Abstract
PURPOSE: To report an atypical case of cystic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of lacrimal gland associated with vision loss. OBSERVATIONS: An 89-year-old woman was presented with a rapidly progressing proptosis, lagophthalmos, and vision loss. Endophthalmitis was also present. Computed tomography scan images showed a hyperdense mass with hypodense cystic areas occupying the superolateral orbit, which displaced the globe antero-inferiorly with optic nerve compression and stretching. An erosion to the adjacent superior and lateral orbital walls was also demonstrated. Complete tumor excision was performed via upper transconjunctival orbitotomy concurrently with enucleation. The immunohistopathological diagnosis was MALT lymphoma. CONCLUSIONS AND IMPORTANCE: This case emphasizes the importance of considering lymphoma in the differential diagnosis of a cystic superolateral orbital mass.