Lacrimal gland extranodal marginal zone B-cell lymphoma in the presence of amyloidosis.

Amyloidosis is a protein metabolism disorder characterised by extracellular deposition of insoluble amorphous hyaline material. Orbital and ocular amyloid lesions account for only 4% of localised disease affecting the head and neck. Ocular adnexal lymphoma accounts for 1-2% of lymphoma, with lacrimal gland lymphomas being relatively uncommon. The most common form affecting the orbit is extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT lymphoma). We report an extremely rare case of co-existent EMZL and amyloidosis of the lacrimal gland. Initial biopsy of the right lacrimal gland confirmed an EMZL with amyloid deposit, and a course of radiotherapy treatment was given. Recurrent lacrimal gland swelling developed within a year. Subsequent biopsy identified amyloidosis with scanty lymphoid tissue. To our knowledge, this is the first reported case of localised lacrimal gland amyloidosis of uncertain type with previous EMZL; the association described in this case report is not yet fully understood.