Hsu Hung-Yuan, Chen Zoe Tzu-Yi, Tsai Ching-Yao, Liou Shiow-Wen, Woung Lin-Chung
Department of Ophthalmology, Taipei City Hospital, Taipei, Taiwan.
Semin Ophthalmol. 2013 Jul;28(4):253-5. doi: 10.3109/08820538.2013.788675. Epub 2013 Apr 29.
Rosai-Dorfman disease is a rare, benign, idiopathic histocytic proliferative disorder that typically presents in young adults with painless cervical lymphadenopathy. Here we report an atypical case of Rosai-Dorfman disease involving orbit tissue and lacrimal gland, unilaterally.
A 69-year-old Asian women developed a painless palpable mass with local edema over the left upper eyelid over several months. Computed tomography (CT) showed an orbital mass with homogenous soft tissue density over the left lacrimal gland and superior orbital area. The patient underwent complete excision of the orbital tumor.
The histopathology revealed diffuse and nodular infiltrations of S-100 positive histiocytes, plasma cells and lymphocytes. Emperipolesis (lymphocytophagocytosis) was also noted. These findings were consistent with Rosai-Dorfman disease. Chest CT revealed hilar lymphadenopathy. Three months after excision of the orbital mass, the patient developed lymphadenopathy in the extremities that resolved spontaneously over a few weeks. There were no complications or recurrence without systemic treatment after the complete excision.
Orbital Rosai-Dorfman disease is a rare disorder, especially in Asia. Though there is no consensus on therapeutic choices, including corticosteroids, chemotherapy, radiation therapy, and surgical excision, the complete surgical excision performed in this case was without complication and had a favorable outcome.
罗萨伊-多夫曼病是一种罕见的、良性的、特发性组织细胞增生性疾病，通常发生于年轻成人，表现为无痛性颈部淋巴结病。在此，我们报告一例非典型的罗萨伊-多夫曼病，单侧累及眼眶组织和泪腺。
一名69岁的亚洲女性在数月内左上眼睑出现一个无痛性可触及肿块，并伴有局部水肿。计算机断层扫描（CT）显示左侧泪腺和眶上区域有一个均匀软组织密度的眼眶肿块。患者接受了眼眶肿瘤的完整切除。
组织病理学显示S-100阳性组织细胞、浆细胞和淋巴细胞的弥漫性和结节性浸润。还发现了嗜血细胞现象（淋巴细胞吞噬作用）。这些发现与罗萨伊-多夫曼病一致。胸部CT显示肺门淋巴结病。眼眶肿块切除三个月后，患者四肢出现淋巴结病，数周内自行消退。完整切除后未经全身治疗，无并发症或复发。
眼眶罗萨伊-多夫曼病是一种罕见疾病，在亚洲尤其如此。尽管对于包括皮质类固醇、化疗、放疗和手术切除在内的治疗选择尚无共识，但本病例中进行的完整手术切除无并发症且预后良好。
