Hida Akira I, Yagi Shin-ichi, Obase Yasushi, Nishimura Hirotake, Akiyama Takashi, Irei Isao, Hamazaki Shuji, Oka Mikio, Sadahira Yoshito
Department of Pathology, Kawasaki Medical School, Kurashiki, Japan.
Pathol Int. 2009 Apr;59(4):265-8. doi: 10.1111/j.1440-1827.2009.02362.x.
Rosai-Dorfman disease (RDD) involving an extranodal site is a diagnostic challenge. Reported herein is the case of a 67-year-old man who presented with a solitary superior mediastinal mass. The lesion was clinically suspected of malignancy including lymphoma because of its high uptake during a (67)Ga-scintigram and (18)F-fluorodeoxyglucose-positron emission tomography. There was no evidence of spread of the disease. Histology of thoracoscopic biopsy specimens indicated granulomatous lesion with infiltration of lymphocytes, plasma cells, and histiocytes with lymphocytes engulfed in their cytoplasm. The lesion did not contain lymph node or thymic elements. On immunohistochemistry the histiocytes were positive for S-100 protein, CD68, and CD163 but were negative for CD1a. These findings suggested a diagnosis of RDD. Despite lack of intervention, the lesion remained almost the same size for 3 years. To the best of the authors' knowledge this is the first case of RDD presenting as a solitary mediastinal mass.
结外Rosai-Dorfman病（RDD）的诊断具有挑战性。本文报告了一例67岁男性患者，其表现为孤立性上纵隔肿块。由于在镓-67闪烁扫描和氟-18氟脱氧葡萄糖正电子发射断层扫描中该病变摄取较高，临床上怀疑为包括淋巴瘤在内的恶性肿瘤。没有疾病播散的证据。胸腔镜活检标本的组织学检查显示为肉芽肿性病变，伴有淋巴细胞、浆细胞浸润，组织细胞胞质内可见吞噬的淋巴细胞。病变中不包含淋巴结或胸腺成分。免疫组织化学检查显示，组织细胞S-100蛋白、CD68和CD163呈阳性，但CD1a呈阴性。这些发现提示诊断为RDD。尽管未进行干预，但该病变3年来大小几乎不变。据作者所知，这是首例表现为孤立性纵隔肿块的RDD病例。
