文章摘要
原发性椎管内黑色素瘤与色素性神经鞘瘤的临床特征分析
Analysis of the Clinical Characteristics of Primary Intracanal Melanoma and Pigmented Schwannoma
投稿时间:2025-06-23  修订日期:2025-06-23
DOI:
中文关键词: 原发性椎管内黑色素瘤  原发性椎管内色素性神经鞘瘤  疾病特征  显微外科手术  预后
英文关键词: primary intravertebral melanoma  primary intravertebral pigmentogenic schwannomas  diease attributes  microsurgery  prognosis
基金项目:
作者单位邮编
李润林 武汉科技大学附属天佑医院 430064
刘胜文 华中科技大学同济医学院附属同济医院 
王煜* 华中科技大学同济医学院附属同济医院 430030
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中文摘要:
      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、鉴别诊断及手术疗效及预后。 方法 回顾性纳入2014年6月至2024年6月华中科技大学同济医学院附属同济医院神经外科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,表现为肢体乏力、肢体麻木、颈部及腰背部疼痛。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T1W1等或高信号,T2W1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(140±46)min(80~212min),术中失血量为(260±100)ml(100~600ml),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(150±48)min(100~228min),术中失血量(250±80) ml(120~580ml),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(每3个月行脊柱增强MRI及全身PET-CT评估,每6个月行脑部MRI筛查转移),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(脑转移2例,肺转移2例),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,无失访病例。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(每6-12个月脊柱MRI平扫+增强),所有患者随访时间12-60个月(中位38个月),均存活且无肿瘤复发及转移证据,1例患者出现轻度神经根刺激症状,经复查MRI排除复发,考虑为术后粘连所致,7例患者均坚持完成预设随访周期。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为严重,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,所以定期随访非常重要。
英文摘要:
      Objective:To investigate the clinical features, differential diagnosis, operative effect and prognosis of primary intravertebral melanoma and pigmented schwannoma.Methods:Clinical data of 5 patients with primary intravertebral melanoma and 7 patients with primary intravertebral pigmentary schwannoma who were surgically treated and confirmed by pathology in the Department of Neurosurgery, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology from June 2014 to June 2024 were retrospectively included. Clinical characteristics, differential diagnosis, surgical efficacy and prognosis of the two diseases were analyzed.Results:The clinical characteristics of the 12 patients were not specific, and the symptoms of spinal cord injury were mainly manifested in the corresponding pathological segments, including limb weakness, limb numbness, neck and back pain. Routine MRI examination of 5 cases of primary intravertebral melanoma and 7 cases of primary intravertebral pigmented schwannoma showed equal or high signal T1W1 and equal or low signal T2W1. There are certain differences in MRI enhancement. Primary intravertebral melanoma usually presents uneven enhancement with unclear boundaries, which can invade adjacent tissues, while primary intravertebral pigmented schwannomas are mostly uniform enhancement with relatively clear boundaries. The operative time of 5 patients with primary intramural melanoma was (140±46)min(80 ~ 212min), the intraoperative blood loss was (260±100)ml(100 ~ 600ml), and no death occurred during the perioperative period. Postoperative MRI showed total resection in 2 cases, subtotal resection in 2 cases and partial resection in 1 case. The operative time of 7 patients with primary intravertebral pigmentogenic schwannomas was (150±48)min(100 ~ 228min), the intraoperative blood loss was (250±80) ml(120 ~ 580ml), and there were no deaths during the perioperative period. Postoperative MRI showed total resection in 7 cases . Five patients with primary intraspinal melanoma were followed up regularly after surgery (spinal enhanced MRI and whole-body PET-CT were performed every 3 months, and brain MRI was performed every 6 months to screen for metastasis). Four patients died within 3-15 months after discharge, with a median survival of 8.5 months. The cause of death was distant metastasis (2 brain metastases and 2 lung metastases). One patient had a survival of more than 24 months. As of the last follow-up (June 2024), no signs of local recurrence or metastasis were found. All patients completed at least 6 months of follow-up, and no cases were lost to follow-up. Seven patients with primary intraspinal pigmented schwannoma underwent regular postoperative imaging monitoring (spine MRI plain scan + enhancement every 6-12 months). All patients were followed up for 12-60 months (median 38 months), and all survived without evidence of tumor recurrence and metastasis. One patient had mild nerve root irritation symptoms, and the recurrence was ruled out by MRI review. It was considered to be caused by postoperative adhesion. All 7 patients insisted on completing the preset follow-up cycle.Conclusion:The clinical features of primary intraspinal melanoma and pigmentary schwannoma are not specific, and MRI is feasible for preliminary differential diagnosis, which should be distinguished by pathological findings.Primary intraspinal melanoma is more serious than pigmented schwannoma. Total microscopic resection of the tumor is the first choice. Postoperative combined with chemoradiotherapy can prolong the survival of patients. Pigmented schwannomas are mostly benign tumors, which can generally achieve total tumor resection, and generally do not need radiotherapy and chemotherapy after surgery, but the prognosis is difficult to predict in the later stage, so regular follow-up is very important.
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