Al-Maghrabi Haneen, Elmahrouk Ahmed, Feteih Maun, Jamjoom Ahmed, Al-Maghrabi Jaudah
Department of Pathology, King Faisal Specialist Hospital and Research Center, MBC-J16, P.O. Box 40047, Jeddah, 21499, Saudi Arabia.
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.
J Cardiothorac Surg. 2020 Feb 21;15(1):37. doi: 10.1186/s13019-020-1085-6.
Rosai-Dorfman disease is a histiocytic lesion that affects lung rarely.
We present a 52-year-old female diagnosed with right intrabronchial mass invading the bronchial wall and the extrabronchial tissues with lymphadenopathy. Multiple bronchoscopic biopsies were not diagnostic. Pneumonectomy was performed and postoperative histology revealed marked mucin impaction and bronchial dilatation. The pulmonary tissue showed areas of hemorrhage and chronic inflammation. The mass exhibited an excessive number of lymphocytes, plasma cells, and numerous histiocytes engulfing them (lymphocytophagocytosis). These histiocytes were S100 protein and CD68 positive. These features are consistent with Rosai-Dorfman disease.
Rosai-Dorfman Disease with pulmonary affection can be misdiagnosed as malignancy. Careful histological examination of the specimen for emperipolesis or lymphocytophagocytosis together with S100 protein and CD68 positivity are the clue for proper diagnosis.
罗萨伊-多夫曼病是一种组织细胞病变，很少累及肺部。
我们报告一例52岁女性，诊断为右支气管内肿块，侵犯支气管壁和支气管外组织并伴有淋巴结病。多次支气管镜活检均未明确诊断。行肺切除术，术后组织学检查显示明显的黏液嵌塞和支气管扩张。肺组织可见出血和慢性炎症区域。肿块中有大量淋巴细胞、浆细胞，并有许多吞噬它们的组织细胞（淋巴细胞吞噬现象）。这些组织细胞S100蛋白和CD68呈阳性。这些特征符合罗萨伊-多夫曼病。
累及肺部的罗萨伊-多夫曼病可能被误诊为恶性肿瘤。仔细检查标本中是否存在血细胞吞噬或淋巴细胞吞噬现象以及S100蛋白和CD68阳性是正确诊断的线索。
