恶性周围神经鞘膜瘤的临床研究(36例报告)

CLINICAL RESEARCH OF MALIGNANT PERIPHERAL NERVE SHEATH TUMOR(36 CASES REPORT)

为探讨恶性周围神经鞘膜瘤 (MNST)的临床及肿瘤生物学特征。对 36例MNST患者的临床病理资料及随访资料进行统计分析 ,并与国外文献结果作对照。结果显示 ,MNST以 4 0～ 6 0岁年龄组患病比例最高 ,男∶女为 2 6∶1,发病部位依次为躯干 >四肢 >头颈部 ;术后复发率为 4 2 10 % ,5年存活率为 8 33% ;未发现由神经鞘瘤或神经纤维瘤病恶变所致病例 ,也未发现与放疗后恶变有关的病例。提示MNST是一种原发的软组织恶性肿瘤 ,早期手术彻底切除是治疗的关键 。

To study the clinical and biological characteristics of malignant peripheral nerve sheath tumor (MNST), the clinical and pathological data of 36 followed up patients with confirmed diagnosis were statistically analyzed. It was found that the incidence was highest in the age group of 40～60 years, the ratio between male and female was 2.6∶1, the site of the tumor ranked trunk>extremities> head and neck region. Postoperative recurrence rate was 42 10%, five year survival rate was 8 33%. Not a single case was found to be malignant degeneration of neurolemmoma or neurofibromatosis, nor as a result of radiotherapy. It is suggested that MNST is a primary soft tissue malignant tumor, and early complete excision is the key treatment. The biological characteristics of this tumor as revealed in this study suggest that it is different from what was reported in the foreign literature.