Xu Jun, Xia Tian, Li Zhongcheng, Zhao Jun, Ju Sheng
Department of Thoracic Surgery, The First Affiliated Hospital of Soochow University, 899 Pinghai Road, Suzhou, 215000, China.
Institute of Thoracic Surgery, The First Affiliated Hospital of Soochow University, Suzhou, 215000, China.
J Cardiothorac Surg. 2024 Dec 4;19(1):640. doi: 10.1186/s13019-024-03147-2.
Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disorder. It may be associated with autoimmune diseases, but there are few reports of concurrent ankylosing spondylitis. RDD is typically characterized by massive bilateral and painless cervical lymphadenopathy, which can also involve extranodal sites and constitutional symptoms, but rarely affect the chest cavity.
In this report, we present a case of a patient with a suspicious invasive anterior mediastinal lesion affecting multiple blood vessels and adjacent tissues. The patient has a history of ankylosing spondylitis. Despite extensive radiological and interventional examinations, the possibility of malignancy could not be completely ruled out. Following surgical resection of the tumor, the final pathology diagnosis suggested RDD.
Our report emphasizes the importance of considering RDD in the differential diagnosis of invasive thoracic malignancies. Thoracoscopy or mediastinoscopy biopsy can improve diagnostic accuracy. Our study contributes to an improved understanding among clinicians regarding the diagnosis and treatment of intrathoracic RDD and provides relevant evidence for future exploration of potential associations between RDD and ankylosing spondylitis.
罗萨伊-多夫曼病（RDD），也称为伴巨大淋巴结病的窦性组织细胞增生症，是一种罕见的组织细胞疾病。它可能与自身免疫性疾病有关，但并发强直性脊柱炎的报道很少。RDD的典型特征是双侧巨大且无痛的颈部淋巴结肿大，也可累及结外部位和全身症状，但很少影响胸腔。
在本报告中，我们展示了一例患者，其前纵隔有可疑的侵袭性病变，累及多条血管和相邻组织。该患者有强直性脊柱炎病史。尽管进行了广泛的放射学和介入检查，但仍不能完全排除恶性肿瘤的可能性。肿瘤手术切除后，最终病理诊断为RDD。
我们的报告强调了在侵袭性胸部恶性肿瘤的鉴别诊断中考虑RDD的重要性。胸腔镜或纵隔镜活检可提高诊断准确性。我们的研究有助于临床医生更好地理解胸内RDD的诊断和治疗，并为未来探索RDD与强直性脊柱炎之间的潜在关联提供相关证据。
