Abstract:
Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disease of unknown etiology. Typically presenting with cervical adenopathy and constitutional symptoms, RDD involves bone in less than 10% of cases-and rarely presents as a primary intraosseous lesion. In this report, we describe the presentation of primary, bilateral intraosseous RDD, the first known case in the literature. Asymmetrically involving the lateral femoral condyles of a 59-year-old male, the lesion was discovered incidentally during evaluation and workup for giant cell tumor of bone involving the left tibia. Confirmation of the diagnosis required multiple biopsies and extensive evaluation-reflecting the diagnostic challenge associated with this case. We discuss the clinical, radiological, and pathological findings that allowed us to establish the diagnosis-as well as key differential diagnostic considerations and clinical outcome to date.
摘要:
Rosai-Dorfman病(RDD)是一种病因不明的罕见组织细胞增生性疾病。通常表现为宫颈腺病和全身症状,RDD在不到10%的病例中涉及骨骼,很少表现为原发性骨内病变。在这份报告中,我们描述了小学的呈现,双侧骨内RDD,文献中已知的第一个案例。不对称累及一名59岁男性的股骨外侧髁,在评估和检查累及左胫骨的骨巨细胞瘤时,偶然发现了病变。诊断的确认需要多次活检和广泛的评估-反映与该病例相关的诊断挑战。我们讨论临床,放射学,和病理发现,使我们能够确定诊断以及关键的鉴别诊断考虑因素和迄今为止的临床结果。