A 57-year-old woman presented with bilateral upper eyelid swelling and excessive tearing for 1 month. Physical examination showed nontender enlarged lacrimal glands and a salmon-colored right-sided conjunctival mass (panel A). Routine laboratory workup was remarkable for a mild anemia (hemoglobin, 10.8 g/dL) and increased lactate dehydrogenase (296 U/L). Left lacrimal gland biopsy established a diagnosis of mantle cell lymphoma (MCL) with small- to medium-size neoplastic B cells (panel B; hematoxylin and eosin [H&E] stain, original magnification ×40) with a CD5⁺CD20⁺CD23^– and cyclin D1⁺ immunophenotype (panels C-D; original magnification ×40). Ki-67 was 30%. Whole-body positron emission tomography scans revealed involvement of the lacrimal glands, generalized lymphadenopathy, and extensive infiltration of the subcutaneous soft tissues of the torso (panels E-F). She achieved a complete remission after 6 cycles of bendamustine and rituximab.

Ocular adnexal non-Hodgkin lymphoma (NHL) represents less than 1% of all NHLs but constitutes the majority of orbital malignancies. Marginal zone lymphoma is the most common histologic subtype. MCL comprises a small minority of ocular adnexal lymphomas and is often associated with stage IV disease and bilateral ocular adnexal involvement. Lacrimal gland involvement is rare in MCL and, although the skin can be involved in MCL, the extent of subcutaneous tissue involvement was striking in our patient.