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局限性结直肠淀粉样变性的手术策略

Surgical strategies for localized colorectal amyloidosis.

作者信息

Tahara Shunya, Kohyama Mohei, Nakamitsu Atsushi, Sugiyama Yoichi, Tazaki Tatsuya, Taogoshi Hiroyuki, Komo Toshiaki, Yamaguchi Takuro, Ueda Mitsuharu, Ishikawa Akira, Takahashi Shinya, Sasaki Masaru

机构信息

Department of Surgery, JA Hiroshima General Hospital, 1-3-3 Jigozen, Hatsukaichi, Hiroshima, 738-8503, Japan.

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjou, Chuo Ward, Kumamoto, Kumamoto, 860-8556, Japan.

出版信息

Surg Case Rep. 2023 Apr 27;9(1):66. doi: 10.1186/s40792-023-01649-0.

Abstract

BACKGROUND

Localized colorectal amyloidosis has a good prognosis, but cases involving bleeding or perforation may require surgery. However, there are few case reports discussing the differences in the surgical strategy between the segmental and pan-colon types.

CASE PRESENTATION

A 69-year-old woman with a history of abdominal pain and melena was diagnosed with amyloidosis localized in the sigmoid colon by colonoscopy. Since preoperative imaging and intraoperative findings could not rule out malignancy, we performed laparoscopic sigmoid colectomy with lymph-node dissection. Histopathological examination and immunohistochemical staining revealed a diagnosis of AL amyloidosis (λ type). We diagnosed localized segmental gastrointestinal amyloidosis, because there was no amyloid protein in the margins, and the tumor was localized. There were no malignant findings.

CONCLUSIONS

Unlike systemic amyloidosis, localized amyloidosis has a favorable prognosis. Localized colorectal amyloidosis can be classified into the segmental type, in which amyloid protein is deposited locally, and the pan-colon type, in which amyloid protein is deposited extensively in the colon. Amyloid protein causes ischemia due to vascular deposition, weakening of the intestinal wall due to muscle layer deposition, and decreased peristalsis due to nerve plexus deposition. No amyloid protein should remain outside the resection area. The pan-colon type is often reported to cause complications such as anastomotic leakage, and primary anastomosis should be avoided. On the other hand, if there is no contamination or tumor remnants in the margin, the segmental type may be considered for primary anastomosis.

摘要

背景

局限性结直肠淀粉样变性预后良好,但涉及出血或穿孔的病例可能需要手术治疗。然而,很少有病例报告讨论节段型和全结肠型在手术策略上的差异。

病例介绍

一名69岁有腹痛和黑便病史的女性通过结肠镜检查被诊断为乙状结肠局限性淀粉样变性。由于术前影像学检查和术中发现无法排除恶性肿瘤,我们进行了腹腔镜乙状结肠切除术并清扫淋巴结。组织病理学检查和免疫组化染色显示诊断为AL型淀粉样变性(λ型)。我们诊断为局限性节段性胃肠道淀粉样变性,因为切缘无淀粉样蛋白,且肿瘤局限。无恶性表现。

结论

与系统性淀粉样变性不同,局限性淀粉样变性预后良好。局限性结直肠淀粉样变性可分为节段型,即淀粉样蛋白局部沉积,和全结肠型,即淀粉样蛋白在结肠广泛沉积。淀粉样蛋白因血管沉积导致缺血,因肌层沉积导致肠壁变薄,因神经丛沉积导致蠕动减弱。切除区域外不应残留淀粉样蛋白。全结肠型常报道会引起诸如吻合口漏等并发症,应避免一期吻合。另一方面,如果切缘无污染或肿瘤残留,节段型可考虑一期吻合。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b003/10140200/18c98d19498d/40792_2023_1649_Fig1_HTML.jpg

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