Ophthalmic Pathology Service, Hyderbad Eye Research Centre, L V Prasad Eye Institute, Hyderbad, India.
J Hematol Oncol. 2008 Jun 28;1:7. doi: 10.1186/1756-8722-1-7.
To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.
Non-comparative case series.
Rosai-Dorfman disease of the orbit constituted 0.09% of all ocular specimens received at our Institute, presenting with a firm rubbery mass causing proptosis; bilateral in 4 (57%) cases. The median age at presentation was 13 years (range 5-65); median duration of symptoms was 6 (range 3-15) years. Lymphadenopathy was noted in 4 (57%); extranodal involvement in 3 (43%). After biopsy, 3 cases were treated with systemic corticosteroids, 2 cases developed local recurrence that responded to systemic corticosteroid therapy. Polymorphous population of lymphocytes, plasma cells, and characteristic S-100-positive histiocytes showing emperipolesis were pathognomonic histologic features.
Rosai-Dorfman disease of the orbit, although rare, should be considered in young individuals with chronic proptosis with rubbery masses. Excision and corticosteroid therapy provide a favorable outcome.
报告眼眶 Rosai-Dorfman 病的临床病理特征、治疗方法和预后。
非对照病例系列。
眼眶 Rosai-Dorfman 病占本研究所接收的所有眼部标本的 0.09%，表现为引起眼球突出的坚实橡胶样肿块；4 例（57%）为双侧病变。发病时的中位年龄为 13 岁（范围 5-65 岁）；中位症状持续时间为 6 年（范围 3-15 年）。4 例（57%）有淋巴结病；3 例（43%）有结外累及。活检后，3 例接受全身皮质类固醇治疗，2 例局部复发，对全身皮质类固醇治疗有反应。多形性淋巴细胞、浆细胞和特征性 S-100 阳性组织细胞的出现是其具有诊断意义的组织学特征。
尽管眼眶 Rosai-Dorfman 病罕见，但对于有慢性眼球突出和橡胶样肿块的年轻患者，应考虑该病的可能。切除和皮质类固醇治疗可获得良好的预后。
