目的：探讨原发性椎管内黑色素瘤与色素性神经鞘瘤的临床特征、鉴别诊断、手术疗效及预后。方 法：回顾性纳入采用手术治疗且经病理学证实的5例原发性椎管内黑色素瘤、7例原发性椎管内色素性神 经鞘瘤患者临床资料，分析两种疾病临床特征、鉴别诊断要点、手术疗效及预后。结果：12例患者临床特征 不具特异性，均表现为相应病变节段脊髓损害症状为主。5例原发性椎管内黑色素瘤、7例原发性椎管内色 素性神经鞘瘤常规MRI检查均大体表现为相应节段T1WI等或高信号，T2WI等或低信号。在MRI增强检 查上存在一定区别，原发性椎管内黑色素瘤通常表现为不均匀强化，边界不清，可对临近组织造成侵犯，原 发性椎管内色素性神经鞘瘤多为均匀强化，边界相对清楚。5例原发性椎管内黑色素瘤手术时间为80～ 212 min，平均（140±46）min；术中失血量为100～600 mL，平均（260±100）mL；围术期无死亡病例。术后复查 MRI显示全切除2例、次全切除2例、部分切除1例。7例原发性椎管内色素性神经鞘瘤手术时间为100～ 228 min，平均（150±48）min；术中失血量120～580 mL，平均（250±80）mL；围术期无死亡病例。术后复查 MRI显示7例均全切除。5例原发性椎管内黑色素瘤患者术后规律随访，4例患者于出院后3～15个月内死 亡，中位生存期为8.5个月，死因均为远处转移。1例患者生存期已超过24个月，截止末次随访（2024年6 月）未见局部复发或转移征象，所有患者均完成至少6个月随访。7例原发性椎管内色素性神经鞘瘤患者 术后定期影像学监测，所有患者随访时间12～60个月，均存活且无肿瘤复发及转移证据。结论：原发性椎 管内黑色素瘤与色素性神经鞘瘤临床特征不具特异性，MRI可行初步鉴别诊断，需依赖病理结果加以鉴别， 原发性椎管内黑色素瘤较色素性神经鞘瘤更严重，显微全切除肿瘤是首选方案，术后联合放化疗可延长患 者的生存期。色素性神经鞘瘤多为良性肿瘤，一般可达到肿瘤的全切除，术后一般无需放化疗，但后期预后 难以预测。

To explore the clinical characteristics, differential diagnosis, surgical outcomes, and prognosis of primary intraspinal melanoma and pigmented neurilemmoma. Methods: A retrospective analysis was conducted on the clinical data of 5 patients with primary intraspinal melanoma and 7 patients with primary intraspinal pigmented neurilemmoma, all of whom underwent surgical treatment and were pathologically confirmed. The clinical characteristics, key points of differential diagnosis, surgical outcomes, and prognosis of these two diseases were analyzed. Results: The 12 patients exhibited non-specific clinical characteristics, primarily presenting with symptoms of spinal cord impairment at the corresponding lesion levels. On conventional MRI, both the 5 cases of primary intraspinal melanoma and the 7 cases of primary intraspinal pigmented neurilemmoma generally showed isointense or hyperintense signals on T1WI and isointense or hypointense signals on T2WI at the corresponding levels. There were certain differences in MRI contrast-enhanced imaging: primary intraspinal melanoma typically demonstrated heterogeneous enhancement with ill-defined borders and invasion of adjacent tissues, while primary intraspinal pigmented neurilemmoma mostly showed homogeneous enhancement with relatively well-defined borders. For the 5 cases of primary intraspinal melanoma, the surgical duration ranged from 80 to 212 minutes, with an average of (140 ± 46) minutes; intraoperative blood loss ranged from 100 to 600 mL, with an average of (260±100) mL; and there were no perioperative deaths. Postoperative MRI revealed complete resection in 2 cases, subtotal resection in 2 cases, and partial resection in 1 case. For the 7 cases of primary intraspinal pigmented neurilemmoma, the surgical duration ranged from 100 to 228 minutes, with an average of (150±48) minutes; intraoperative blood loss ranged from 120 to 580 mL, with an average of (250±80) mL; and there were no perioperative deaths. Postoperative MRI showed complete resection in all 7 cases. Among the 5 patients with primary intraspinal melanoma who were regularly followed up after surgery, 4 patients died within 3 to 15 months after discharge, with a median survival time of 8.5 months. The cause of death in all cases was distant metastasis. One patient had a survival time exceeding 24 months and showed no signs of local recurrence or metastasis as of the last follow-up (June 2024). All patients completed at least 6 months of follow-up. The 7 patients with primary intraspinal pigmented neurilemmoma underwent regular postoperative imaging monitoring. All patients were followed up for 12 to 60 months, and all were alive with no evidence of tumor recurrence or metastasis. Conclusion: Primary intraspinal melanoma and pigmented neurilemmoma exhibit non-specific clinical characteristics. MRI can be used for preliminary differential diagnosis, but pathological results are required for definitive identification. Primary intraspinal melanoma is more severe than pigmented neurilemmoma. Microscopic total tumor resection is the preferred treatment option, and postoperative adjuvant radiotherapy and chemotherapy can prolong patient survival. Pigmented neurilemmoma is mostly a benign tumor, and total tumor resection can generally be achieved. Postoperative radiotherapy and chemotherapy are usually not required, but long-term prognosis is difficult to predict.