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双侧肾周组织多灶性硬纤维瘤伴胆管周围定位。

Multifocal bilateral desmoid tumour of perirenal tissues with peribiliary localization.

作者信息

Sacconi Beatrice, Argirò Renato, Iannarelli Angelo, Di Gaeta Alessandro, Bezzi Mario

机构信息

Department of Radiological, Oncological and Anatomopathological Sciences, Sapienza University of Rome, Rome, Italy.

出版信息

BJR Case Rep. 2015 Dec 16;2(2):20150099. doi: 10.1259/bjrcr.20150099. eCollection 2016.

Abstract

Desmoid tumour (DT) is an unusual, benign tumour, more frequently observed in patients with familial polyposis and pregnant females. It usually presents as a single mass lesion, more frequently showing a compressive rather than an infiltrative growth pattern. We report a case of a 70-year-old male presenting with a multifocal, bilateral infiltrative DT of the perirenal tissue, with involvement of the choledochus wall. The patient was partly treated with tamoxifen and docetaxel, but both therapies were discontinued in accordance with the patient's decision owing to mild toxicity; however, a CT examination performed 3 months later showed an unexpected remarkable reduction of the tumour at all sites. At 1 year follow-up, new pathologic tissue was visible surrounding the right renal pelvis and the calices.

摘要

硬纤维瘤(DT)是一种罕见的良性肿瘤,在家族性息肉病患者和怀孕女性中更常见。它通常表现为单个肿块病变,更常呈现压迫性而非浸润性生长模式。我们报告一例70岁男性,其表现为双侧肾周组织多灶性浸润性硬纤维瘤,并累及胆总管壁。该患者部分接受了他莫昔芬和多西他赛治疗,但由于轻度毒性,根据患者的决定两种治疗均停止;然而,3个月后进行的CT检查显示所有部位的肿瘤意外显著缩小。在1年随访时,可见右肾盂和肾盏周围有新的病理组织。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7f42/6180855/eff0de7f3351/bjrcr.20150099.g001.jpg

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