Lu Aimei, Xie Xiaohong, Han Jinyan, Yu Jingjing, Qin Xiangcheng, Xu Ping
Department of Ultrasound, Ningbo Yinzhou Second Hospital, Ningbo, China.
Department of Pathology, Ningbo Yinzhou Second Hospital, Ningbo, China.
Transl Androl Urol. 2023 Feb 28;12(2):347-352. doi: 10.21037/tau-22-742. Epub 2023 Feb 9.
Rosai-Dorfman-Destombes disease (RDD) is a rare histioproliferative disease with unknown etiology. It commonly occurs in lymph nodes and can affect extra-nodal tissues and organs. Renal RDD is extremely rare, only a few cases have been reported, and its clinical symptoms and imaging findings are non-specific. To date, no literature has summarized its imaging manifestations in a large number of cases. Due to the involvement of different tissues and organs, there is no standard treatment for RDD. It has been reported that RDD patients with kidney involvement have a poor prognosis. Thus, understanding of renal RDD need to be extended.
We present a rare case of renal RDD in an asymptomatic 67-year-old male. The results of an ultrasound examination indicated that both kidneys were surrounded by hypoechoic soft tissue lesions, and there was a huge mass in the left kidney, which had a clear boundary with the renal capsule. The results of contrast-enhanced ultrasound (CEUS) showed hypo-enhancement in the bilateral perinephric lesions and mass. However, the computed tomography urography (CTU) findings revealed no obvious enhancement. The patient then underwent a series of laboratory tests, but no relevant information was found. To make a clear diagnosis, the urologist then removed the left perirenal mass and some perirenal tissues, and the patient was finally pathologically diagnosed with extra-nodal RDD. The patient remains asymptomatic, and no treatment has been administered to date.
This case may be the first reported case in which CEUS was performed and the second reported case of asymptomatic renal RDD. Based on the previous literature reports, we found that some specific characteristics of renal RDD include bilateral perirenal lesions with a "hairy kidney" appearance. CEUS and/or CTU can be used to help differentiate a solitary mass of RDD from common tumors, to avoid misdiagnosis leading to unnecessary nephrectomy.
罗萨伊-多夫曼-德斯顿贝斯病（RDD）是一种病因不明的罕见组织细胞增生性疾病。它通常发生于淋巴结，也可累及结外组织和器官。肾RDD极为罕见，仅有少数病例报道，其临床症状和影像学表现无特异性。迄今为止，尚无文献对大量病例的影像学表现进行总结。由于RDD可累及不同组织和器官，因此尚无标准治疗方案。据报道，累及肾脏的RDD患者预后较差。因此，需要进一步了解肾RDD。
我们报告一例罕见的无症状67岁男性肾RDD病例。超声检查结果显示双肾均被低回声软组织病变包绕，左肾有一巨大肿块，与肾包膜边界清晰。超声造影（CEUS）结果显示双侧肾周病变及肿块呈低增强。然而，计算机断层扫描尿路造影（CTU）结果显示无明显强化。患者随后接受了一系列实验室检查，但未发现相关信息。为明确诊断，泌尿外科医生切除了左肾周肿块及部分肾周组织，患者最终经病理诊断为结外RDD。患者仍无症状，迄今为止未接受任何治疗。
该病例可能是首例进行CEUS检查的病例，也是第二例无症状肾RDD病例。基于既往文献报道，我们发现肾RDD的一些特征包括双侧肾周病变呈“毛肾”外观。CEUS和/或CTU可用于帮助鉴别RDD的孤立肿块与常见肿瘤，避免误诊导致不必要的肾切除术。
