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肝脏孤立性纤维瘤:病例报告及文献复习

Solitary fibrous tumour of the liver: case report and review of the literature.

作者信息

Neeff Hannes, Obermaier R, Technau-Ihling K, Werner M, Kurtz C, Imdahl A, Hopt U T

机构信息

Department of General and Visceral Surgery, Albert-Ludwigs University of Freiburg, Hugstetterstrasse 55, 79106 Freiburg, Germany.

出版信息

Langenbecks Arch Surg. 2004 Aug;389(4):293-8. doi: 10.1007/s00423-004-0488-5. Epub 2004 Jun 19.

Abstract

BACKGROUND

Solitary fibrous tumours (SFTs) of the liver are very rare entities. Although firstly described to be tumours of pleural origin, SFTs have been reported in various organs such as the meninges, orbit, upper respiratory tract, thyroid, peritoneum, retroperitoneum and soft tissues. Histologically, this tumour often shows alternating cellular and relatively acellular areas. The cellular areas show a wide variety of patterns, making it difficult for it to be differentiated from other mesenchymal tumours. Its immunohistochemical positivity for CD34 and vimentin is believed to be unique. Histiogenesis of SFTs, however, is not yet fully understood. They are known to be usually benign, with only few reports indicating their ability to metastasize.

PATIENTS AND METHODS

We review the literature on SFTs of the liver and report on the case of a 63-year-old female patient with a large SFT of the right liver.

CONCLUSIONS

Surgical resection seems to be the treatment of choice. Local recurrence is scarce. Due to the very limited number of cases, data regarding the long-term survival of patients are lacking.

摘要

背景

肝脏孤立性纤维瘤(SFTs)是非常罕见的实体瘤。尽管最初被描述为起源于胸膜的肿瘤,但SFTs已在多种器官中被报道,如脑膜、眼眶、上呼吸道、甲状腺、腹膜、腹膜后和软组织。在组织学上,这种肿瘤常表现为细胞区和相对无细胞区交替出现。细胞区呈现多种模式,使其难以与其他间叶组织肿瘤相鉴别。其CD34和波形蛋白免疫组化阳性被认为具有独特性。然而,SFTs的组织发生尚未完全明确。已知它们通常为良性,仅有少数报道表明其有转移能力。

患者和方法

我们回顾了关于肝脏SFTs的文献,并报告了一例63岁患有右肝巨大SFT的女性患者的病例。

结论

手术切除似乎是首选治疗方法。局部复发少见。由于病例数量非常有限,缺乏关于患者长期生存的数据。

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