Dr. SHAIFALI CHAHAR

Dr. FAIROOZ PUTHIYAPURAYIL MANJANDAVIDA

Abstract

Ocular adnexal lymphoma (OAL) represents the malignant spectrum of lymphoproliferative disease. It comprises of 6-8% of orbital tumors and 10-15% of adnexal lesions, majority being low-grade Non-Hodgkins lymphoma (NHL). Bilaterality is reported only in 10-20% of cases. It is the most common malignant tumor encountered in the orbit, involvement of extraocular muscles is very rare accounting only in 0.17% of all orbital lymphomas. Extraocular muscles can be affected by various etiologies whose clinico-radiological features can overlap making it a challenging diagnosis. We describe a case series of extra-ocular muscle lymphoma in four eyes of three patients involving lateral rectus (n=2), inferior rectus (n=2) medial rectus (n=1), and inferior oblique (n=1). Incision biopsy was confirmed as extra-nodal, marginal zone, B-cell NHL. No systemic involvement was present. Treatment included ultra-low dose 2/2 Gy radiotherapy, and intraorbital injection rituximab showing excellent resolution.