Extranodal Rosai-Dorfman Disease with Paranasal Sinuses and Intracranial Involvement: A Case Report

鼻竇腔及顱內之淋巴結外Rosai-Dorfman disease：病例報告

黃于庭(Yu-Ting Huang) ； 吳樹鏗(Shu-Hang Ng) ； 高常發(Sheung-Fat Ko) ； 黃浩輝(Ho-Fai Wong) ； 陳耀亮(Yao-Liang Chen) ； 黃敏政(Mun-Ching Huang) ； 杜振豐(Cheng-Hong Toh) ； 衛優遊(Yu-Yau Wai)

《中華放射線醫學雜誌》 34卷3期 (2009/09) Pp. 191-196

摘要

Rosai-Dorfman disease（以下簡稱RDD）是一種罕見的良性疾病，主要引起淋巴結腫大，也可侵犯淋巴結以外的區域，淋巴結外的RDD以異時性的鼻腔和顱內腫塊為表現的病例未曾被報導過。我們提出此一病例為49歲女性患有鼻塞而後產生頭痛，CT顯示鼻腔內部腫塊但未造成骨骼破壞，MR影像則顯示鼻竇腔內腫塊有良好的顯影，病人在接受手術治療後病理組織顯示有emperipolesis(lymphophagocytosis)，且免疫染色呈現S-100陽性反應，一年後追蹤MR影像發現除了鼻腔之復發腫塊外，顱內也出現貼著硬腦膜之腫塊，且該腫塊在T2加權影像下有低訊號的區域，即使病人沒有淋巴結腫大，辨視這些影像特徵將有助於該疾病的診斷。

並列摘要

Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly involving lymph nodes. Extranodal RDD presenting as metachronous nasal and intracranial masses has not previously been described. Herein, we reported such a case of RDD in a 49 year-old female who presented with nasal obstruction and subsequent headache. Pre-enhanced sinus CT showed diffuse polypoid masses in the nasal cavity without bony destruction. MRI demonstrated a strongly enhanced, infiltrative lesion filling the sinonasal cavity. The patient underwent surgical resection, and histopathology showed emperipolesis (lymphophagocytosis) and immuno- histochemistry showed S-100 positivity. The follow-up MRI one year later showed sinonasal tumor recurrence. In addition, it revealed a newly developed intracranial dura-based mass that exhibited hypointense foci on T2-weighted imaging. Recognition of these imaging findings is helpful in suggesting the diagnosis of RDD even in the absence of lymphadenopathy.