Yang Chenlong, Li Guang, Fang Jingyi, Wu Liang, Yang Tao, Deng Xiaofeng, Xu Yulun
Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China.
J Neurooncol. 2015 May;122(3):539-47. doi: 10.1007/s11060-015-1742-0. Epub 2015 Feb 27.
Spinal paragangliomas are extremely rare tumors, most frequently involving the cauda equina and the filum terminale. We aimed to investigate the clinical manifestations, radiological features, management, and follow-up data of primary spinal paraganglioma. We present the clinical data and long-term outcomes from a consecutive surgical series of 19 patients with pathologically diagnosed spinal paragangliomas. All of the patients had undergone surgical resection. Pre- and postoperative magnetic resonance imaging was performed and follow-up data and neurological functional assessment are presented and discussed. The mean age at diagnosis was 47.7 years, with a significant male predominance. The primary clinical symptoms were low back pain and sciatica. Magnetic resonance images (MRI) showed characteristic signs that help differentiate paragangliomas from other spinal tumors, including a "salt & pepper" sign, serpiginous flow void, and a peripheral hypointense rim. Also, a well-encapsulated appearance can be found intraoperatively. During a mean follow-up period of 62.1 months, remnant tumor progression was noted on MRI in three patients with incomplete resection. Pain symptoms were relieved immediately after surgical intervention, while motor and sphincter dysfunction were much slower to improve. Differential diagnosis of paraganglioma based on MR images alone is challenging, but the presence of specific characteristic features provides suggestive clues; however, accurate diagnosis depends on pathological criteria. Despite the benign course, gross total resection is ideal, given an increased risk of recurrence in situ. Timely recognition and surgical treatment should be emphasized to avoid progressive neurological deficits.
脊柱副神经节瘤是极其罕见的肿瘤,最常累及马尾和终丝。我们旨在研究原发性脊柱副神经节瘤的临床表现、影像学特征、治疗方法及随访数据。我们展示了19例经病理诊断为脊柱副神经节瘤患者的连续手术系列的临床资料和长期预后。所有患者均接受了手术切除。进行了术前和术后磁共振成像,并展示和讨论了随访数据及神经功能评估。诊断时的平均年龄为47.7岁,男性明显居多。主要临床症状为腰痛和坐骨神经痛。磁共振图像(MRI)显示了有助于将副神经节瘤与其他脊柱肿瘤区分开来的特征性表现,包括“椒盐”征、蜿蜒状血流空洞和周边低信号环。此外,术中可见其包膜完整。在平均62.1个月的随访期内,3例切除不完全的患者MRI显示残留肿瘤进展。手术干预后疼痛症状立即缓解,而运动和括约肌功能障碍改善较慢。仅根据MR图像对副神经节瘤进行鉴别诊断具有挑战性,但特定特征的存在提供了提示线索;然而,准确诊断取决于病理标准。尽管病程良性,但鉴于原位复发风险增加,理想的情况是进行全切除。应强调及时识别和手术治疗以避免进行性神经功能缺损。