巨大横结肠肠系膜黏液性脂肪肉瘤合并直肠癌及主动脉缩窄1例报告及文献复习。

IF 1.7 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY
Meng Wang, Jian Sun, Zhi-Qiang Song, Xi-Qi Chen, Guang-Dong Xie, Yong Zhu, Yong-Kun Zhou
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引用次数: 0

摘要

背景:脂肪肉瘤是起源于脂肪细胞的恶性间充质肿瘤。黏液样脂多糖(MLPS)是一种常见的亚型,主要发生在四肢、腹膜后和深部软组织,很少发生在胃肠道。原发性肠系膜内窥镜特别罕见,特别是在横结肠肠系膜。病例总结:本报告描述了一例65岁女性患者,她表现为腹胀,被诊断为横结肠肠系膜的巨大粘液性LPS。入院后,患者接受了全面的评估。胸部和腹部增强CT显示一个大的恶性肿瘤并主动脉夹层,结肠镜检查发现直肠癌。考虑到患者的病情和手术风险,首先进行介入手术来控制主动脉缩窄,然后通过剖腹手术切除肿瘤。术中及组织病理学检查证实一巨大粘液性脂多糖起源于横结肠肠系膜。术后患者接受化疗,定期随访CT。直肠肿瘤虽未复发,但肠系膜肿瘤广泛复发,压迫肠腔,造成机械性梗阻,严重威胁患者生命。第二次手术暂时缓解了梗阻症状;然而,它对肿瘤进展的影响有限。4个月后,患者因疾病进展死亡。结论:对于合并多种疾病的MLPS,目前尚无规范的治疗方法,手术仍是主要治疗方法。然而,复发、转移和术后预后不良仍然严重威胁着患者的生存。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Giant transverse colonic mesenteric mucinous liposarcoma combined with rectal cancer and aortic coarctation: A case report and review of literature.

Background: Liposarcomas (LPSs) are malignant mesenchymal tumors originating from adipocytes. Myxoid LPS (MLPS), a common subtype, predominantly arises in the extremities, retroperitoneum, and deep soft tissues, with a rare occurrence in the gastrointestinal tract. Primary mesenteric LPS is particularly uncommon, especially in the transverse colonic mesentery.

Case summary: This report describes the case of a 65-year-old female patient who presented with abdominal distension and was diagnosed with a giant mucinous LPS of the transverse colonic mesentery. Upon admission, the patient underwent a comprehensive evaluation. Contrast-enhanced computed tomography (CT) of the chest and abdomen revealed a large malignant tumor with aortic dissection, while colonoscopy identified rectal cancer. Given the patient's condition and surgical risk, an interventional procedure was first performed to manage the aortic coarctation, followed by tumor resection via laparotomy. Intraoperative and histopathological findings confirmed a giant mucinous LPS originating from the transverse colon mesentery. Postoperatively, the patient underwent chemotherapy and regular follow-up CT. Although the rectal tumor did not recur, the mesenteric tumor showed extensive recurrence, compressing the intestinal lumen and causing mechanical obstruction, which severely threatened the patient's life. A second operation temporarily relieved the obstructive symptoms; however, it had a limited effect on tumor progression. The patient died four months later due to disease progression.

Conclusion: While no standardized treatment exists for MLPS co-occurring with multiple diseases, operation remains the mainstay. However, recurrence, metastasis, and poor postoperative prognosis continue to pose serious threats to patient survival.

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