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病例报告及文献复习:青年人大腹腔内尤文肉瘤。

Case report and literature review: A young man with giant intra-abdominal Ewing sarcoma.

机构信息

Gusu School, Nanjing Medical University, Nanjing, China.

Department of Hepatobiliary Surgery, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou, China.

出版信息

Medicine (Baltimore). 2024 Oct 4;103(40):e39983. doi: 10.1097/MD.0000000000039983.

Abstract

RATIONALE

Extraosseous Ewing sarcoma (EES) is a rare manifestation within the Ewing sarcoma tumor family (ESFT). Its clinical manifestations lack specificity, intestinal obstruction is the main symptom but can also present with abdominal pain, gastrointestinal bleeding, and other discomforts, making it prone to misdiagnosis as intestinal mesenchymal tumor.

PATIENT CONCERNS

A 29-year-old male was admitted to the hospital with intestinal obstruction symptoms and abdominal CT suggesting "left abdominal occupation."

DIAGNOSIS

The patient was initially misdiagnosed as intestinal mesenchymal tumor, and was later definitively diagnosed as abdominal Ewing sarcoma by postoperative pathology and genetic testing.

INTERVENTIONS

Due to the patient's surgical indication, surgical resection with exploratory laparotomy was performed and then the patient underwent systemic chemotherapy.

OUTCOMES

Intraoperatively, we found a 15-cm tumor originating from the proximal jejunum, with invasion into the peritoneum, duodenum, jejunum, and colon. Finally, the pathological report revealed Ewing sarcoma.

LESSONS

Giant abdominal Ewing sarcoma with a diameter of 15 cm is rare. Considering postoperative pathology and genetic testing, abdominal Ewing sarcoma was suspected. The patient was successfully treated using surgery.

摘要

背景

骨外尤文肉瘤(EES)是尤文肉瘤肿瘤家族(ESFT)中的一种罕见表现。其临床表现缺乏特异性,肠梗阻是主要症状,但也可表现为腹痛、胃肠道出血等不适,容易误诊为肠间质瘤。

病例介绍

一名 29 岁男性因肠梗阻症状入院,腹部 CT 提示“左腹部占位”。

诊断

患者最初被误诊为肠间质瘤,术后病理和基因检测最终明确诊断为腹部尤文肉瘤。

干预措施

由于患者有手术指征,进行了剖腹探查术切除,并随后进行了全身化疗。

结果

术中发现肿瘤起源于近端空肠,直径 15cm,侵犯腹膜、十二指肠、空肠和结肠。最终,病理报告显示为尤文肉瘤。

结论

直径达 15cm 的巨大腹部尤文肉瘤罕见。考虑到术后病理和基因检测,怀疑为腹部尤文肉瘤。患者通过手术成功治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e509/11460867/62c04b619525/medi-103-e39983-g001.jpg

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