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胆囊切除术后侵袭性腹腔内硬纤维瘤病复杂病例

Complex Case of Aggressive Intra-abdominal Desmoid-type Fibromatosis Status Post Cholecystectomy.

作者信息

Rangunwala Jigarkumar, Sitta Juliana, Prakash Varsha, Vyas Kshama, Roda Manohar

机构信息

Radiology, University of Mississippi Medical Center, Jackson, USA.

Pathology, University of Mississippi Medical Center, Jackson, USA.

出版信息

Cureus. 2020 Mar 6;12(3):e7193. doi: 10.7759/cureus.7193.

Abstract

Desmoid-type fibromatosis (DF), also known as desmoid tumor, is an extremely rare, benign, mesenchymal fibrous tumor with no potential for metastasis. It can arise from any part of the body, most commonly extra-abdominally. Intra-abdominal DF can present sporadically, in sites of previous trauma, surgical scars and irradiation, or in association with familial adenomatous polyposis and Gardner syndrome. Intra-abdominal DF is uncommon and especially rare after a common surgery like cholecystectomy. We report a rare case of a 67-year-old male who presented with a locally aggressive intra-abdominal DF in the gallbladder fossa, status post cholecystectomy. This progressively enlarging infiltrative enhancing solid mass in the gallbladder fossa on serial computed tomography and magnetic resonance imaging demonstrated gastric outlet obstruction, biliary obstruction, portal vein narrowing and encasement of hepatic artery. Diagnosis of DF in this postoperative setting was delayed and challenging due to uncharacteristic clinical presentation. Radiologists should be aware of this unusual diagnosis and spectrum of imaging findings to help in timely surgical management and planning.

摘要

韧带样型纤维瘤病(DF),也称为韧带样瘤,是一种极其罕见的良性间充质纤维瘤,无转移潜能。它可发生于身体的任何部位,最常见于腹部外。腹腔内DF可散发出现,见于既往创伤、手术瘢痕和放疗部位,或与家族性腺瘤性息肉病和加德纳综合征相关。腹腔内DF并不常见,在胆囊切除术等普通手术后尤其罕见。我们报告一例罕见病例,一名67岁男性,在胆囊切除术后,胆囊窝出现局部侵袭性腹腔内DF。在连续计算机断层扫描和磁共振成像上,胆囊窝内这个逐渐增大的浸润性强化实性肿块显示出胃出口梗阻、胆道梗阻、门静脉狭窄和肝动脉包绕。由于临床表现不典型,在这种术后情况下DF的诊断延迟且具有挑战性。放射科医生应意识到这种不寻常的诊断及影像学表现谱,以帮助及时进行手术管理和规划。

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