Malignant peripheral nerve sheath tumors - Prognostic factors and survival in a series of patients treated at a single institution
Malignant peripheral nerve sheath tumors - Prognostic factors and survival in a series of patients treated at a single institution
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DOI：
10.1002/cncr.22098
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发表时间：
2006-09-01
期刊：
影响因子：
6.2
通讯作者：
Gronchi, Alessandro
中科院分区：
文献类型：
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作者：
Anghileri, Matteo;Miceli, Rosalba;Gronchi, Alessandro
背景。作者探讨了伴有和不伴有I型神经纤维瘤病（NF-1）的恶性周围神经鞘肿瘤（MPNST）患者的预后因素和临床结果。本文回顾了25年来在意大利米兰国立肿瘤研究所（Istituto Nazionale per Studio e la Cura dei Tumori）接受手术治疗的295例局限性MPNST患者。46例患者伴有NF-1综合征，159例患者无NF-1综合征。研究了局部复发率、远处转移率和生存率。130例患者表现为原发疾病，75例患者有局部复发肿瘤。10年疾病特异性死亡率为43%，连续无病生存率不大于40%。原发或复发疾病的表现、肿瘤大小和肿瘤部位（躯干vs四肢）是生存最强的独立预测因子。边缘状态和放射治疗也起作用，主要与它们对局部结果的影响有关。病理分级影响远处转移，但只能观察到生存趋势。有NF-1和没有NF-1的患者之间没有显著的独立差异。据作者所知，这是迄今为止最大的单机构系列研究之一。结果证实，MPNST患者与其他软组织肉瘤患者具有相似的预后因素，并且具有一些最差的临床结果。NF-1综合征本身的存在并不影响生存，但NF-1患者更有可能有更大的肿瘤。因此，这类患者应仔细随访，尽早发现疾病。
BACKGROUND. The authors explored the prognostic factors and clinical outcomes of patients who had malignant peripheral nerve sheath tumors (MPNST) with and without neurofibromatosis type I (NF-1).METHODS. Two hundred five patients with localized MPNST who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed. Forty-six patients had concomitant NF-1 syndrome, and 159 patients did not. Local recurrence, distant metastases, and survival rates were studied.RESULTS. One hundred thirty patients presented with primary disease, and 75 patients had locally recurrent tumors. The disease - specific mortality rate was 43% at 10 years, with a continuously disease-free survival rate of no greater than 40%. Presentation with either primary or recurrent disease, tumor size, and tumor site (trunk vs. extremity) were the strongest independent predictors of survival. Margin status and radiation therapy also played a role, mostly related to their effect on local outcome. Pathologic grade influenced distant metastases, but only a trend for survival could be observed. No significant independent differences between patients with and without NF-1 were observed.CONCLUSIONS. To the authors' knowledge, this was among the largest single-institution series to date. The results confirmed that patients with MPNST share similar prognostic factors with patients who have other soft tissue sarcomas and have some of the worst clinical outcomes. The presence of NF-1 syndrome per se did not affect survival, but patients with NF-1 were more likely to have larger tumors. Therefore, such patients should be followed carefully to detect disease as early as possible.