Miami Project to Cure Paralysis, Department of Neurological Surgery, Miller School of Medicine, University of Miami, Miami, Florida.
Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota.
Neurosurgery. 2019 Dec 1;85(6):E975-E991. doi: 10.1093/neuros/nyz321.
Synovial sarcoma of the nerve is a rare entity with several cases and case series reported in the literature. Despite an improved understanding of the biology, the clinical course is difficult to predict.
To compile a series of patients with synovial sarcoma of the peripheral nerve (SSPN) and assess clinical and pathological factors and their contribution to survival and recurrence.
Cases from 2 institutions collected in patients undergoing surgical intervention for SSPN. Systematic review including PubMed and Scopus databases were searched for related articles published from 1970 to December 2018. Eligibility criteria: (1) case reports or case series reporting on SSPN, (2) clinical course and/or pathological features of the tumor reported, and (3) articles published in English.
From patients treated at our institutions (13) the average follow-up period was 3.2 yr. Tumor recurrence was seen in 4 cases and death in 3. Systematic review of the literature yielded 44 additional cases with an average follow-up period of 3.6 yr. From pooled data, there were 10 recurrences and 7 deaths (20% and 14%, respectively). Adjuvant treatment used in 62.5% of cases. Immunohistochemical markers used in diagnosis varied widely; the most common are the following: Epithelial membrane antigen (EMA), cytokeratin, vimentin, cluster of differentiation (CD34), and transducin-like enhancer of split 1 (TLE1). Statistical analysis illustrated tumor size and use of chemotherapy to be negative predictors of survival. No other factors, clinically or from pathologist review, were correlated with recurrence or survival.
By combining cases from our institution with historical data and performing statistical analysis we show correlation between tumor size and death.
神经滑膜肉瘤是一种罕见的实体瘤，文献中有几例病例和病例系列报道。尽管对生物学有了更好的了解，但临床病程仍难以预测。
收集一系列周围神经滑膜肉瘤（SSPN）患者的资料，评估临床和病理因素及其对生存和复发的影响。
在 2 家机构中收集接受手术治疗的 SSPN 患者的病例。系统检索了从 1970 年至 2018 年 12 月发表的相关文献，包括 PubMed 和 Scopus 数据库。纳入标准：（1）报道 SSPN 的病例报告或病例系列，（2）报道肿瘤的临床过程和/或病理特征，（3）以英文发表的文章。
在我院治疗的患者（13 例）的平均随访期为 3.2 年。4 例出现肿瘤复发，3 例死亡。对文献的系统回顾得出了另外 44 例病例，平均随访期为 3.6 年。从汇总数据来看，有 10 例复发，7 例死亡（分别为 20%和 14%）。62.5%的病例采用辅助治疗。诊断中使用的免疫组化标志物差异很大；最常见的有以下几种：上皮膜抗原（EMA）、细胞角蛋白、波形蛋白、CD34 和转导素样增强子分裂 1（TLE1）。统计分析表明肿瘤大小和化疗的使用是生存的负预测因子。没有其他因素，无论是临床还是病理医生评估，都与复发或生存相关。
通过将我院的病例与历史数据相结合并进行统计分析，我们显示肿瘤大小与死亡之间存在相关性。
