Rosai dorfman disease of the orbit
<p>Abstract</p> <p>Objective</p> <p>To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.</p> <p>Design</p> <p>Non-comparative case series.</p> <p>Results</p> <p>Rosa...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2008-06-01
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| Series: | Journal of Hematology & Oncology |
| Online Access: | http://www.jhoonline.org/content/1/1/7 |
| _version_ | 1819115750125731840 |
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| author | Honavar Santosh G Naik Milind N Vemuganti Geeta K |
| author_facet | Honavar Santosh G Naik Milind N Vemuganti Geeta K |
| author_sort | Honavar Santosh G |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Objective</p> <p>To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.</p> <p>Design</p> <p>Non-comparative case series.</p> <p>Results</p> <p>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.</p> <p>Conclusion</p> <p>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.</p> |
| first_indexed | 2024-12-22T05:06:09Z |
| format | Article |
| id | doaj.art-efb5ef7ae0444c40a8f4ea972bc10bb4 |
| institution | Directory Open Access Journal |
| issn | 1756-8722 |
| language | English |
| last_indexed | 2024-12-22T05:06:09Z |
| publishDate | 2008-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Hematology & Oncology |
| spelling | doaj.art-efb5ef7ae0444c40a8f4ea972bc10bb42022-12-21T18:38:07ZengBMCJournal of Hematology & Oncology1756-87222008-06-0111710.1186/1756-8722-1-7Rosai dorfman disease of the orbitHonavar Santosh GNaik Milind NVemuganti Geeta K<p>Abstract</p> <p>Objective</p> <p>To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.</p> <p>Design</p> <p>Non-comparative case series.</p> <p>Results</p> <p>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.</p> <p>Conclusion</p> <p>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.</p>http://www.jhoonline.org/content/1/1/7 |
| spellingShingle | Honavar Santosh G Naik Milind N Vemuganti Geeta K Rosai dorfman disease of the orbit Journal of Hematology & Oncology |
| title | Rosai dorfman disease of the orbit |
| title_full | Rosai dorfman disease of the orbit |
| title_fullStr | Rosai dorfman disease of the orbit |
| title_full_unstemmed | Rosai dorfman disease of the orbit |
| title_short | Rosai dorfman disease of the orbit |
| title_sort | rosai dorfman disease of the orbit |
| url | http://www.jhoonline.org/content/1/1/7 |
| work_keys_str_mv | AT honavarsantoshg rosaidorfmandiseaseoftheorbit AT naikmilindn rosaidorfmandiseaseoftheorbit AT vemugantigeetak rosaidorfmandiseaseoftheorbit |