Rosai-Dorfman氏病為一原因不明之罕見疾病,常見臨床表現為廣泛性無痛性頸部淋巴結腫大,淋巴結外侵犯可發生於鼻腔、呼吸道、皮膚、眼眶、骨骼等,通常是自限性良性疾病。本文報告一鼻竇炎術後鼻腔內Rosai-Dorfman氏病:62歲女性,十年前接受過雙側功能性鼻竇內視鏡手術,於門診主訴鼻涕痰液間斷性有血絲數個月,鼻咽內視鏡檢查右側下鼻甲內側上方一息肉樣鼻腫瘤,電腦斷層檢查顯示,右側鼻腫瘤合併右側上頜竇鼻竇炎。術後病理報告顯示一發炎性病灶,其包含大量泡沫組織球(foamy histiocyte,細胞質呈現泡沫狀,組織免疫染色呈現S-100/CD3/CD30/CD56/D68陽性)、淋巴球、漿細胞等發炎細胞之浸潤,同時可見胞飲現象(emperipolesis)。綜合以上病理發現,證實為Rosai-Dorfman氏病。本文除了分享我們的治療經驗,並回顧了Rosai-Dorfman氏病相關的文獻。
Rosai-Dorfman disease, also known as sinus histiocytosis, is a rare disease of an unknown etiology. The common presentations are bilateral painless cervical lymphadenopathy. Extranodal invasion includes the nose, respiratory tract, skin, eyes, bone, and nerves. We report a case of nasal Rosai-Dorfman disease in a 62-year-old woman with a history of endoscopy sinus surgery. She was sent to our hospital because of intermittent blood-stained nasal discharge for months. A nasopharyngoscopy examination revealed a right side nasal tumor medial to the right side of the upper inferior turbinate. Computed tomography revealed a right side nasal tumor near the orifice of the maxillary sinus with right side maxillary sinusitis. We performed endoscopy sinus surgery to resect the tumor. Pathological examination revealed an inflammatory lesion with a heterogeneous population including lymphocytes, plasma cells, and histiocytes. Focal emperipolesis was observed. Immunostaining revealed an S100 positive result. Based on morphology and immunostaining results, Rosai-Dorfman disease was diagnosed. No recurrence has been observed until now. We discuss the present case and review the literature on Rosai-Dorfman disease.