鼻窦部结外Rosai-Dorfman病的诊治

目的　探讨鼻窦部结外Rosai Dorfman病的临床表现、组织学特征、诊断、鉴别诊断及治疗。方法　分析国内罕见的发生于鼻窦的结外Rosai Dorfman病的临床、组织学和影像学资料。结果　病变位于鼻窦 ,曾先后多次复发、手术切除并病理误诊。组织学分析可见大量梭形细胞混有泡沫细胞和少量浆细胞 ,胞质丰富呈泡沫样。免疫组化分析显示 ,组织细胞呈S 10 0和CD6 8阳性。结论　鼻窦部结外Rosai Dorfman病诊断困难 ,易误诊 ,临床应与鼻硬结病、嗜酸性肉芽肿、浆细胞瘤、良性纤维组织细胞瘤等进行鉴别 ,免疫组化细胞呈S 10 0、CD6 8阳性有助诊断。此病可采用化疗、放疗和手术治疗等综合治疗 ,对重要部位的病变侵犯 ,彻底的手术切除是最有效治疗方法 ,术后应跟踪随访

DIAGNOSIS AND MANAGEMENT OF EXTRANODAL ROSAI-DORFMAN DISEASE IN NASAL SINUSES

Objective To evaluate the clinical manifestations, pathological features, diagnosis, differential diagnosis and treatment of extranodal Rosai Dorfman disease(RDD) in paranasal sinuses. Methods The clinical manifestations, pathological findings, and imaging data of a rare case of extranodal RDD in the paranasal sinuses, who had received repeated surgical interventions, were analyzed. Results The lesion was situated in the paranasal sinuses, It had been repeatedly removed and misdiagnosed. Physical examination showed a red spherical mass in the nasal cavity. CT scan of the sinuses revealed a 5cm 5 cm soft tissue opacification of the right nasal cavity and paranasal sinuses. There was also erosion of the lateral wall of the maxillary sinus, the medial wall of orbit, parts of pterygopalatine fossa, and infratemporal fossa. Through the previous incision, all visible tumours in the lateral nasal cavity, paranasal sinuses, pterygopalatine fossa and orbital base were again removed. The tumour was attached to the orbital periosteum. The orbit was not removed, but the tumour was stripped off as completely as possible. Microscopic examination revealed intermingling spindled shaped and polygonal histiocytes, admixing with few plasma cells. The infiltrating histiocytes had large round or oval nuclei. The cytoplasm was abundant, granular or vacuolated and foamy. Immunohistochemical studies showed that the histiocytes were strongly reactive with antibodies against S100 protein and Vimetin. Conclusion The diagnosis of extranodal RDD in the paranasal sinuses should be differentiated with rhinoscleroma, eosinophilic granuloma, plasmacytoma, or fibrous histiocytoma. The identification of the distinctive histocytes which are typically immunoreactive to S 100 and CD68 can be quite helpful for correct diagnosis. Surgical excision supplemented by chemotherapy and radiotherapy, may be the optimal treatment, and long term follow up is essential.