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一例罕见的胃肠道间质瘤伴肝转移,肿瘤浸润下腔静脉并延伸至右心房,手术切除后早期复发

A Rare Case of Gastrointestinal Stromal Tumor with a Liver Metastasis Infiltrating the Inferior Vena Cava and Extending to the Right Atrium with an Early Recurrence after Surgical Extraction.

作者信息

Siamkouris Dimitrios, Schloesser Marc, Yousef Amr, Offers Elmar

机构信息

Department of Cardiology, Dreifaltigkeits Hospital Lippstadt, Germany, Academic Teaching Hospital of Westfaelische Wilhelms University Muenster, Germany.

出版信息

Case Rep Cardiol. 2019 Feb 5;2019:2623403. doi: 10.1155/2019/2623403. eCollection 2019.

Abstract

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract. The major cause of GIST is the presence of an abnormal form of tyrosine protein kinase (KIT) protein also known as CD117, which causes uncontrollable growth of the gastrointestinal cells. Most studies report incidences between 10 and 15 cases of GISTs per million. Metastases to the liver and peritoneum are the most frequent. We report a case of advanced GIST with a liver metastasis infiltrating the inferior vena cava (IVC) and extending to the right atrium in the form of a large, floating, isolated intracardiac liver metastasis with diastolic prolapsing through the tricuspid valve. This is a very rare manifestation. One week after heart surgery and removal of a 5 × 6 cm tumor mass from the right atrium and the IVC, echocardiography depicted an early recurrence.

摘要

胃肠道间质瘤(GISTs)是胃肠道的间叶组织肿瘤。GIST的主要病因是存在一种异常形式的酪氨酸蛋白激酶(KIT)蛋白,也称为CD117,它会导致胃肠道细胞不可控地生长。大多数研究报告称,每百万人口中GIST的发病率为10至15例。肝转移和腹膜转移最为常见。我们报告了一例晚期GIST,肝转移灶侵犯下腔静脉(IVC)并延伸至右心房,表现为一个巨大的、漂浮的、孤立的心内肝转移瘤,舒张期经三尖瓣脱垂。这是一种非常罕见的表现。心脏手术并从右心房和IVC切除一个5×6厘米的肿瘤块一周后,超声心动图显示早期复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a02/6379877/195a3281dbe9/CRIC2019-2623403.001.jpg

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