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Rosai-Dorfman 病表现为胸腺内有脂肪的单发肿块:一例报告。

Rosai-Dorfman disease manifesting as a solitary mass with fat in the thymus a case report.

机构信息

Department of Radiology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.

Department of Pathology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.

出版信息

J Cardiothorac Surg. 2024 Apr 4;19(1):173. doi: 10.1186/s13019-024-02714-x.

Abstract

BACKGROUND

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare, self-limiting disease that predominantly affects children and young adults. Moreover, the disease is characterized by painless bilateral cervical lymphadenopathy in 95% of the patients. However, few reports are available on the Rosai-Dorfman disease of the thymus.

CASE PRESENTATION

We report a rare case of thymic Rosai-Dorfman disease detected using computed tomography. During a medical examination, a 50-year-old man underwent a chest computed tomography scan, which revealed an anterior mediastinal single mass with fat in the thymus. A thymectomy was performed to completely remove the tumor using a thoracoscopic technique due to a clinical suspicion of thymoma. Furthermore, Rosai-Dorfman disease was confirmed using histological and immunohistochemical analyses.

CONCLUSIONS

To the best of our knowledge, this is the sixth case of thymus-affecting solitary Rosai-Dorfman disease with histological and immunohistochemical evidence. Fat in the thymus, as was present in this case, has never been described in Rosai-Dorfman disease previously. Our results highlight the challenge of diagnosing this uncommon tumor before surgery, and more cases need to be reported to help with the preoperative diagnosis of such a rare tumor.

摘要

背景

伴有巨大淋巴结病的窦组织细胞增生症,又称 Rosai-Dorfman 病,是一种罕见的、自限性疾病,主要影响儿童和年轻成人。此外,该病的特征是 95%的患者存在无痛性双侧颈淋巴结病。然而,关于胸腺 Rosai-Dorfman 病的报道很少。

病例介绍

我们报告了一例使用计算机断层扫描检测到的罕见胸腺 Rosai-Dorfman 病病例。在一次体检中,一名 50 岁男性接受了胸部计算机断层扫描,显示胸腺内有一个前纵隔单发肿块,内有脂肪。由于临床怀疑胸腺瘤,我们采用胸腔镜技术进行了胸腺切除术,以完全切除肿瘤。此外,通过组织学和免疫组织化学分析证实了 Rosai-Dorfman 病的诊断。

结论

据我们所知,这是第六例具有组织学和免疫组织化学证据的胸腺单发 Rosai-Dorfman 病。像本病例中那样的胸腺内脂肪以前从未在 Rosai-Dorfman 病中描述过。我们的结果强调了在手术前诊断这种罕见肿瘤的挑战,需要更多的病例报告来帮助此类罕见肿瘤的术前诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d93/10996155/4b92cc555cb6/13019_2024_2714_Fig1_HTML.jpg

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