Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors
Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors
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DOI：
10.1007/s11060-006-9266-2
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发表时间：
2007-04-01
影响因子：
3.9
通讯作者：
Mautner, Victor Felix
中科院分区：
文献类型：
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作者：
Hagel, Christian;Zils, Ulrich;Mautner, Victor Felix
回顾性研究了散发性和1型神经纤维瘤病（NF 1）相关的恶性外周神经鞘瘤（MPNST）的临床病程和组织病理学差异。集体包括38名NF 1患者和14名散发患者。NF 1患者在诊断时明显年轻（p < 0.001），并且生存时间明显短于散发患者（中位生存期17个月vs.42个月，Breslow p < 0.05）。NF 1患者的局部复发和转移扩散的时间间隔也明显较短（分别为9.4个月vs. 30.0个月，p < 0.01; 9.1个月vs. 33.2个月，p < 0.001）。在具有原始组织病理学数据的患者中（22例NF 1患者，14例散发病例），与散发肿瘤相比，NF 1相关MPNST显示出显著更高的细胞构成（p < 0.001），而散发MPNST具有显著更高的多形性（p < 0.01）。最重要的是，虽然组织病理学变量与散发性MPNST的法国国家癌症防治中心分级相关，但NF 1相关肿瘤的情况并非如此。NF 1相关和散发性MPNST在临床过程和组织病理学方面的差异可能反映了两组肿瘤在生物学和病理机制方面的一些根本差异。我们的研究结果表明，需要一个单独的分级计划，考虑到遗传背景的NF 1患者。
The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively. The collective comprised 38 NF1 patients and 14 sporadic patients. NF1 patients were significantly younger at diagnosis (p < 0.001) and had a significantly shorter survival time than sporadic patients (median survival 17 months vs. 42 months, Breslow p < 0.05). The time interval to local recurrence and metastatic spread was also significantly shorter in NF1 patients (9.4 months vs. 30.0 months, p < 0.01; 9.1 months vs. 33.2 months, p < 0.001, respectively). In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p < 0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p < 0.01). Most importantly, while histopathological variables correlated with French Federation Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors. The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups. Our findings indicate the necessity for a separate grading scheme which takes into account the genetic background in NF1 patients.