TY - JOUR
T1 - Extranodal rosai-dorfman disease of the orbit
T2 - Clinical features of 8 cases
AU - Tan, Jeffrey J.
AU - Narang, Sherry
AU - Purewal, Baljeet
AU - Langer, Paul D.
AU - Blaydon, Sean
AU - Schwarcz, Robert M.
AU - McCormick, Steven A.
AU - Kim, Joon Y.
AU - Walrath, Joseph D.
AU - Burt, Benjamin O.
AU - Shinder, Roman
N1 - Publisher Copyright:
© 2016 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
PY - 2016
Y1 - 2016
N2 - Purpose:To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. Methods: Multicenter retrospective case series. Results: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confrmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. Conclusions: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.
AB - Purpose:To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. Methods: Multicenter retrospective case series. Results: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confrmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. Conclusions: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.
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U2 - 10.1097/IOP.0000000000000589
DO - 10.1097/IOP.0000000000000589
M3 - Article
C2 - 26561955
AN - SCOPUS:84946887894
SN - 0740-9303
VL - 32
SP - 458
EP - 461
JO - Ophthalmic Plastic and Reconstructive Surgery
JF - Ophthalmic Plastic and Reconstructive Surgery
IS - 6
ER -