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直肠原发性未分化多形性肉瘤的外科治疗:病例报告及文献复习。

Surgical management of primary undifferentiated pleomorphic sarcoma of the rectum: a case report and review of the literature.

机构信息

Department of Surgery, Katsushika Medical Center, The Jikei University School of Medicine, 6-41-2 Aoto, Katsushika-Ku, Tokyo, 125-8506, Japan.

Department of Gastrointestinal Surgery, The Jikei University School of Medicine, 3-25-8 Nishishimbashi, Minato-ku, Tokyo, 105-8461, Japan.

出版信息

World J Surg Oncol. 2022 Jun 13;20(1):199. doi: 10.1186/s12957-022-02671-6.

Abstract

BACKGROUND

Undifferentiated pleomorphic sarcoma (UPS) is a malignant soft tissue tumor that has been reclassified from malignant fibrous histiocytoma with the development of the pathological diagnosis. It principally occurs in the extremities but rarely occurs in the rectum. We herein report a rare case of UPS arising in the rectum.

CASE PRESENTATION

A 85-year-old woman was referred to our hospital with a complaint of anal pain, which had persisted for several months. Computed tomography (CT) showed a 53 × 58 × 75 mm mass on the left side of the rectum. Colonoscopy revealed a submucosal elevation in the rectum without any exposure of the tumor to the surface. Contrast-enhanced CT and magnetic resonance imaging revealed an 80-mm mass that originated in the rectal muscular propria, and we suspected a gastrointestinal stromal tumor. No lymph node metastasis or distant metastasis was observed. We performed a laparoscopic Hartmann's operation. Intraoperatively, severe adhesion around the tumor caused tumor injury and right ureteral dissection. Thus, laparoscopic right ureteral anastomosis and ureteral stenting were additionally performed. The operation time was 6 h and 3 min, and the estimated blood loss was small. The patient was discharged without complications 25 days after surgery. A pathological examination showed that the tumor was composed of highly heterogeneous cells with no specific differentiation traits, leading to a diagnosis of UPS. Contrast-enhanced CT performed 2 months after surgery showed bilateral pelvic lymph node enlargement, which indicated recurrence. Considering the patient's age, we performed radiotherapy (50 Gy/25 Fr targeting the pelvic region). At present, 16 months have passed since the completion of radiotherapy. Contrast-enhanced CT shows that the recurrent lymph nodes have disappeared, and no new distant metastasis has been observed.

CONCLUSIONS

We reported a case of UPS arising in the rectum. The surgical procedure and indication of preoperative therapy should be carefully selected because complete removal of the tumor is desirable in UPS.

摘要

背景

未分化多形性肉瘤(UPS)是一种恶性软组织肿瘤,随着病理诊断的发展,已从恶性纤维组织细胞瘤中重新分类。它主要发生在四肢,但很少发生在直肠。本文报告一例罕见的直肠 UPS 病例。

病例介绍

一名 85 岁女性因肛门疼痛持续数月就诊于我院。计算机断层扫描(CT)显示直肠左侧有一个 53×58×75mm 的肿块。结肠镜检查显示直肠黏膜下隆起,无肿瘤暴露于表面。增强 CT 和磁共振成像显示起源于直肠固有肌层的 80mm 肿块,我们怀疑是胃肠道间质瘤。未观察到淋巴结转移或远处转移。我们进行了腹腔镜 Hartmann 手术。术中,肿瘤周围严重粘连导致肿瘤损伤和右输尿管分离。因此,还进行了腹腔镜右输尿管吻合术和输尿管支架置入术。手术时间为 6 小时 3 分钟,估计出血量少。术后 25 天患者无并发症出院。病理检查显示肿瘤由高度异质性细胞组成,无特定分化特征,诊断为 UPS。术后 2 个月行增强 CT 检查显示双侧骨盆淋巴结肿大,提示复发。考虑到患者年龄,我们对骨盆区域进行了 50Gy/25Fr 的放疗。目前,放疗完成后已过去 16 个月。增强 CT 显示复发性淋巴结已消失,未发现新的远处转移。

结论

我们报告了一例直肠 UPS 病例。由于 UPS 理想的治疗方案是完全切除肿瘤,因此应仔细选择手术程序和术前治疗的适应证。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9939/9195365/b53edbfcb069/12957_2022_2671_Fig1_HTML.jpg

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