目的是回顾接受 HSRT 治疗的 JFS 患者的临床和影像学结果。方法 作者回顾性分析了 2009 年 1 月至 2020 年 1 月期间在作者中心接受 HSRT 的 74 例 JFS 患者。其中，53名患者新诊断为JFS，19名患者既往有显微手术切除史，另外2名患者因伽玛刀放射治疗后肿瘤复发而接受射波刀治疗。共有 73 名患者先前存在 CN 症状和体征。肿瘤体积中位数为14.8 cm3（范围0.5-41.2 cm3），其中大多数（70.3%）≥10 cm3。根据肿瘤大小制定放射剂量方案，较大肿瘤使用更多剂量。规定的中位边缘剂量为 18.2 Gy/2 分次、21.0 Gy/3 分次和 21.6 Gy/4 分次。结果 中位随访时间为 103 个月（范围 18-158 个月）。治疗后，42 名患者（56.8%）肿瘤消退，27 名患者（36.5%）肿瘤稳定，5 名患者（6.8%）肿瘤进展。其中，MRI显示1名患者获得完全缓解。3 名患者因肿瘤进展而在中位 25 个月时接受了手术。一名患者因 HSRT 后出现的脑积水而接受脑室腹腔分流术插入，与肿瘤进展无关。5年无进展生存率为93.2%。46 名患者先前存在的颅神经病变有所改善，14 名患者保持稳定，14 名患者恶化。结论 HSRT 被证明是 JFS 的安全有效的主要或辅助治疗策略，尽管 14 名患者 (18.9%) 在治疗后经历了一定程度的延迟症状恶化。这种治疗方案被证明可以提供出色的肿瘤控制和 CN 功能的改善。