Department of Thoracic Surgery, Aulss3 Serenissima Ospedale dell'Angelo, Venice Mestre, Italy.
Pathology Department, Aulss3 Serenissima Ospedale dell'Angelo, Venice Mestre, Italy.
Ann Thorac Surg. 2020 Jan;109(1):e45-e47. doi: 10.1016/j.athoracsur.2019.04.089. Epub 2019 Jun 14.
Rosai-Dorfman disease (RDD) is a rare benign disorder of the histiocytes, affecting lymph nodes in its classic form. Extranodal RDD is considered the uncommon subtype and potentially impairs all tissues and intrathoracic organs. In our report, a F-fluorodeoxyglucose positron emission tomography scan of a mediastinal mass infiltrating the lungs and of widespread lesions suggested the presence of a metastatic disease. Open thoracic biopsy was required for immunochemistry and histopathology. Mediastinal mass with lesions disseminated throughout the body is an unusual presentation of extranodal RDD that calls for a challenging differential diagnosis to rule out the suspicion of metastatic malignancy.
罗-道二氏病（RDD）是一种罕见的组织细胞良性疾病，以淋巴结受累为其经典表现。结外 RDD 被认为是罕见亚型，可能累及全身各种组织和胸内器官。本例患者为胸腔内纵隔肿块累及肺部，同时伴有广泛病变，氟-18 脱氧葡萄糖正电子发射断层扫描（FDG-PET）提示转移瘤可能。为明确诊断，需进行开胸活检以进行免疫组化和组织病理学检查。纵隔肿块伴全身播散性病变是结外 RDD 不常见的表现，需要进行具有挑战性的鉴别诊断，以排除转移性恶性肿瘤的可能。
