Oramas Diana M, Moran Cesar A
Department of Pathology, The University of Alabama at Birmingham, Birmingham, AL, United States.
University of Texas, MD Anderson Cancer Center, Houston, TX, United States.
Pathol Res Pract. 2022 Jun;234:153917. doi: 10.1016/j.prp.2022.153917. Epub 2022 Apr 26.
Three cases of primary Rosai-Dorfman disease of the thymus and lung are presented. The patients are 3 men between the ages of 42 and 47 years who presented with non-specific symptoms including cough, chest pain, and shortness of breath. Clinically, the patients did not have any other pertinent clinical history. Diagnostic imaging revealed in one patient a cystic anterior mediastinal mass, while in two other patients the imaging was that of an intrapulmonary mass, one in the right upper lobe and the other in the left lower lobe. The three patients undergo surgical resection of the mass. In the cases in which the tumor mass was in the lung, both patients had a lobectomy while in the patient with anterior mediastinal mass, surgical resection via thoracotomy was performed. The intrapulmonary tumors were described as soft and yellowish measuring 2.5 and 3.0 cm in greatest diameter, while the mediastinal mass was described as cystic measuring 4.0 cm in diameter. Histologically, all tumors show similar features in terms of a proliferation of large histiocytes admixed with an inflammatory component composed predominantly of plasma cells. Immunohistochemical stains show positive staining for CD68 and S-100 protein, while negative for keratin, CD1a, and langerin. The cases herein presented highlight the ubiquitous distribution of Rosai-Dorfman disease and the importance of keeping this entity in the differential diagnosis of histiocytic proliferation in the thymus or lung.
本文报告了3例胸腺和肺原发性Rosai-Dorfman病。患者为3名男性，年龄在42至47岁之间，表现为咳嗽、胸痛和呼吸急促等非特异性症状。临床上，患者无其他相关病史。诊断性影像学检查显示，1例患者为囊性前纵隔肿块，另外2例患者为肺内肿块，1例位于右上叶，另1例位于左下叶。3例患者均接受了肿块的手术切除。在肿瘤位于肺部的病例中，2例患者均接受了肺叶切除术，而对于前纵隔肿块患者，则通过开胸手术进行了手术切除。肺内肿瘤质地柔软，呈淡黄色，最大直径分别为2.5 cm和3.0 cm，而纵隔肿块为囊性，直径4.0 cm。组织学上，所有肿瘤在大组织细胞增生并伴有主要由浆细胞组成的炎症成分方面表现出相似特征。免疫组化染色显示CD68和S-100蛋白呈阳性染色，而角蛋白、CD1a和朗格汉斯蛋白呈阴性染色。本文报道的病例突出了Rosai-Dorfman病的广泛分布以及在胸腺或肺组织细胞增生的鉴别诊断中考虑该疾病的重要性。
