Abstract
ObjectiveTo report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.DesignNon-comparative case series.ResultsRosai-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.ConclusionRosai-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.
Highlights
Rosai-Dorfman disease, known as sinus histiocytosis with massive lymphadenopathy is a rare disorder characterized by nonmalignant proliferation of distinctive histiocytes within lymph node sinuses and other extranodal sites
Clinical Profile Of the 7537 ocular specimens received during the study period, seven patients were diagnosed as Rosai-Dorfman disease, constituting 0.09 % of all ocular specimens and 2.3% of orbital lesions (7 of 300)
In 1969, two pathologists Juan Rosai and Ronald Dorfman reported a distinct histiocytic disorder in young black males presenting with bilateral, painless, massive cervical lymphadenopathy with a protracted clinical course, and in most instances associated with fever, anemia, neutrophilia, elevated erythrocyte sedimentation rate, and polyclonal gammopathy[1]
Summary
Rosai-Dorfman disease, known as sinus histiocytosis with massive lymphadenopathy is a rare disorder characterized by nonmalignant proliferation of distinctive histiocytes within lymph node sinuses and other extranodal sites. It is a self-limiting disorder of unknown etiology that occurs worldwide in children and young adults [1,2,3,4,5]. Orbital mass in Rosai-Dorman disease could mimic lymphoma, lacrimal gland tumors, and other histiocytic tumors. We report the clinical manifestations, management, and outcome of a series of seven histopathologically proven cases of Rosai-Dorfman disease of the orbit
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