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累及胰腺的硬纤维瘤病:临床、细胞病理学和放射学相关性的回顾性病例系列研究。

Desmoid fibromatosis involving the pancreas: A retrospective case series with clinical, cytopathologic and radiologic correlation.

机构信息

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA.

出版信息

Ann Diagn Pathol. 2022 Oct;60:152015. doi: 10.1016/j.anndiagpath.2022.152015. Epub 2022 Jul 22.

Abstract

Desmoid-type fibromatosis (desmoid tumors) which involve the pancreas is an infrequent diagnosis which clinically can mimic both neoplastic and non-neoplastic lesions of the pancreas. The cytologic features of loosely cohesive cytologically bland (myo)fibroblastic cells are non-specific, however the long fascicular growth pattern and the presence of β-catenin mutation with positive nuclear immunohistochemical staining or molecular testing allows for definitive diagnosis. While many previously reported desmoid tumors of the pancreas have been surgically resected, conservative management with a "watch and wait" approach is also an effective mode of management for these tumors. Herein, we report the largest case series of pancreatic desmoid tumors with clinical, cytopathologic, and radiologic correlation.

摘要

累及胰腺的韧带样纤维瘤病(韧带样纤维瘤)是一种罕见的诊断,临床上可模拟胰腺的肿瘤性和非肿瘤性病变。松散聚集的细胞学上温和(肌)纤维母细胞的细胞学特征是非特异性的,然而,长束状生长模式和β-连环蛋白突变的存在,以及核免疫组织化学染色或分子检测阳性,可明确诊断。虽然以前报道的许多胰腺韧带样纤维瘤已行手术切除,但“观察和等待”的保守治疗方法也是这些肿瘤的有效治疗模式。在此,我们报告了最大的一组具有临床、细胞病理学和放射学相关性的胰腺韧带样纤维瘤病例。

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