A Rare Case of Rosai-Dorfman Disease with Lacrimal Gland Involvement

淚腺之Rosai-Dorfman Disease－罕見病例報告

劉虹慧(Hung-Hui Liu) ； 黃致翰(Chih-Han Huang) ； 黃惇暐(Dun-Wei Huang) ； 徐國峰(Kuo-Feng Hsu) ； 陳錫根(Shyi-Gen Chen)

《臺灣整形外科醫學會雜誌》 29卷4期 (2020/12) Pp. 311-318

摘要

Background: The Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder, with 83%-95% of patients presenting with bilateral painless cervical lymphadenopathy. However, few patients had no lymphadenopathy and isolated extranodal disease. The cases of an isolated lacrimal gland without any systemic involvement are even more rare with only four case reports having been published, not including our case report1-3. Aim and Objectives: To report an extremely rare case of isolated extranodal RDD presenting with only lacrimal gland involvement. Materials and Methods: We present a 65-year-old man who experienced unilateral lacrimal gland no erythematous mass with compression ptosis. After direct excision, the histology proved a lacrimal gland RDD. Three years later, the patient had similar tumor on the other eyelid and again was proved the same diagnosis. We treated these tumors with direct complete excision through double fold of upper eyelid. No any systemic medical agents or radiotherapy was delivered. Results: The excised tumor is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Neither recurrence nor other systemic involvement was observed at 30 months after the last surgical excision. The compression ptosis relieved completely after removal of tumor. Conclusion: Rosai-Dorfman Disease with only lacrimal gland involvement is extremely rare especially developed in both sides of lacrimal gland within different time period. We successfully treated this patient with direct excision and achieved good result in a 30- month follow-up.

關鍵字

Rosai-Dorfman disease ； histiocytosis sinus mucosa ； lacrimal gland

並列摘要

背景：羅塞－朵夫曼病（Rosai-Dorfmandiseas，簡稱RDD）是一種罕見的組織球細胞增生疾病，約八九成的病人在臨床上會表現出雙側無痛性的頸部淋巴腺體腫大，只有十分少數的病人會沒有出現淋巴腫大的症狀而是以淋巴結外病兆來來表徵，而病兆單獨出現在眼部表的症狀而已徵的更為罕見，尤其是只涉及淚腺的羅塞－朵夫曼病更是到目前為止全球只有四個病例被報告過，而我們這個案例為第五例。目的及目標：報告一個極罕見只單獨影響淚腺的羅塞－朵夫曼疾病的案例並成功切除病灶得到良好結果。材料及方法：我們報告一名六十五歲男性因左側上眼皮腫瘤導致眼瞼下垂而來求診,施予腫瘤切除後，病理報告為羅塞－朵夫曼疾病。門診追蹤三年後，病人換右側上眼皮長出腫瘤，並導致輕微眼瞼下垂，我們一樣施予腫瘤切除，病理報告同樣為羅塞－朵夫曼疾病。對於此病人，兩次病程中，均只給予手術切除治療，無任何系統性免疫抑制藥物，類固醇，化療或放射性治療介入。結果：兩次切除術中的病兆在組織學上表現的特徵是密集的組織細胞浸潤以及混合性的發炎細胞浸潤混合有淋巴細胞，漿細胞和嗜中性粒細胞伴隨著。病人眼瞼下垂在手術治療後得到立即的改善，且沒有任何的併發症。病人之後追蹤30個月，均無任何局部或廣泛性復發。結論：病兆只單獨影響淚腺的羅塞－朵夫曼病是十分罕見的，尤其此病人是在不同時間裡分別經歷了兩次只分別單獨影響左右側淚腺的羅塞－朵夫曼病。我們成功的以單純手術切除的方式治療此患者，並在30個月的追蹤中取得療良好的效果。