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1例罕见的脐尿管炎性肌纤维母细胞瘤。

A rare case of urachal inflammatory myofibroblastic tumor.

作者信息

George Rose, Swerdloff Daniel, Akgul Mahmut, Nazeer Tipu, Mian Badar M

机构信息

Department of Pathology, Albany Medical Center, Albany, NY, 12208, USA.

Department of Urology, Albany Medical Center, Albany, NY, 12208, USA.

出版信息

Urol Case Rep. 2021 Jan 20;36:101575. doi: 10.1016/j.eucr.2021.101575. eCollection 2021 May.

Abstract

Inflammatory myofibroblastic tumors (IMT) of the urachus is a rare neoplastic condition characterized by proliferation of spindle cell, likely derived from myofibroblasts or fibroblasts, with acute and chronic inflammatory infiltrate. Urachal IMT present with abdominal/pelvic pain and urinary symptoms. These often manifest as abdominal mass involving adjacent structures. We describe a case of young female with urachal IMT that was excised with a wide margin to ensure complete removal of all adjacent affected tissue using robotic-assisted laparoscopic approach. Immunohistochemical evidence of ALK and ALK gene rearrangement were confirmed in this tumor which are diagnostic of IMT.

摘要

脐尿管炎性肌纤维母细胞瘤(IMT)是一种罕见的肿瘤性疾病,其特征为梭形细胞增殖,可能起源于肌成纤维细胞或成纤维细胞,并伴有急慢性炎症浸润。脐尿管IMT表现为腹部/盆腔疼痛和泌尿系统症状。这些症状常表现为累及相邻结构的腹部肿块。我们描述了一例年轻女性脐尿管IMT病例,采用机器人辅助腹腔镜手术,广泛切除肿瘤边缘以确保完全切除所有相邻的受累组织。该肿瘤经免疫组织化学证实存在ALK及ALK基因重排,这是IMT的诊断依据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a2d/7840849/a281dad06af7/gr1.jpg

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