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一名青年男性的巨大腹腔内硬纤维瘤:病例报告及文献综述

Giant Intra-Abdominal Desmoid Tumor in a Young Man: A Case Report and Literature Review.

作者信息

Tanaka Yusuke, Toyokawa Takahiro, Yoshii Mami, Miki Yuichiro, Tamura Tatsuro, Lee Shigeru, Maeda Kiyoshi

机构信息

Department of Gastroenterological Surgery, Tsukazaki Hospital, Himeji, Hyogo, Japan.

Department of Gastroenterological Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Osaka, Japan.

出版信息

Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.24-0019. Epub 2025 Mar 15.

Abstract

INTRODUCTION

Desmoid tumors are rare soft-tissue tumors with a high recurrence rate; however, histologically, these tumors are benign. We describe a case in which a giant desmoid tumor was resected in a young man without any apparent causative factors.

CASE PRESENTATION

A 21-year-old man was referred to our hospital for treatment after presenting to a nearby hospital with right inguinal pain. Abdominal magnetic resonance imaging showed an intra-abdominal mass measuring 34 × 15 × 8 cm with partial signal hyperintensity on T2-weighted imaging and hypointensity on T1-weighted imaging, extending from the left abdominal cavity to the pelvic region. Although no definitive diagnosis was obtained preoperatively, surgery was performed under suspicion of gastrointestinal stromal tumor or other significant disease. A mass was identified firmly adherent to the transverse colon, gastric wall, and diaphragm, and these organs were partially resected. The excised specimen measured 38 × 21 × 8 cm and weighed 6400 g. Macroscopically, the tumor showed a smooth surface and homogeneous interior. Pathological examination revealed atypical cells with spindle-shaped nuclei and collagen fiber hyperplasia in the stroma, and immunostaining was negative for c-kit, CD34, desmin, S-100, and positive for β-catenin, leading to a confirmed diagnosis of desmoid tumor. Fifteen months after surgery, a local recurrence with a diameter of 3.0 cm was identified, and the patient remains under careful follow-up.

CONCLUSIONS

Intra-abdominal desmoid tumors larger than 30 cm are extremely rare. When encountering a young patient with a large intra-abdominal tumor, the possibility of desmoid tumor should be considered.

摘要

引言

硬纤维瘤是一种罕见的软组织肿瘤,复发率高;然而,从组织学角度来看,这些肿瘤是良性的。我们描述了一例在一名年轻男性中切除巨大硬纤维瘤的病例,该病例无任何明显致病因素。

病例介绍

一名21岁男性因右侧腹股沟疼痛就诊于附近医院后被转诊至我院接受治疗。腹部磁共振成像显示腹腔内有一大小为34×15×8 cm的肿块,在T2加权成像上呈部分信号高增强,在T1加权成像上呈低增强,从左腹腔延伸至盆腔区域。尽管术前未获得明确诊断,但在怀疑为胃肠道间质瘤或其他重大疾病的情况下进行了手术。发现一个肿块与横结肠、胃壁和膈肌紧密粘连,对这些器官进行了部分切除。切除标本大小为38×21×8 cm,重6400 g。大体上,肿瘤表面光滑,内部均匀。病理检查显示间质中有核呈梭形的非典型细胞和胶原纤维增生,免疫组化c-kit、CD34、结蛋白、S-100均为阴性,β-连环蛋白为阳性,确诊为硬纤维瘤。手术后15个月,发现局部复发,直径为3.0 cm,该患者仍在密切随访中。

结论

腹腔内直径大于30 cm的硬纤维瘤极为罕见。当遇到一名患有巨大腹腔肿瘤的年轻患者时,应考虑硬纤维瘤的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b97a/11926331/713234ba28c7/scr-11-01-24-0019-g001.jpg

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