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病例报告:罗萨伊-多夫曼病在肝脏的罕见表现酷似肝癌。

Case report: Uncommon manifestations of Rosai-Dorfman disease in the liver mimicking HCC.

作者信息

Ren Huipeng, Zhang Hao, Wan Qinyun, Pang Yuhui, Tian Hongzhe, Ren Zhuanqin, Cai Yuan

机构信息

Department of Medical Imaging, Baoji Central Hospital, Baoji, China.

Department of Pathology, Baoji Central Hospital, Baoji, China.

出版信息

Front Oncol. 2024 Jun 28;14:1408353. doi: 10.3389/fonc.2024.1408353. eCollection 2024.

Abstract

Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis (LCH) disorder characterized by systemic extranodal lesions. Common cases include skin lesions, whereas liver lesions are rare. This study presents a case of a 66-year-old woman with a solitary extranodal liver lesion who underwent successful surgical treatment followed by glucocorticoid therapy. The patient did not experience any symptoms before surgery. The liver lesion was incidentally discovered during a routine ultrasound examination. Enhanced CT scan revealed the lesion with the characteristic of washout, similar to primary hepatic cancer (HCC). CT scans of the head, neck, chest, and abdominal pelvis revealed no lymph node or other organ lesions. After surgery, the liver lesion was diagnosed as RDD, and subsequent whole-body examinations did not reveal any skin lesions. The definitive diagnosis was solid liver RDD in adults. Although there were no typical cases of bilateral cervical lymph node lesions, ultrasound and CT examinations promptly detected liver lesions, leading to the correct diagnosis through surgical resection. The findings from this case indicate that RDD can occur in rare extrasegmental areas, and the imaging characteristics of liver lesions are not specific, indicating the importance of avoiding delayed diagnosis.

摘要

罗萨伊-多夫曼-德斯顿贝斯病(RDD)是一种罕见的非朗格汉斯细胞组织细胞增多症(LCH)疾病,其特征为系统性结外病变。常见病例包括皮肤病变,而肝脏病变则较为罕见。本研究报告了一例66岁女性患者,该患者有一个孤立的结外肝脏病变,接受了成功的手术治疗并随后接受了糖皮质激素治疗。患者在手术前没有任何症状。肝脏病变是在常规超声检查中偶然发现的。增强CT扫描显示该病变具有廓清特征,类似于原发性肝癌(HCC)。头部、颈部、胸部和腹部盆腔的CT扫描未发现淋巴结或其他器官病变。手术后,肝脏病变被诊断为RDD,随后的全身检查未发现任何皮肤病变。最终诊断为成人实性肝脏RDD。尽管没有双侧颈部淋巴结病变的典型病例,但超声和CT检查及时发现了肝脏病变,通过手术切除得以正确诊断。该病例的研究结果表明,RDD可发生在罕见的节外区域,肝脏病变的影像学特征不具有特异性,这表明避免延迟诊断的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a624/11239378/5f885de33fce/fonc-14-1408353-g001.jpg

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