皮肤Rosai-Dorfman病

目的探讨皮肤Rosai-Dorfman病(CRDD)的临床病理学特征、免疫学表型和鉴别诊断。方法分析8例CRDD患者的临床表现,对标本进行病理学和免疫组织化学(EnVision二步法,抗体包括S-100蛋白、CD68、CD1a、CD45、CD20、CD79a、CD3和CD45RO)染色观察。结果8例患者临床皮损均为四肢或躯干多个丘疹、结节和(或)融合斑块,无淋巴结肿大等其他系统性病变。镜下特点为真皮和(或)皮下脂肪组织内数量不一的S-100蛋白阳性窦组织细胞伴大量淋巴细胞和浆细胞浸润。10％～50％组织细胞胞质内见淋巴细胞伸入。病程较长者病灶内见席纹样排列硬化性纤维间质,夹杂个别微脓疡形成。2例脉管间隙内可见窦组织细胞。结论CRDD是一种良性组织细胞持续增生性病变,与巨淋巴结病性窦组织细胞增生症不同,系统累及可能极小,预后较好。组织学上应避免与其他类型组织细胞增生症、真皮隆突性纤维肉瘤、黄色瘤及皮肤淋巴组织增生性病变等混淆。免疫组织化学S-100蛋白、CD68等标记对鉴别诊断有帮助。     

淋巴结外Rosai-Dorfman病7例临床病理分析

目的探讨淋巴结外Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理特征、诊断及鉴别诊断。方法对7例结外RDD患者进行HE染色和免疫组化检查,并复习相关文献。结果 7例结外RDD患者中男性4例,女性3例,年龄21～57岁(平均41岁),4例位于中枢神经系统,胫骨、甲状软骨和皮下组织各1例,均为结外单发病变。组织学改变:病变组织在低倍镜下呈大小不一的不规则结节样结构,结节内"明暗"相间。高倍镜下"暗区"为浸润的淋巴细胞、浆细胞等;"明区"是散在、成簇或片状分布的组织细胞。后者体积大,胞质丰富,泡状核,可见小核仁。部分组织细胞胞质内见吞噬完整的淋巴细胞和浆细胞等("伸入"现象)。免疫表型:组织细胞S-100蛋白和CD68均阳性,CD1a阴性。结论结外RDD少见,因组织细胞吞噬现象不明显且临床及影像学特征缺乏特异性,易被误诊。结节状分布的结构特点以及低倍镜下明暗相间的组织学特征是诊断RDD的重要线索。免疫组化标记有助于明确诊断。     

上呼吸道结外Rosai-Dorfman病临床病理学观察

目的 探讨上呼吸道结外Rosai-Dorfman病的临床病理特征及鉴别诊断.方法 采用光镜及免疫组织化学LSAB法结合临床资料,对10例发生于上呼吸道的结外Rosai-Dorfman病进行临床病理学分析.结果 10例患者中,男性3例,女性7例,发病年龄20 ～61岁,平均38岁.肿瘤分别位于鼻腔鼻窦(7例)、鼻咽(2例)和硬腭气管(1例).患者多以鼻塞、鼻衄或鼻腔鼻咽新生物就诊.CT显示鼻腔鼻窦等相应部位占位性病变,无骨质破坏.组织学显示肿瘤由相互交错的淡染区与暗染区组成,淡染区见大圆形或多边形的组织细胞,部分组织细胞见吞噬现象;暗染区由聚集的淋巴细胞和浆细胞组成.免疫组织化学染色显示组织细胞弥漫强阳性表达S-100蛋白,部分表达CD68等.随访6例,经手术治疗均健在,无复发.结论 上呼吸道结外Rosai-Dorfman病较为少见,多发生于鼻腔鼻窦,形态学上易与鼻硬结病相混淆,常需免疫组织化学染色加以鉴别.     

Rosai-Dorfman病的临床病理、免疫表型和病原学观察

目的观察Rosai—Doffman病的临床病理特征,探讨多种抗原标记的免疫表型及HHV8-DNA、HPV—DNA和EBER的表达及其与病因的关系,并比较国内外Rosm—Doffman病的发病特征。方法对16例Rosm-Doffman病进行临床病理回顾性研究;9例行免疫表型检测,包括CD163、CD68（PG—M1）、CD21、CD1a、CD20、CIMSRO、CD8、CIM、S100、M—CSF和HHV8;用原位杂交技术进行EBV—mRNA和HPV—DNA检测。结果（1）男女之比为4．33：1。只有淋巴结病变的占62．5％（10／16）,颈淋巴结多见,50％的病例有多部位淋巴结累及;结外病变占37．5％（6／16）。结外病变者的局部复发率较高。（2）淋巴结病变以明显扩张的淋巴窦内有不等量体积大、多角形、胞质内含有淋巴细胞和浆细胞的组织细胞为特征;结外病变均有不同程度纤维组织增生和以浆细胞为主的混合炎细胞浸润,胞质内含有淋巴细胞和浆细胞的大多角形组织细胞数量多寡不一,呈簇状或散在分布,且随病程进展,纤维组织的量渐多,特征性组织细胞数量渐少。（3）病变组织中大多角形组织细胞均表达S—100蛋白,以及CD68PG—M1、CD163和M—CSF,均不表达CD1a和CD21;组织细胞吞入的有T和B细胞,以T细胞为多,且CIM和CD8表型细胞均有。9例均为HHV8和EBEV阴性。（4）国内报道共62例,其中仅淋巴结病变34例,仅结外病变18例,淋巴结和结外同时存在病变10例。比较国内外文献,国外患者年龄较国内的年轻10岁以上,且女性比例较高。结论Rosai—Dofman病在国内较少。结外Rosai—Dorfman病的诊断有一定难度。HHV8-DNA、HPV—DNA和EBER的表达与病因关联不明确。国内外患者的发病年龄和性别构成不同。     

Rosai-Dorfman disease of the breast mimicking cancer

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare proliferative histiocytic disorder of the lymph nodes. Extranodal involvement occurs in a considerable number of cases; however, involvement of the breast is very rare, and it is even rarer for the lesion to be localized in the breast alone without affecting any other sites. This report describes the case of a 50-year-old Brazilian woman with a lump confined to her left breast that had clinical and radiological characteristics indistinguishable from cancer. The proliferation of histiocytes, displaying lymphophagocytosis and an S-100 protein immunophenotype on a core biopsy of the lesion, led to a diagnosis of Rosai-Dorfman disease and permitted conservative therapy. Recognition of this rare condition, when occurring at an unexpected site such as the breast, is difficult, and the correct diagnosis is important prior to therapeutic management.     

Extranodal Rosai-Dorfman disease: a clinicopathologic analysis of 7 cases

目的 探讨淋巴结外Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理特征、诊断及鉴别诊断.方法 对7例结外RDD患者进行HE染色和免疫组化检查,并复习相关文献.结果 7例结外RDD患者中男性4例,女性3例,年龄21～57岁(平均41岁),4例位于中枢神经系统,胫骨、甲状软骨和皮下组织各1例,均为结外单发病变.组织学改变:病变组织在低倍镜下呈大小不一的不规则结节样结构,结节内"明暗"相间.高倍镜下"暗区"为浸润的淋巴细胞、浆细胞等;"明区"是散在、成簇或片状分布的组织细胞.后者体积大,胞质丰富,泡状核,可见小核仁.部分组织细胞胞质内见吞噬完整的淋巴细胞和浆细胞等("伸入"现象).免疫表型:组织细胞S-100蛋白和CD68均阳性,CD1a阴性.结论 结外RDD少见,因组织细胞吞噬现象不明显且临床及影像学特征缺乏特异性,易被误诊.结节状分布的结构特点以及低倍镜下明暗相间的组织学特征是诊断RDD的重要线索.免疫组化标记有助于明确诊断.     

Unusual bilateral renal histiocytosis. Extranodal variant of Rosai-Dorfman disease.

A 37-year-old Bangladeshi presented with large bilateral masses involving the hilus of the kidneys. No lymphadenopathy was noted. Nephrectomy was performed. Histopathologically, it revealed a lymphohistiocytic and plasma cell inflammatory tumoral proliferation with characteristic lymphophagocytosis by the S100-positive-CD1-negative histiocytes. Extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) should be entertained in the differential diagnosis of bilateral masses involving the kidneys.     

Extranodal Rosai-Dorfman disease of the kidney and coexistent poorly differentiated prostatic adenocarcinoma

We present a case of a patient who was initially diagnosed with poorly differentiated prostatic adenocarcinoma on prostate needle core biopsy. Upon staging workup, a computed tomographic scan showed a 9-cm left renal mass involving mainly the pelvicalyceal system. Positron emission tomographic scan showed increased uptake in para-aortic, paracaval, and retrocaval lymph nodes suspicious for metastatic disease. Left nephrectomy revealed involvement with extranodal Rosai-Dorfman disease. Lymphadenectomy revealed metastatic prostatic adenocarcinoma; however, the lymph nodes did not show evidence of Rosai-Dorfman disease. Isolated involvement of the kidney by Rosai-Dorfman disease is very rare. The combination of prostatic adenocarcinoma and isolated extranodal Rosai-Dorfman disease of the kidney makes this case unique.     

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

A child of 11 years with a solitary involvement of the right radius by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is presented. The literature of bone involvement in this condition is reviewed.