Rathinam Arun, Gupta Sushan, Khanam Rukhsaar, Sahai Tanmay
Internal Medicine, Carle Foundation Hospital, Urbana, USA.
Hematology and Oncology, Carle Foundation Hospital, Urbana, USA.
Cureus. 2023 Jul 3;15(7):e41334. doi: 10.7759/cureus.41334. eCollection 2023 Jul.
Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder primarily involving lymph nodes. Extranodal RDD has a heterogenous presentation, and isolated pulmonary involvement is rare. We report the only case of RDD presenting as an isolated pleural mass. Our patient was a 55-year-old female with multiple comorbidities who presented with chest pain. Imaging revealed an enlarging pleural-based lesion. She underwent resection of the pleural mass, showing an atypical histiocytic infiltrate in a prominent background of collagenous fibrosis. Immunohistochemistry shows CD1a-negative and S100-positive atypical histiocytic cells demonstrating emperipolesis, confirming the diagnosis of RDD. She is currently on six-month CT surveillance with no recurrence of the disease. This case highlights the unique pulmonary presentation of RDD. It also underscores that observations may be appropriate in isolated asymptomatic pleural involvement cases.
罗萨伊-多夫曼病（RDD）是一种罕见的非朗格汉斯组织细胞增多症，主要累及淋巴结。结外RDD表现多样，孤立性肺部受累罕见。我们报告了唯一一例表现为孤立性胸膜肿块的RDD病例。我们的患者是一名55岁女性，有多种合并症，因胸痛就诊。影像学检查发现一个逐渐增大的胸膜下病变。她接受了胸膜肿块切除术，病理显示在显著的胶原纤维背景中有非典型组织细胞浸润。免疫组化显示CD1a阴性、S100阳性的非典型组织细胞有吞噬现象，确诊为RDD。她目前每六个月接受一次CT监测，疾病无复发。该病例突出了RDD独特的肺部表现。它还强调，对于孤立性无症状胸膜受累病例，观察可能是合适的。
