Rosai-Dorfman Disease Presenting as Unilateral Neck Masses-Case Report

以單側頸部腫塊表現之羅塞－朵夫曼病－病例報告

陳佳郡(Chia-Chun Chen) ； 張世倫(Shih-Lun Chang) ； 張聖宗(Sheng-Tsung Chang)

《台灣耳鼻喉頭頸外科雜誌》 56卷3期 (2021/09) Pp. 197-201

https://doi.org/10.6286/jtohns.202109_56(3).197

摘要

Rosai-Dorfman disease is a rare disorder with a prevalence of 1 : 200,000. Most patients present with bilateral, massive and painless cervical lymphadenopathy, while extranodal involvement has been reported in 43% cases. There is no ideal protocol for the treatment, and multiple strategies of therapy have been used with varying success, including observation, surgery, corticosteroids, chemotherapy, radiotherapy and immunomodulatory therapy. We presented a case of a 49-year-old woman with Rosai-Dorfman disease presenting as unilateral neck masses, treated with surgical excision with no local recurrence noted 6 months postoperatively. Due to its rarity, alternating symptoms and various treatment strategies, we reported this case and reviewed the relevant literature regarding the diagnosis and treatment. We aimed at highlighting the importance of a thorough evaluation for neck masses, which might be a sign of Rosai-Dorfman disease requiring further systemic survey.

關鍵字

neck mass ； Rosai-Dorfman disease

並列摘要

羅塞－朵夫曼病（Rosai-Dorfman disease）為一種罕見疾病，盛行率為1:200,000。臨床表現多為無痛之雙側頸部淋巴結腫大，但有43%的病人會以淋巴結外的形式表現。目前治療方法並無單一準則，如觀察追蹤、手術、類固醇治療、化學治療、放射治療及免疫製劑治療等，皆有文獻提及。本病例報告為1名49歲女性，患有右側頸部腫塊2週，藥物治療無效，經手術切除後確診為羅塞－朵夫曼病，術後追蹤至今6個月，並無局部頸部淋巴結復發。由於羅塞－朵夫曼病為罕見疾病，且臨床表現及治療方式多元，我們特此提出此病例報告並回顧相關文獻資料，以強調針對頸部腫塊之病患進行徹底評估的重要性，若診斷為羅塞－朵夫曼病則需進一步的全身系統性檢查。

並列關鍵字

neck mass ； Rosai-Dorfman disease（頸部腫塊 ； 羅塞－朵夫曼病）