Yang Chenlong, Fang Jingyi, Li Guang, Jia Wenqing, Liu Hai, Qi Wei, 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.
Department of Neuro-pathology, Beijing Neurosurgical Institute, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China.
J Neurooncol. 2016 Apr;127(2):279-86. doi: 10.1007/s11060-015-2006-8. Epub 2016 Mar 3.
Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. The aim of this study was to investigate the clinical manifestations, radiological features, management, and follow-up data of spinal meningeal melanocytomas. We present the clinical data and long-term outcomes from a consecutive surgical series of 16 patients with pathologically diagnosed spinal meningeal melanocytomas. All of the patients underwent surgical resection. Pre and postoperative MRI was performed. Follow-up data and neurological functional assessment is presented and discussed. The mean age at diagnosis was 42.0 years, with a significant male predominance. The primary clinical symptoms were weakness or numbness of the extremities. The appearance of melanocytoma on MRI is typically isointense to hyperintense on T1-weighted images, hypointense on T2-weighted images, and contrast enhancement tends to be remarkable and homogeneous. In most cases, gross total resection is achievable; however, in rare cases with dumbbell-shaped tumors involving the extraspinal region, a staging operation and subtotal resection should be attempted. During a mean follow-up period of 58.1 months, the symptoms were completely relieved in all the patients, and no tumor progression or recurrence was noted. Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.
脑膜黑素细胞瘤是一种罕见的良性肿瘤,最常位于后颅窝和椎管内。本研究的目的是探讨脊柱脑膜黑素细胞瘤的临床表现、影像学特征、治疗方法及随访数据。我们展示了16例经病理诊断为脊柱脑膜黑素细胞瘤患者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。术前行磁共振成像(MRI)检查,术后也进行了MRI检查。并展示和讨论了随访数据及神经功能评估结果。诊断时的平均年龄为42.0岁,男性明显居多。主要临床症状为肢体无力或麻木。黑素细胞瘤在MRI上的表现通常在T1加权图像上呈等信号至高信号,在T2加权图像上呈低信号,且对比增强往往显著且均匀。在大多数情况下,可以实现肿瘤全切;然而,在罕见的累及椎管外区域的哑铃形肿瘤病例中,应尝试分期手术和次全切除。在平均58.1个月的随访期内,所有患者症状均完全缓解,未发现肿瘤进展或复发。中枢神经系统黑素细胞肿瘤在MRI扫描上有典型表现,随黑色素的含量和分布而变化。然而,恶性黑色素瘤和黑素细胞瘤的鉴别诊断仍取决于病理标准。脊柱脑膜黑素细胞瘤病程良性,适合肿瘤全切。完全切除后预后良好。