Lymphomas of the Orbit.
Gizem Tumer, Roger Turbin, Valerie A Fitzhugh, Paul D Langer, Neena M Mirani. University of Medicine and Dentistry of New Jersey- New Jersey Medical School, Newark
Background: Lymphoma, the commonest malignant tumor affecting the orbit, may arise primarily from the orbit, extend from contiguous structures, or develop as a metastasis of a systemic lymphoma. Primary lymphoma of the ocular adnexa represents 5-15% of all extranodal non-Hodgkin lymphomas, yet only 1% of all non-Hodgkin lymphomas. Current literature suggests marginal zone lymphoma to be the most commonly diagnosed non-Hodgkin lymphoma affecting the orbit. Herein we describe our experience with a large series of orbital lymphomas arising from or secondarily involving the orbit.
Design: Retrospective chart review of clinical and pathologic features of 32 biopsy proven orbital lymphomas evaluated in a single tertiary referral institution from 2001 to 2010. Diagnosis was based on histomorphology, immunohistochemistry, and cytogenetic analysis and classified according to the World Health Organization classification.
Results: Thirty-two patients ages 29-86 years (68.6, mean; 73 median) showed male to female ratio of 1.46:1. Presenting signs and symptoms included pain, proptosis, restrictive strabismus or diplopia, eyelid mass or swelling, “salmon patch” conjunctival lesion, ptosis, hypoglobus, optic neuropathy and vision loss. Anatomic locations of the lesions included the eyelid, canthus, orbit (intra and extraconal), lacrimal gland and conjunctiva. All 32 lymphomas were of the non-Hodgkin type and of B cell origin. Among all cases, 11 were mucosa associated lymphoid tissue (MALT) lymphomas, 9 were diffuse large B-cell lymphoma, 6 were mantle cell lymphoma, 3 were follicular lymphoma and, 3 were small lymphocytic lymphoma. Staging revealed the site of origin to be isolated orbital or orbital with extension to contiguous structures (11 cases), orbital with systemic disease found on initial staging (3 cases), orbital with subsequent dissemination (6 cases), secondary orbital lesions of known systemic disease (8 cases) and staging not known (4 cases).
Conclusions: Orbital lymphoma is a disease of the elderly and the vast majority are non-Hodgkin lymphomas of B-cell phenotype. While the diagnosis of these lesions can be challenging, morphology and ancillary studies can assist in determination of phenotype. MALT lymphoma is the most common type in our study which is consistent with several other large studies.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 282, Wednesday Afternoon