Abstract：
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.
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.
摘要：
目的：颈静脉孔神经鞘瘤（JFSs）很少见，生长缓慢的良性肿瘤。今天,JFS的管理选项包括观察，手术,和辐射。然而,最佳治疗策略仍存在争议。立体定向放射外科是显微外科的微创替代或辅助治疗方案。伽玛刀放射外科适用于具有中小型肿瘤和正常颅神经（CN）功能的JFS患者。大分割立体定向放射治疗（HSRT）具有潜在的放射生物学优势，与单级立体定向放射外科相比，可以更好地保存正常结构。本文的目的是回顾使用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月在作者中心接受HSRT的74例JFS患者。其中,53例新诊断为JFS，19例患者既往有显微外科手术史，另外2例患者因伽玛刀治疗后肿瘤复发而接受了射波刀治疗。共有73例患者具有预先存在的CN症状和体征。中位肿瘤体积为14.8cm3（范围0.5-41.2cm3），其中大多数(70.3%)≥10cm3。根据肿瘤大小规定了辐射剂量方案，更多的部分用于较大的肿瘤。规定的中位边缘剂量为18.2Gy/2分数，21.0Gy/3分数，和21.6Gy/4级分。
结果：中位随访时间为103个月（18-158个月）。治疗后,42例（56.8%）患者肿瘤消退，27例（36.5%）患者肿瘤稳定，和5（6.8％）经历了肿瘤进展。其中,MRI显示1例患者完全缓解。由于肿瘤进展，三名患者在中位25个月接受了手术。一名患者因HSRT后发生的脑积水而接受了脑室腹膜分流术，而与肿瘤进展无关。5年无进展生存率为93.2%。46例患者先前存在的颅神经病变得到改善，14年保持稳定，14年恶化。
结论：HSRT被证明是JFSs安全有效的主要或辅助治疗策略，尽管14例患者（18.9％）在治疗后出现了一定程度的延迟症状恶化。该治疗选择被证明提供优异的肿瘤控制和CN功能的改善。
方法：作者回顾性分析了2009年1月至2020年1月在作者中心接受HSRT的74例JFS患者。其中,53例新诊断为JFS，19例患者既往有显微外科手术史，另外2例患者因伽玛刀治疗后肿瘤复发而接受了射波刀治疗。共有73例患者具有预先存在的CN症状和体征。中位肿瘤体积为14.8cm3（范围0.5-41.2cm3），其中大多数(70.3%)≥10cm3。根据肿瘤大小规定了辐射剂量方案，更多的部分用于较大的肿瘤。规定的中位边缘剂量为18.2Gy/2分数，21.0Gy/3分数，和21.6Gy/4级分。
结果：中位随访时间为103个月（18-158个月）。治疗后,42例（56.8%）患者肿瘤消退，27例（36.5%）患者肿瘤稳定，和5（6.8％）经历了肿瘤进展。其中,MRI显示1例患者完全缓解。由于肿瘤进展，三名患者在中位25个月接受了手术。一名患者因HSRT后发生的脑积水而接受了脑室腹膜分流术，而与肿瘤进展无关。5年无进展生存率为93.2%。46例患者先前存在的颅神经病变得到改善，14年保持稳定，14年恶化。
结论：HSRT被证明是JFSs安全有效的主要或辅助治疗策略，尽管14例患者（18.9％）在治疗后出现了一定程度的延迟症状恶化。该治疗选择被证明提供优异的肿瘤控制和CN功能的改善。
