MALIGNANT PERIPHERAL-NERVE SHEATH TUMORS - A CLINICOPATHOLOGICAL STUDY OF 120 CASES
MALIGNANT PERIPHERAL-NERVE SHEATH TUMORS - A CLINICOPATHOLOGICAL STUDY OF 120 CASES
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DOI：
10.1002/1097-0142(19860515)57:10
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发表时间：
1986-05-15
期刊：
影响因子：
6.2
通讯作者：
ILSTRUP, DM
中科院分区：
文献类型：
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作者：
DUCATMAN, BS;SCHEITHAUER, BW;ILSTRUP, DM
我们回顾了71年来120例恶性周围神经鞘肿瘤（MPNST）。120例患者中，男性52例，女性68例，平均诊断年龄35.3岁；12例患者年龄小于20岁。该系列包括62例（52%）神经纤维瘤病患者，13例（11%）放射后肉瘤患者，19例（16%）化生灶患者。在本系列研究中，神经纤维瘤病中MPNST的发生率为4.6%，在普通临床人群中为0.001%。肿瘤bbb5 cm及存在神经纤维瘤病对预后有不利影响（P < 0.05）。当这两种特征同时存在时，生存率大大降低。四肢有肿瘤的患者比头颈部有病变的患者表现更好。肿瘤部位的化生灶或既往放疗均未改变预后。每个肿瘤根据细胞结构、多形性、有丝分裂指数和坏死程度分为1 ~ 4级。生存率与分级或有丝分裂率均无显著相关性。全切除比次全切除能提高生存率。这在小病变的患者中最为明显，这可能反映了充分切除大肿瘤的困难。辅助放疗或化疗似乎不影响生存。MPNST是一种侵袭性的罕见肿瘤，肿瘤体积大、存在神经纤维瘤病和完全切除是最重要的预后指标。
A review was done of 120 cases of malignant peripheral nerve sheath tumor (MPNST) seen during a 71-year period. Of the 120 patients, 52 were males and 68 were females with a mean age at diagnosis of 35.3 years; 12 patients were younger than 20 years. The series included 62 (52%) patients with neurofibromatosis, 13 (11%) with postradiation sarcomas, and 19 (16%) with metaplastic foci. The incidence of MPNST arising in neurofibromatosis was 4.6% in the current series and 0.001% in the general clinic population. Tumors > 5 cm and the presence of neurofibromatosis adversely affected the prognosis (P < 0.05). When both features were present, survival was greatly decreased. Patients with tumor in the extremities did better than those with head or neck lesions. Metaplastic foci or previous radiation at the tumor site did not alter the prognosis. Each tumor was graded 1 to 4 on the basis of cellularity, pleomorphism, mitotic index, and necrosis. No significant correlation was noted between survival and either grade or mitotic rate. Survival was improved when total rather than subtotal resection was done. This was most marked in patients with a small lesion, which may reflect the difficulty in adequately excising large tumors. Adjuvant radiation or chemotherapy did not appear to affect survival. The MPNST is an aggressive uncommon neoplasm, and large tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators.