CAJ | 학술논문

目的探讨多发性Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理特征、诊断、鉴别诊断及预后.方法对7例多发性RDD进行组织形态学、免疫组织化学及特殊染色检查,并随访及复习相关文献,进行总结分析.结果 7例病例中,女性3例,男性4例,年龄18 ～ 60岁(中位年龄45.7岁),肿块最大径0.8～6.0 cm(平均为3.0 cm).肿块分别位于:左手臂皮下、甲状腺、气管旁;左上颌皮下、右颊皮下;左胸壁皮下、右腹股沟皮下;双侧颈部皮下;有胫骨上段、右大腿皮肤;右额叶、大脑廉旁;双侧颈部淋巴结.其中例1～6均为淋巴结外组织,而例7为淋巴结.7例均表现为渐进性增大的肿块.病理组织学上,7例表现相似,呈细胞疏松区和密集区交替分布的结节状结构,细胞疏松区可见淋巴窦样结构,其间有一些体积较大、胞质丰富淡染的组织细胞,可见胞质内吞噬物和淋巴细胞“伸入运动”;细胞密集区可见较多淋巴细胞浆细胞、中性粒细胞等浸润.其中2例中可见散在淋巴滤泡浸润.免疫表型:组织细胞呈S-100蛋白、CD163及CD68阳性.结论多发性RDD罕见,确诊主要依靠形态学及免疫组织化学标记,临床及病理均需与肉芽肿性疾病、IgG4相关的硬化性疾病、炎性肌纤维母细胞瘤、纤维组织细胞瘤、Langerhans细胞增生症等相鉴别,治疗以手术切除为主,预后较好.
목적 탐토다발성Rosai-Dorfman병(Rosai-Dorfman disease,RDD)적림상병리특정、진단、감별진단급예후.방법 대7례다발성RDD진행조직형태학、면역조직화학급특수염색검사,병수방급복습상관문헌,진행총결분석.결과 7례병례중,녀성3례,남성4례,년령18 ～ 60세(중위년령45.7세),종괴최대경0.8～6.0 cm(평균위3.0 cm).종괴분별위우:좌수비피하、갑상선、기관방;좌상합피하、우협피하;좌흉벽피하、우복고구피하;쌍측경부피하;유경골상단、우대퇴피부;우액협、대뇌렴방;쌍측경부림파결.기중례1～6균위림파결외조직,이례7위림파결.7례균표현위점진성증대적종괴.병리조직학상,7례표현상사,정세포소송구화밀집구교체분포적결절상결구,세포소송구가견림파두양결구,기간유일사체적교대、포질봉부담염적조직세포,가견포질내탄서물화림파세포“신입운동”;세포밀집구가견교다림파세포장세포、중성립세포등침윤.기중2례중가견산재림파려포침윤.면역표형:조직세포정S-100단백、CD163급CD68양성.결론 다발성RDD한견,학진주요의고형태학급면역조직화학표기,림상급병리균수여육아종성질병、IgG4상관적경화성질병、염성기섬유모세포류、섬유조직세포류、Langerhans세포증생증등상감별,치료이수술절제위주,예후교호.
Objective To investigate the clinicopathologic features,diagnosis,differential diagnosis of multiple Rosai-Dorfman disease (RDD).Methods Seven cases of multiple RDD were treated in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command of Chinese PLA.The disease was analyzed,focusing on the process of diagnosis,the treatment and follow-up.Histopathology,immunohistochemical profiles and relative literature were reviewed to reveal the characteristics of this disease.Results The seven cases,occurred in 3 women and 4 men from 18 to 60 years of age (median 45.71 years),with masses measured of 0.8-6.0 cm (average size of 3.0 cm).Masses located in,left subcutaneous arm,thyroid,paratrachea,left maxilla,right subcutaneous cheek,left subcutaneous chest wall,right subcutaneous inguina,bilateral subcutaneous neck,right tibia,right thigh skin,right frontal lobe of brain,cerebral parafalx and bilateral lymph nodes of the neck,respectively.Among the cases,from the first case to the sixth case were extranodal tissue,and the seventh case was located in lymph nodes.Cases showed progressive increase of the mass.Histologically,all lesions of seven cases were similar with nodular structures presenting with alternating hyper-and hypo-cellular areas.The hypo-cellular areas revealed lymphsinustoid structure characterized by variable numbers of large histiocytes,which had an abundant cytoplasm,pale to eosinophilic in appearance,phagocytozed intact lymphocytes or emperipolesis.While hyper-cellular areas revealed the infiltration of lymphocytes,plasma cells,neutrophils and numerous collagen fiber.Two cases also revealed the infiltration of lymphoid follicles.Immunohistochemically,the large histiocytes were strongly positive for S-100,CD163 and CD68 protein.Conclusions Multiple RDD is rare.In clinic and pathology,it needs to be differentiated from granulomatous diseases,IgG4-related sclerotic diseases,inflammatory myofibroblastic tumor,fibrohistiocytoma,Langerhans cell histiocytosis,and so on.The primary approach of treatment for multiple RDD is complete surgical excision and its prognosis is good.