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Malignant Lymphoproliferative Disorders Extending into the Orbit from the Paranasal Sinuses 

Authors: Alan Pelega; Manraj K. S. Heranb; Valerie A. Whitec; Wilma Y. Changd; Jack Rootmane
Affiliations:   a Ophthalmology and Visual Sciences, University of British Columbia and the Vancouver General Hospital, Vancouver, British Columbia, Canada
b Radiology, University of British Columbia and the Vancouver General Hospital, Vancouver, British Columbia, Canada
c Ophthalmology and Visual Sciences, and Pathology and Laboratory Medicine, University of British Columbia and the Vancouver General Hospital, Vancouver, British Columbia, Canada
d Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, British Columbia, Canada
e Pathology and Laboratory Medicine, University of British Columbia and the Vancouver General Hospital, Vancouver, British Columbia, Canada
DOI: 10.1080/01676830802656869
Publication Frequency: 6 issues per year
Published in: journal Orbit, Volume 28, Issue 2 & 3 April 2009 , pages 80 - 87
Subject: Ophthalmology;
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Abstract

Background/Aims: To describe the clinical features, imaging, histologic spectrum, treatment and prognosis of patients with malignant orbital lymphoproliferative disorders extending from the paranasal sinuses. Methods: Patients were culled from the University of British Columbia Orbit Clinic (1977-2004) and their clinical charts, imaging and pathology specimens reviewed. Results: The study included 6 patients with non-Hodgkin's lymphoma (NHL) and 5 multiple myeloma (MM). Orbital symptoms at presentation were periorbital swelling, diplopia, proptosis and pain. Major clinical findings were globe displacement, abnormal ocular movements, periorbital fullness, fifth nerve hypoesthesia and inflammatory signs. Imaging showed a soft tissue mass in one or more sinuses invading the orbit. Three NHL patients were still alive at last follow-up with one patient lost to follow-up. Two NHL patients and all MM died. Conclusion: Presenting symptoms of malignant orbital lymphoproliferative disorders extending from the paranasal sinuses include mass effect occasionally with infiltrative and inflammatory characteristics accompanied by symptoms of sinus disease. On imaging, a soft tissue mass in the sinuses and orbit with bone destruction is commonly seen. Biopsy is essential for conclusive diagnosis. Treatment should be initiated promptly as local symptoms are frequently relieved and survival is possible, especially in cases of NHL.
Keywords: secondary orbital lymphomas; paranasal sinus lymphoma
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