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胃肠道间质瘤的皮肤转移:病例系列及文献综述

Skin Metastasis of Gastrointestinal Stromal Tumors: A Case Series and Literature Review.

作者信息

Liu Peng, Tan Fengbo, Liu Heli, Ge Jie, Liu Sheng, Lei Tianxiang, Zhao Xianhui

机构信息

Department of Gastrointestinal Surgery, Xiangya Hospital, Central South University, Changsha 410008, Hunan, People's Republic of China.

出版信息

Cancer Manag Res. 2020 Aug 24;12:7681-7690. doi: 10.2147/CMAR.S261823. eCollection 2020.

Abstract

BACKGROUND

Gastrointestinal stromal tumors (GISTs) extremely and rarely metastasize to the skin, and such metastases have not been well characterized.

METHODS

Retrospective analysis of clinicopathological data of patients with skin metastasis of a GIST (SM-GIST) admitted to Xiangya Hospital (Changsha, Hunan, China) and literature review were conducted.

RESULTS

Including our 4 cases, a total of 17 cases have been reported to date. The mean age of the patients was 55.4 years (29~70 years) and there was not sex predominance (male 10 and female 7). Primary tumors were often located in the stomach (n=9), duodenum (n=2) and small bowel (n=2). Meanwhile, SM-GIST mainly occurred in head and face (n=6), extremities (n=6), followed by abdomen wall (n=5), back (n=3) and chest (n=2). Mutation analysis revealed that the frequency of wild-type GIST (WT-GIST), exon 9, 11 and 13 mutations was 6, 1, 4 and 1, respectively. The average time to SM-GIST was 4.22 years, specifically 4.59 years in gastric and 3.8 years in non-gastric. Moreover, for the resection only group (including chemotherapy), such average time was 3.63 years, while for the combined group (resection and tyrosine kinase inhibitors (TKIs)), it was about 4.74 years. The mean survival was approximately 6.2 years. However, after the diagnosis of SM-GIST, survival was only about 1.69 years.

CONCLUSION

SM-GIST is a rare malignant condition. Non-gastric GIST, surgery without TKIs, high invasiveness and tumor burden, and molecular subtype (mutation in exon 9, 11 and wild-type) may be conducive to the development of SM-GIST. Additionally, it is also a sign of poor prognosis.

摘要

背景

胃肠道间质瘤(GISTs)极少发生皮肤转移,且此类转移尚未得到充分的特征描述。

方法

对湘雅医院(中国湖南长沙)收治的胃肠道间质瘤皮肤转移(SM - GIST)患者的临床病理资料进行回顾性分析,并进行文献复习。

结果

包括我们的4例患者,迄今为止共报道了17例。患者的平均年龄为55.4岁(29~70岁),无性别优势(男性10例,女性7例)。原发肿瘤常位于胃(n = 9)、十二指肠(n = 2)和小肠(n = 2)。同时,SM - GIST主要发生在头面部(n = 6)、四肢(n = 6),其次为腹壁(n = 5)、背部(n = 3)和胸部(n = 2)。突变分析显示,野生型GIST(WT - GIST)、外显子9、11和13突变的频率分别为6、1、4和1。发生SM - GIST的平均时间为4.22年,具体而言,胃GIST为4.59年,非胃GIST为3.8年。此外,仅行手术组(包括化疗)的平均时间为3.63年,而联合组(手术加酪氨酸激酶抑制剂(TKIs))约为4.74年。平均生存期约为6.2年。然而,在诊断为SM - GIST后,生存期仅约1.69年。

结论

SM - GIST是一种罕见的恶性疾病。非胃GIST、未使用TKIs的手术、高侵袭性和肿瘤负荷以及分子亚型(外显子9、11突变和野生型)可能有利于SM - GIST的发生发展。此外,它也是预后不良的标志。

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