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胸腺瘤样窦组织细胞增生症:一例报告。

Thymic Rosai-Dorfmann disease: a case report.

机构信息

Department of Cardiothoracic Surgery, The First People's Hospital of Neijiang, Sichuan, 641000, China.

Department of Pathology, The First People's Hospital of Neijiang, Sichuan, 641000, China.

出版信息

J Cardiothorac Surg. 2024 Jun 21;19(1):351. doi: 10.1186/s13019-024-02919-0.

Abstract

BACKGROUND

Rosai-Dorfman disease (RDD), known as sinus histiocytosis with massive lymphadenopathy, commonly involves lymph nodes in the neck or mediastinum, although extranodal involvement is observed in approximately 40% of RDD patients. RDD involving only the thymus has rarely been reported. Here, we report a case of RDD originating in the thymus. The lesion was surgically removed, and a cure was finally achieved. There was no recurrence after telephone follow-up for 3 years.

CASE PRESENTATION

A 52-year-old male was accidentally found to have a 7 × 6 cm anterior mediastinum lump by chest computed tomography (CT). The mediastinal lesion was resected by surgery, and postoperative pathology revealed RDD originating from the thymus. Regular telephone follow-up after surgery lasted 3 years and showed that the patient remained in good condition without any relevant symptoms.

CONCLUSIONS

RDD originating in the thymus cannot be characterized from CT images and is easily misdiagnosed as a traditional mediastinal tumor. This is mainly because there is so little disease in this area that physicians are not aware of it. We report this case with the hope that clinicians will have a better understanding of this disease. According to our follow-up results, surgery is an effective means of treatment.

摘要

背景

罗道尔夫-多夫曼病(RDD),又称窦组织细胞增生伴巨大淋巴结病,常累及颈部或纵隔淋巴结,但约 40%的 RDD 患者存在结外累及。仅累及胸腺的 RDD 很少见。在此,我们报告一例起源于胸腺的 RDD。病变通过手术切除,最终治愈。电话随访 3 年无复发。

病例介绍

一名 52 岁男性因胸部 CT 偶然发现前纵隔 7×6cm 肿块。纵隔病变通过手术切除,术后病理显示 RDD 起源于胸腺。手术后定期电话随访 3 年,患者状况良好,无相关症状。

结论

起源于胸腺的 RDD 不能从 CT 图像上进行特征描述,容易误诊为传统纵隔肿瘤。这主要是因为该区域的疾病非常少见,医生没有意识到。我们报告这个病例,希望临床医生能更好地了解这种疾病。根据我们的随访结果,手术是一种有效的治疗手段。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a198/11191219/bca89bb2c8f4/13019_2024_2919_Fig1_HTML.jpg

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