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[一例小肠系膜硬纤维瘤病]

[A Case of Desmoid-Type Fibromatosis of the Mesentery of Small Intestine].

作者信息

Ogura Takuya, Kunisaki Chikara, Sato Sho, Sato Tsutomu, Endo Itaru

机构信息

Dept. of Surgery, Gastroenterological Center Yokohama City University.

出版信息

Gan To Kagaku Ryoho. 2024 Mar;51(3):334-335.

Abstract

Desmoid-type fibromatosis is a relatively rare disease, often associated with familial adenomatous polyposis and a history of abdominal surgery. A 43-year-old male patient presented with abdominal pain and contrast-enhanced CT showed a mass in the lower abdomen. The mass was a 4×4×3 cm white, dense tumor with a wreath-like arrangement of eosinophilic spindle-shaped cells. Immunostaining showed KIT(-), CD34(-), desmin(-), β-catenin(+), SMA(few+), and the diagnosis was desmoid-type fibrosis. Six months after surgery, there was no apparent recurrence.

摘要

韧带样型纤维瘤病是一种相对罕见的疾病,常与家族性腺瘤性息肉病及腹部手术史相关。一名43岁男性患者因腹痛就诊,增强CT显示下腹部有一肿块。该肿块为一个4×4×3 cm的白色致密肿瘤,由嗜酸性梭形细胞呈花环样排列构成。免疫组化显示KIT(-)、CD34(-)、结蛋白(-)、β-连环蛋白(+)、平滑肌肌动蛋白(少量+),诊断为韧带样型纤维瘤病。术后6个月,未见明显复发。

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