Lacrimal gland orbital lobe cysts associated with MALT lymphoma and primary Sjögren's syndrome. Orbit 2005 Dec;24(4):257-63
Date
12/16/2005Pubmed ID
16354635DOI
10.1080/01676830590922084Scopus ID
2-s2.0-29044440420 (requires institutional sign-in at Scopus site) 17 CitationsAbstract
PURPOSE: To describe, for the first time, the occurrence of a large cyst in the orbital lobe of the lacrimal gland in association with marginal zone B-cell MALT lymphoma in three female patients, two of whom also had primary Sjögren's syndrome.
METHODS: Retrospective case note review.
RESULTS: Three female patients, aged 35, 41 and 47 years, presented with progressive proptosis caused by an enlarging cyst within the orbital lobe of the lacrimal gland. Two of the patients had primary Sjögren's syndrome. Excision of the cyst and part of the lacrimal gland revealed marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type within the gland or wall of the cyst in all cases. The cysts were partially lined by ductular epithelium. One patient had systemic lymphoma, was treated with chemotherapy and remains in remission one year later; the other two had disease apparently confined to the lacrimal gland and are disease-free at 9 months and at three years after local irradiation, respectively.
CONCLUSION: To our knowledge, these are the first reported cases of cysts affecting the orbital lobe of the lacrimal gland in patients with B-cell MALT lymphoma of the gland. Although cystic changes have been described in other exocrine glands, lung and thymus in patients with Sjögren's syndrome, this association has also not been described in the lacrimal gland before.
Author List
Cassidy DT, McKelvie P, Harris GJ, Rose GE, McNab AAAuthor
Gerald J. Harris MD Professor in the Ophthalmology and Visual Sciences department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultCysts
Diagnosis, Differential
Female
Humans
Lacrimal Apparatus Diseases
Lymphoma, B-Cell, Marginal Zone
Magnetic Resonance Imaging
Middle Aged
Sjogren's Syndrome
Tomography, X-Ray Computed