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肺横纹肌肉瘤小肠转移致肠套叠 1 例报告。

Small bowel metastasis from pulmonary rhabdomyosarcoma causing intussusception: a case report.

机构信息

Clinical Medical College of Jiangsu University, Department of Gastrointestinal Surgery, Kunshan First People's Hospital affiliated to Jiangsu University, Jiangsu, Kunshan, 215300, China.

出版信息

BMC Gastroenterol. 2019 May 10;19(1):71. doi: 10.1186/s12876-019-0990-4.

Abstract

BACKGROUND

Rhabdomyosarcoma (RMS), especially primary pulmonary RMS, is an extremely rare type of soft tissue sarcoma in adults. Small bowel is an uncommon site for metastases.

CASE PRESENTATION

This report described an unusual case of jejunum metastasis from primary pulmonary RMS causing intussusception in a 75-year-old man. The patient consulted for 2 weeks of continuous dyspnea. Chest computed tomography (CT) demonstrated a large mass involving the left lower lobe. Transthoracic biopsy confirmed the existence of pleomorphic RMS. Immunohistochemical studies showed positive findings about desmin and MyoD1. The results of gastroscopy, colonoscopy and abdominal CT were all negative. Positron emission tomography/CT demonstrated a fluorodeoxyglucose-reactive large lesion in the left lower lobe without metastatic lesions. The patient received synchronous chemoradiotherapy. After 9 months, the patient presented with intermittent upper abdominal pain with nausea and vomiting. CT showed small bowel dilatation secondary to intussusception. The patient subsequently received laparotomy, and the intussuscepted small bowel segment was resected. Histological examination revealed pleomorphic RMS involving the mucosa, submucosa, and muscular tissues.

CONCLUSIONS

RMS is highly aggressive and metastatic. The metastatic disease can rapidly progress to cause subsequent complications. The possibility of small bowel metastasis should be considered, although it is extremely rare.

摘要

背景

横纹肌肉瘤(RMS),特别是原发性肺 RMS,是成人中一种极其罕见的软组织肉瘤。小肠是转移的罕见部位。

病例介绍

本报告描述了一例罕见的原发性肺 RMS 引起回肠转移并导致 75 岁男性肠套叠的病例。该患者因持续呼吸困难就诊 2 周。胸部计算机断层扫描(CT)显示左肺下叶有一个大肿块。经胸活检证实存在多形性 RMS。免疫组织化学研究显示结蛋白和 MyoD1 阳性。胃镜、结肠镜和腹部 CT 结果均为阴性。正电子发射断层扫描/CT 显示左肺下叶有一个氟脱氧葡萄糖反应性大病变,无转移病灶。患者接受了同步放化疗。9 个月后,患者出现间歇性上腹痛、恶心和呕吐。CT 显示小肠扩张继发肠套叠。随后患者接受了剖腹手术,切除了套叠的小肠段。组织学检查显示多形性 RMS 累及黏膜、黏膜下层和肌肉组织。

结论

RMS 具有高度侵袭性和转移性。转移疾病可迅速进展并导致后续并发症。尽管非常罕见,但应考虑小肠转移的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc1f/6509787/568d9a5252b6/12876_2019_990_Fig1_HTML.jpg

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