Abstract：
OBJECTIVE: Malignant melanotic nerve sheath tumors are rare tumors characterized by neoplastic melanin-producing Schwann cells. In this study, the authors report their institution\'s experience in treating spinal and peripheral malignant melanotic nerve sheath tumors and compare their results with the literature.
METHODS: Data were collected from 8 patients who underwent surgical treatment for malignant melanotic nerve sheath tumors between 1996 and 2023 at Mayo Clinic and 63 patients from the literature. Time-to-event analyses were performed for the combined group of 71 cases to evaluate the risk of recurrence, metastasis, and death based on tumor location and type of treatment received. Unpaired 2-sample t-tests and Fisher\'s exact tests were used to determine statistical significance between groups.
RESULTS: Between 1996 and 2023, 8 patients with malignant melanotic nerve sheath tumors underwent surgery at the authors\' institution, while 63 patients were identified in the literature. The authors\' patients and those in the literature had the same mean age at diagnosis (43 years). At the authors\' institution, 5 patients (63%) experienced metastasis, 6 patients (75%) experienced long-term recurrence, and 5 patients (62.5%) died. In the literature, most patients (60.3%) were males, with a peak incidence between the 4th and 5th decades of life. Nineteen patients (31.1%) were diagnosed with Carney complex. Nerve root tumors accounted for most presentations (n = 39, 61.9%). Moreover, 24 patients (38.1%) had intradural lesions, with 54.2% (n = 13) being intramedullary and 45.8% (n = 11) extramedullary. Most patients underwent gross-total resection (GTR) (n = 41, 66.1%), followed by subtotal resection (STR) (n = 12, 19.4%), STR with radiation therapy (9.7%), and GTR with radiation therapy (4.8%). Sixteen patients (27.6%) experienced metastasis, 23 (39.7%) experienced recurrence, and 13 (22%) died. Kaplan-Meier analyses showed no significant differences among treatment approaches in terms of recurrence-free, metastasis-free, and overall survival (p > 0.05). Similar results were obtained when looking at the differences with respect to intradural versus nerve root location of the tumor (p > 0.05).
CONCLUSIONS: Malignant melanotic nerve sheath tumors are rare tumors with a high potential for malignancy. They carry a dismal prognosis, with a pooled local recurrence rate of 42%, distant metastasis rate of 27%, and mortality rate of 26%. The findings from this study suggest a trend favoring the use of GTR alone or STR with radiation therapy over STR alone. Mortality was similar regardless, which highlights the need for the development of effective treatment options to improve survival in patients with melanotic schwannomas.
METHODS: Data were collected from 8 patients who underwent surgical treatment for malignant melanotic nerve sheath tumors between 1996 and 2023 at Mayo Clinic and 63 patients from the literature. Time-to-event analyses were performed for the combined group of 71 cases to evaluate the risk of recurrence, metastasis, and death based on tumor location and type of treatment received. Unpaired 2-sample t-tests and Fisher\'s exact tests were used to determine statistical significance between groups.
RESULTS: Between 1996 and 2023, 8 patients with malignant melanotic nerve sheath tumors underwent surgery at the authors\' institution, while 63 patients were identified in the literature. The authors\' patients and those in the literature had the same mean age at diagnosis (43 years). At the authors\' institution, 5 patients (63%) experienced metastasis, 6 patients (75%) experienced long-term recurrence, and 5 patients (62.5%) died. In the literature, most patients (60.3%) were males, with a peak incidence between the 4th and 5th decades of life. Nineteen patients (31.1%) were diagnosed with Carney complex. Nerve root tumors accounted for most presentations (n = 39, 61.9%). Moreover, 24 patients (38.1%) had intradural lesions, with 54.2% (n = 13) being intramedullary and 45.8% (n = 11) extramedullary. Most patients underwent gross-total resection (GTR) (n = 41, 66.1%), followed by subtotal resection (STR) (n = 12, 19.4%), STR with radiation therapy (9.7%), and GTR with radiation therapy (4.8%). Sixteen patients (27.6%) experienced metastasis, 23 (39.7%) experienced recurrence, and 13 (22%) died. Kaplan-Meier analyses showed no significant differences among treatment approaches in terms of recurrence-free, metastasis-free, and overall survival (p > 0.05). Similar results were obtained when looking at the differences with respect to intradural versus nerve root location of the tumor (p > 0.05).
CONCLUSIONS: Malignant melanotic nerve sheath tumors are rare tumors with a high potential for malignancy. They carry a dismal prognosis, with a pooled local recurrence rate of 42%, distant metastasis rate of 27%, and mortality rate of 26%. The findings from this study suggest a trend favoring the use of GTR alone or STR with radiation therapy over STR alone. Mortality was similar regardless, which highlights the need for the development of effective treatment options to improve survival in patients with melanotic schwannomas.
摘要：
目的：恶性黑色素神经鞘瘤是一种罕见的肿瘤，其特征是产生黑色素的雪旺细胞。在这项研究中,作者报告了他们的机构在治疗脊柱和周围恶性黑素性神经鞘瘤方面的经验，并将其结果与文献进行了比较。
方法：数据收集自1996年至2023年在梅奥诊所接受恶性黑色素神经鞘瘤手术治疗的8例患者和文献中的63例患者。对71例合并组进行事件发生时间分析，以评估复发风险。转移,以及根据肿瘤位置和接受治疗类型的死亡。使用非配对2样本t检验和Fisher精确检验来确定组间的统计学显著性。
结果：在1996年至2023年之间，有8例恶性黑素性神经鞘瘤患者在作者机构接受了手术，而文献中确定了63例患者。作者的患者和文献中的患者在诊断时的平均年龄相同（43岁）。在作者机构，5例患者（63%）发生转移，6例患者（75％）经历了长期复发，死亡5例(62.5%)。在文学中,大多数患者(60.3%)是男性，在生命的第4到第5个十年之间发病率最高。19例患者（31.1％）被诊断为卡尼复合体。神经根瘤占大多数（n=39，61.9%）。此外，24例（38.1%）有硬膜内病变，其中54.2%（n=13）为髓内，45.8%（n=11）为髓外。大多数患者接受了全切除（GTR）（n=41，66.1％），其次是次全切除术（STR）（n=12，19.4%），STR与放射治疗（9.7％），和GTR+放射治疗(4.8%)。16例患者（27.6%）出现转移，23例（39.7%）复发，13人（22%）死亡。Kaplan-Meier分析显示，在无复发方面，治疗方法之间没有显着差异。无转移,和总生存期(p>0.05)。当观察关于肿瘤的硬膜内与神经根位置的差异时,获得了类似的结果(p>0.05)。
结论：恶性黑色素神经鞘瘤是一种具有高度恶性潜能的罕见肿瘤。他们的预后很差，合并的局部复发率为42%，远处转移率为27%，死亡率为26%。这项研究的结果表明，与单独使用STR相比，倾向于单独使用GTR或STR进行放射治疗。死亡率是相似的，无论如何，这突出了需要开发有效的治疗方案来改善黑色素神经鞘瘤患者的生存率。
方法：数据收集自1996年至2023年在梅奥诊所接受恶性黑色素神经鞘瘤手术治疗的8例患者和文献中的63例患者。对71例合并组进行事件发生时间分析，以评估复发风险。转移,以及根据肿瘤位置和接受治疗类型的死亡。使用非配对2样本t检验和Fisher精确检验来确定组间的统计学显著性。
结果：在1996年至2023年之间，有8例恶性黑素性神经鞘瘤患者在作者机构接受了手术，而文献中确定了63例患者。作者的患者和文献中的患者在诊断时的平均年龄相同（43岁）。在作者机构，5例患者（63%）发生转移，6例患者（75％）经历了长期复发，死亡5例(62.5%)。在文学中,大多数患者(60.3%)是男性，在生命的第4到第5个十年之间发病率最高。19例患者（31.1％）被诊断为卡尼复合体。神经根瘤占大多数（n=39，61.9%）。此外，24例（38.1%）有硬膜内病变，其中54.2%（n=13）为髓内，45.8%（n=11）为髓外。大多数患者接受了全切除（GTR）（n=41，66.1％），其次是次全切除术（STR）（n=12，19.4%），STR与放射治疗（9.7％），和GTR+放射治疗(4.8%)。16例患者（27.6%）出现转移，23例（39.7%）复发，13人（22%）死亡。Kaplan-Meier分析显示，在无复发方面，治疗方法之间没有显着差异。无转移,和总生存期(p>0.05)。当观察关于肿瘤的硬膜内与神经根位置的差异时,获得了类似的结果(p>0.05)。
结论：恶性黑色素神经鞘瘤是一种具有高度恶性潜能的罕见肿瘤。他们的预后很差，合并的局部复发率为42%，远处转移率为27%，死亡率为26%。这项研究的结果表明，与单独使用STR相比，倾向于单独使用GTR或STR进行放射治疗。死亡率是相似的，无论如何，这突出了需要开发有效的治疗方案来改善黑色素神经鞘瘤患者的生存率。
