13 岁女孩肝脏巨大未分化肉瘤行解剖性肝切除治疗：病例报告及文献复习
A large undifferentiated sarcoma of the liver in a 13-year-old girl treated with anatomical resection: a case report and review of the literature.
机构信息
Department of Pediatric Surgery, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran.
出版信息
BMC Gastroenterol. 2022 Jan 3;22(1):2. doi: 10.1186/s12876-021-02076-x.
BACKGROUND
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumor accounting for 6-13% of primary liver tumors. Accurate preoperative diagnosis is difficult, with a rather high misdiagnosis rate. Herein, we reported a very large UESL treated with anatomical resection. Our case is amongst the largest pediatric UESLs in the literature.
CASE PRESENTATION
Herein, we report a 13-year-old girl presenting with right upper quadrant abdominal pain, postprandial vomiting, and abdominal distention, in which radiographic imaging demonstrated a huge UESL (28 × 20 × 12 cm). The patient was treated with partial hepatectomy and the 5 kg tumor was removed. The patient was discharged in good condition, with no significant complaints in her follow-up.
CONCLUSIONS
Although different treatment strategies have been reported for UESL cases, anatomical resection is still the main treatment approach, especially for large tumors.
背景
肝未分化胚胎肉瘤（UESL）是一种罕见的肝脏肿瘤，占原发性肝脏肿瘤的 6-13%。准确的术前诊断较为困难，误诊率较高。在此，我们报告了一例经解剖性切除术治疗的非常大的 UESL。我们的病例是文献中最大的儿科 UESL 之一。
病例介绍
本病例报告了一名 13 岁女孩，因右上腹疼痛、餐后呕吐和腹胀就诊，影像学检查显示巨大的 UESL（28×20×12cm）。患者接受了部分肝切除术，切除了 5kg 的肿瘤。患者恢复良好出院，随访时无明显不适。
结论
尽管已有报道提出了不同的 UESL 治疗策略，但解剖性切除术仍是主要的治疗方法，尤其是对于大肿瘤。
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