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肝脏孤立性纤维瘤:当前知识综述及1例新病例报告

Solitary Fibrous Tumor of the Liver: a Review of the Current Knowledge and Report of a New Case.

作者信息

Beltrán Marcelo A

出版信息

J Gastrointest Cancer. 2015 Dec;46(4):333-42. doi: 10.1007/s12029-015-9769-1.

Abstract

INTRODUCTION

Solitary fibrous tumors of the liver (SFTL) are uncommon tumors; to the present day, less than 50 cases has been reported in the English scientific literature, most of which behaved as benign tumors. The present article reports a new case of SFTL and has the main purpose of updating the current knowledge of SFTL because due to its rarity, its clinical presentation, study, treatment, and prognosis are not well known. The clinical presentation, radiologic study, surgical treatment, immunohistochemical study, and prognosis are updated and comprehensively discussed.

METHODS

Using the common search engines, a search of the English literature was conducted for "Solitary Fibrous Tumor of The Liver," and the relevant articles were retrieved, reviewed, and analyzed.

RESULTS

All published articles reported anecdotal SFTLs, or SFTLs were included in large series analyzing solitary fibrous tumors on different sites.

CONCLUSION

The SFTL is an uncommon neoplasm. The clinical presentation is habitually indolent and its behavior is uncertain. In some cases, the SFTL acts as an aggressive sarcoma with poor prognosis. Currently, only surgery offers a therapeutic opportunity for these patients. Due to the lack of current knowledge of long-term behavior of supposedly benign SFTLs and to the lack of specific therapies, methodical long-term follow-up is essential to ensure the survival of patients treated for SFTL.

摘要

引言

肝脏孤立性纤维瘤(SFTL)是罕见肿瘤;截至目前,英文科学文献中报道的病例不足50例,其中大多数表现为良性肿瘤。本文报告了1例SFTL新病例,主要目的是更新当前对SFTL的认识,因为由于其罕见性,其临床表现、研究、治疗及预后尚不为人熟知。本文对其临床表现、放射学检查、手术治疗、免疫组化研究及预后进行了更新并全面讨论。

方法

使用常用搜索引擎,以“肝脏孤立性纤维瘤”为关键词检索英文文献,检索、查阅并分析相关文章。

结果

所有已发表文章均报告了孤立的SFTL病例,或在分析不同部位孤立性纤维瘤的大型系列研究中纳入了SFTL病例。

结论

SFTL是一种罕见肿瘤。其临床表现通常不明显,行为难以确定。在某些情况下,SFTL表现为侵袭性肉瘤,预后不良。目前,只有手术为这些患者提供了治疗机会。由于对所谓良性SFTL的长期行为缺乏了解,且缺乏特异性治疗方法,系统的长期随访对于确保接受SFTL治疗患者的生存至关重要。

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