神经纤维瘤病的临床体征影响恶性外周神经鞘瘤的预后。
Clinical signs of neurofibromatosis impact on the outcome of malignant peripheral nerve sheath tumors.
机构信息
Clinical Division of Oncology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
出版信息
Oncology. 2014;86(2):122-6. doi: 10.1159/000357137. Epub 2014 Jan 28.
OBJECTIVE
Malignant peripheral nerve sheath tumors (MPNST) are a rare subtype of sarcoma, with a poor outcome. MPNST are regarded as being sporadic or associated with neurofibromatosis type 1 (NF1). Few comparative overall-survival (OS) data in these 2 subsets of MPNST patients exist. The aim of this retrospective study was to assess OS in sporadic and NF1-associated MPNST patients.
METHODS
Fourteen consecutive patients with initial localized as well as initial metastatic MPNST were diagnosed and treated in our department. Patients with sporadic MPNST were assigned to group A and those with NF1-associated MPNST to group B.
RESULTS
Eight versus 6 patients were allocated to groups A and B. Primary tumors were located on the extremities in all but 1 patient. Two patients in group A and 4 patients in group B experienced a relapse. Four patients died in each of the 2 groups. Median follow-up was 66.2 and 57.2 months in group A and group B, respectively. Median OS in group A was 46.9 months versus 12.7 months in group B.
CONCLUSIONS
In this small, single-center study, sporadic-MPNST patients had a longer median OS than those with NF1-associated MPNST.
目的
恶性外周神经鞘瘤（MPNST）是一种罕见的肉瘤亚型，预后较差。MPNST 被认为是散发性的或与神经纤维瘤病 1 型（NF1）相关。这两种 MPNST 患者亚组的总生存（OS）数据很少。本回顾性研究旨在评估散发性和 NF1 相关 MPNST 患者的 OS。
方法
在我院诊断并治疗了 14 例初发局限性和初发转移性 MPNST 的连续患者。将散发性 MPNST 患者分配到 A 组，NF1 相关 MPNST 患者分配到 B 组。
结果
A 组和 B 组分别有 8 例和 6 例患者。除 1 例患者外，所有患者的原发性肿瘤均位于四肢。A 组中有 2 例患者和 B 组中有 4 例患者复发。A 组和 B 组各有 4 例患者死亡。A 组的中位随访时间为 66.2 个月，B 组为 57.2 个月。A 组的中位 OS 为 46.9 个月，B 组为 12.7 个月。
结论
在这项小型单中心研究中，散发性 MPNST 患者的中位 OS 长于 NF1 相关 MPNST 患者。
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