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15 岁男性正中神经内滑膜肉瘤 1 例报告并文献复习

Intraneural Synovial Sarcoma of the Median Nerve in a 15-Year-Old Male: A Case Report and Review of Literature.

机构信息

Brody School of Medicine, East Carolina University, Greenville, NC, USA.

出版信息

Hand (N Y). 2022 Mar;17(2):NP1-NP5. doi: 10.1177/1558944720975139. Epub 2021 Jan 7.

Abstract

There have been 8 synovial sarcomas of the median nerve reported. We report a case of a 15-year-old male with synovial sarcoma of the right-hand median nerve. Patient presented with a 2-month history of enlarging mass at the base of the right thenar eminence associated with numbness in the median nerve distribution. Physical examination revealed a soft mass over the thenar eminence and paresthesia in the median nerve distribution. He underwent excision of the tumor, which revealed a well-encapsulated lesion encompassing the median nerve, involving the first, second, and radial aspect of the third web space as well as recurrent branches of the median nerve. Following excision of the tumor, a thorough metastatic workup was negative for metastatic disease. He was staged as III, T2b, N0, M0-poorly differentiated monophasic synovial sarcoma of the right median nerve. Postoperatively the patient was started on chemotherapy and radiation. Intraneural synovial sarcoma is extremely rare. Our case is the youngest with the longest follow-up. He is currently at a status of 3 years posttreatment with no signs of recurrence and excellent use of his right hand. This case is of particular interest due to the rarity of the disease along with this being the best outcome reported in the literature to-date.

摘要

已有 8 例正中神经滑膜肉瘤的报道。我们报告了 1 例 15 岁男性右手正中神经滑膜肉瘤。患者表现为右手大鱼际隆起处肿块进行性增大伴正中神经分布区麻木 2 个月。体格检查发现大鱼际处有一质软肿块,正中神经分布区感觉异常。他接受了肿瘤切除术,发现肿瘤包膜完整,累及正中神经,累及第一、第二和第三掌骨间的桡侧以及正中神经的复发性分支。肿瘤切除后,全面的转移灶检查未发现转移病灶。他被分期为 III、T2b、N0、M0-低度分化单相滑膜肉瘤,累及右侧正中神经。术后患者开始化疗和放疗。神经内滑膜肉瘤极为罕见。我们的病例是目前报道的此类病例中年龄最小、随访时间最长的病例。目前,他在治疗后 3 年,没有复发迹象,右手功能恢复良好。由于该疾病罕见,而且这是迄今为止文献中报道的最好结果,因此该病例特别有趣。

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