胸壁软组织Rosai-Dorfman病一例及文献复习

Chest wall soft tissues Rosai-Dorfman’s disease:a case report and literature review

目的:探讨1例胸壁软组织Rosai-Dorfman病患者临床病理特征,免疫组化,诊断与鉴别诊断,生物学行为和预后。方法:1例左侧胸壁Rosai-Dorfman病患者进行镜下病理观察及免疫组化检查,并结合相关文献复习。结果:切除标本肉眼见,实性结节状肿块,大小3.5 cm×3 cm×1.8 cm,灰黄,质中。镜下病变呈交替性分布的淡染带和深染带,淡染带为大片状或结节状分布的组织细胞组成,深染带为淋巴细胞和浆细胞浸润及纤维化。组织细胞体积较大,胞浆丰富呈泡沫状或嗜酸性,部分胞浆内可见"吞噬"淋巴细胞、浆细胞和红细胞。免疫组化示:大组织细胞表达CD11c、CD68、MAC387、S-100蛋白及溶菌酶,CD1a阴性。结论:软组织Rosai-Dorfman病是一种少见病,较少引起临床和病理医生足够重视,易误诊和误治。了解和掌握Rosai-Dorfman病的病理学特征并结合免疫表型可以明确诊断,病理诊断是目前唯一能够确诊软组织Rosai-Dorfman病的方法。软组织Rosai-Dorfman病以手术切除为主,应适当扩大手术范围,彻底切除病变组织以预防复发。

Objective:To explore the clinicopathologic features,immunohistochemical,diagnosis and differential diagnosis,biological behavior or prognosis of Rosai-Dorfman’s disease(RDD)in one of chest wall soft tissues.Methods:Histopathological features and immunohistochemical of a patient with RDD in the left chest was observed,and the literature was reviewed for analysis.Results:The gross of surgical specimens appears as solid nodule,3.5 cm×3 cm×1.8 cm,grayish yellow and Medium texture.Under light microscopy,pathological changes were composed of light and deep staining areas,the light staining areas consisted of histiocyte with large patchy and nodositas,the deep staining areas were composed of lymphocytes,plasma cells and fibrous tissue.Histiocyte with large volumes,rich foamy or eosinophilic cytoplasm,in which lymphocytes,neutrophils or red blood cell could be seen stretching into the movement.IHC:CDllc(+),CD68(+),MAC387(+),S-100(+),lysozyme(+),CDla(-).Conclusion:RDD of soft tissues is a rare disease,which has not been paid enough attention to by clinical and pathological doctors,and it may lead to misdiagnosed and mistreated.The diagnosis of RDD can be confirmed by pathological features and immunophenotype.Pathological diagnosis is the only way to diagnose RDD in soft tissue.The soft tissue RDD is mainly resected by operation.The scope of operation should be expanded properly and the diseased tissue should be completely cleared to prevent recurrence.