Lee Jong Seok, Lee Jung-Il
Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Brain Tumor Res Treat. 2025 Jul;13(3):106-111. doi: 10.14791/btrt.2025.0014.
Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign, but locally aggressive tumor that typically affects adolescent males. While surgical resection is the standard treatment, achieving total resection is often challenging in advanced-stage tumors with intracranial extension, resulting in high recurrence rates. Gamma knife radiosurgery (GKRS) has been suggested as a potential adjuvant or salvage therapy, but evidence remains limited. In this study, we report three pediatric cases of advanced-stage JNA treated with GKRS following incomplete surgical resection. Two patients demonstrated durable local tumor control with a significant reduction in tumor size until 2 and 12 years after GKRS. The other patient with partial coverage of tumor by prescription isodose 12 Gy showed a reduction of tumor volume at 6 months but subsequent progression at 1 year. No GKRS-related complications were observed during the follow-up period. Our findings suggest that GKRS appears to be a potentially safe and effective treatment modality for residual or recurrent JNAs. Fractionated or staged GKRS combined with surgery may be a preferable strategy for large tumors in which extensive surgery alone, conventional radiotherapy, or single-fraction radiosurgery may be associated with increased morbidity in pediatric populations.
青少年鼻咽血管纤维瘤(JNA)是一种罕见的良性但具有局部侵袭性的肿瘤,通常影响青春期男性。虽然手术切除是标准治疗方法,但对于有颅内侵犯的晚期肿瘤,实现完全切除往往具有挑战性,导致高复发率。伽玛刀放射外科手术(GKRS)已被建议作为一种潜在的辅助或挽救治疗方法,但证据仍然有限。在本研究中,我们报告了3例不完全手术切除后接受GKRS治疗的晚期JNA儿科病例。2例患者在GKRS后2年和12年显示出持久的局部肿瘤控制,肿瘤大小显著减小。另1例肿瘤被12 Gy处方等剂量线部分覆盖的患者在6个月时肿瘤体积减小,但在1年时出现进展。随访期间未观察到与GKRS相关的并发症。我们的研究结果表明,GKRS似乎是一种潜在安全有效的治疗残余或复发性JNA的方法。分次或分期GKRS联合手术可能是治疗大肿瘤的更优策略,因为单纯广泛手术、传统放疗或单次分割放射外科手术可能会增加儿科患者的发病率。