Lacrimal gland extranodal marginal zone B-cell lymphoma in the presence of amyloidosis. (4th May 2022)

• Record Type: Journal Article
• Authors: Chean, Chung Shen; Sovani, Vishakha; Boden, Ali; Knapp, Christopher
• Is Part Of: Orbit. Volume 41:Number 3(2022)
• Page Start: 350
• Page End: 353
• Publication Date: 2022-05-04
• Subjects: 'amyloidosis' -- 'lacrimal gland' -- 'lymphoma'; Eye-sockets -- Surgery -- Periodicals; Eye-sockets -- Diseases -- Periodicals; Orbit -- surgery -- Periodicals; Orbital Diseases -- Periodicals; 617.7
• DOI: 10.1080/01676830.2020.1852578
• Languages: English
• ISSNs: 0167-6830

Abstract

ABSTRACT: 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.