Malignant Peripheral Nerve Sheath Tumor-An Analysis of 33 Cases and Literature Review

惡性周邊神經腱鞘瘤

吳傳壽(Chuan-Shou Wu) ； 方榮煌(Rong-Hwang Fang) ； 趙靜宜(Jing-Yi Chao) ； 葉發來(Fa-Lai Yeh) ； 林進德(Jin-The Li) ； 黃志宏(Chih-Hung Hwang) ； 沈秉輝(Bing-Hwei Shen) ； 顏上惠(Sang-Hue Yen) ； 馬旭(Hsu Ma)

《中華民國整形外科醫學會雜誌》 9卷4期 (2000/12) Pp. 225-234

https://doi.org/10.7096/JPSA.200012.0225

摘要

惡性周邊神經腱鞘瘤是一種少見的腫瘤，約只佔軟組織肉瘤10%。我們將1976年到1999年在台北榮總經病理診斷為惡性周邊神經腱鞘瘤、惡性神經鞘瘤、神經性肉瘤、纖維神經肉瘤等的病人做回顧分析，探討有關惡性周邊神精腱鞘瘤的臨床特性，存活率以及對治療的反應。總共有三十三個病人，包括十八個男性及十五個女性。平均診斷時年齡為39.6歲。所有病人皆接受外科手術治療。其中70%的病人接受輔助治療。十二個病人患有纖維神經瘤病。十個病人可以找出腫瘤的神經來源。平均的最大腫瘤直徑為8.2公分。主要的臨床症狀為腫塊。二年及五年的存活率各為65%及25%。病人的存活與年齡、腫瘤的大小、腫瘤位置、手術方法及手術後病灶邊緣狀態有關。患有纖維神經瘤病的病人在存活率沒有統計意義，但平均存活月數較差。
我們的結果顯示惡性周邊神經腱鞘瘤是一種極惡性但少見的腫瘤。而對於患有纖維神經瘤病的病人應該密切的追蹤是否產生此惡性腫瘤。對於大部分的病人，積極的外科治療是達到增加存活率最主要的方法。

並列摘要

Malignant peripheral nerve sheath tumor (MPNST) is a rare tumor which makes up 10% all of soft tissue sarcomas. A retrospective study was undertaken to review the clinical characteristics, survival, and response to therapy. A total of 33 patients with malignant peripheral nerve sheath tumor, malignant neurilemoma, neurogenic sarcoma, neurofibrosarcoma, or malignant schwannoma histologically confirmed by a pathologist, between September 1976 and September 1999 at VGH-Taipei, was reviewed. Of the 33 patients, 18 were males and 15 were females with a mean age at diagnosis of 39.6 years. Twelve (36%) of the patients had neurofibromatosis, whereas 21 (64%) did not. A nerve of origin was identified in 10 (33%) of these patients. The mean greatest dimension of a tumor was 8.2 cm. The major clinical symptom was a palpable mass. Surgical resection was the main modality of treatment for all patients; 70% also received adjuvant therapy. The total survival rate was 65% at 2 years and 25% at 5 years, respectively. The median survival time for the entire sample was 34 months. Both overall survival times were significantly influenced by age, tumor size, tumor location, surgical method, and surgical margin status. No definite significant difference in survival rates between patients with and without NF-I was noted (P=0.076), but the median survival time appeared to be shorter for the group of NF-J patients (18 vs. 57 months). Adjuvant radiation therapy and chemotherapy did not affect survival rates.
The present results indicate that the MPNST is an uncommon aggressive neoplasm, and it is recommended that patients with NF-I be closely followed up for MPNST development. For most cases, treatment should include aggressive surgery with a free surgical margin.

並列關鍵字

Malignant peripheral tissue nerve sheath tumor (MPNST) ； soft tissue sarcoma ； neurofibromatosis wide excision ； survival