Hospital Espanhol, Federal University of Bahia, Salvador, Brazil.
J Bras Pneumol. 2009 Jul;35(7):717-20. doi: 10.1590/s1806-37132009000700015.
Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.
罗道尔夫-多夫曼病(RDD)由窦组织细胞增生伴巨大淋巴结病组成。多达 43%的病例会出现结外累及。然而,孤立性软组织 RDD 较为罕见。孤立性纵隔 RDD 极为罕见,仅有三例既往报道。RDD 后纵隔受累仅在播散性 RDD 的情况下有报道。在此,我们报告了一例 49 岁女性患者,其病史为两年的颈部疼痛和淋巴结肿大,且自行缓解。CT 扫描显示左侧椎旁有一个直径为 6 厘米的肿块。患者接受了肿块的手术切除。手术标本的显微镜检查和免疫表型分析导致了 RDD 的诊断。在 12 个月的随访期间,患者主诉轻度咳嗽和胸痛。定期影像学检查未显示复发迹象,且未发现术后颈部淋巴结肿大。