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肝脏未分化胚胎性肉瘤酷似急性阑尾炎。病例报告及文献复习

Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis. Case report and review of the literature.

作者信息

Sakellaridis Timothy, Panagiotou Ioannis, Georgantas Themistoclis, Micros George, Rontogianni Dimitra, Antiochos Christos

机构信息

1st Surgical Department, 401 Army Force General Hospital of Athens, Greece.

出版信息

World J Surg Oncol. 2006 Feb 20;4:9. doi: 10.1186/1477-7819-4-9.

Abstract

BACKGROUND

Undifferentiated embryonal sarcoma (UES) of liver is a rare malignant neoplasm, which affects mostly the pediatric population accounting for 13% of pediatric hepatic malignancies, a few cases has been reported in adults.

CASE PRESENTATION

We report a case of undifferentiated embryonal sarcoma of the liver in a 20-year-old Caucasian male. The patient was referred to us for further investigation after a laparotomy in a district hospital for spontaneous abdominal hemorrhage, which was due to a liver mass. After a through evaluation with computed tomography scan and magnetic resonance imaging of the liver and taking into consideration the previous history of the patient, it was decided to surgically explore the patient. Resection of I-IV and VIII hepatic lobe. Patient developed disseminated intravascular coagulation one day after the surgery and died the next day.

CONCLUSION

It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy.

摘要

背景

肝未分化胚胎性肉瘤(UES)是一种罕见的恶性肿瘤,主要影响儿童群体,占儿童肝脏恶性肿瘤的13%,成人中也有少数病例报道。

病例报告

我们报告一例20岁白种男性的肝未分化胚胎性肉瘤病例。该患者因区医院剖腹探查发现肝脏肿块导致自发性腹腔出血而转诊至我院作进一步检查。在对肝脏进行计算机断层扫描和磁共振成像全面评估并考虑患者既往病史后,决定对患者进行手术探查。切除肝Ⅰ-Ⅳ和Ⅷ段。患者术后一天发生弥散性血管内凝血,次日死亡。

结论

这是一种罕见的、高度恶性的肝脏肿瘤,几乎仅影响儿童群体。预后较差,但最近的证据表明,无论是否进行术后化疗,完整手术切除后有可能实现长期生存。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d34f/1403776/2b39011bf1a4/1477-7819-4-9-1.jpg

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