DIAGNOSIS AND MANAGEMENT OF EXTRANODAL ROSAI-DORFMAN DISEASE IN NASAL SINUSES
- VernacularTitle:鼻窦部结外Rosai-Dorfman病的诊治
- Author:
Lei LEI
;
Dingrong ZHONG
;
Rongguang WANG
;
Al ET
;
- Publication Type:Journal Article
- Keywords:
Rosai Dorfman disease;
sinus histiocytosis with massive lymphadenopathy;
paranasal sinuses;
diagnosis, differential
- From:
Medical Journal of Chinese People's Liberation Army
2001;0(11):-
- CountryChina
- Language:Chinese
-
Abstract:
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.
