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Journal of Clinical Oncology, Vol 24, No 6 (February 20), 2006: pp. 1011-1013 © 2006 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.01.1544
CASE 3. Red Eye in Sjögren's Syndrome Due to Underlying Mucosa-Associated Lymphoid Tissue LymphomaMedizinische Onkologie/Hämatologie, Departement Innere Medizin, and Institut für Radiologie, Kantonsspital, St Gallen, Switzerland A 68-year-old woman presented to her primary care physician complaining of itching in her left eye. It was interpreted as a symptom of her Sjögren's syndrome, diagnosed 15 years ago, and treated symptomatically. Half a year later, she was suffering from pain and irritation and in addition the eye became redder. After a few months, she was admitted to our institution for further investigation and treatment. Her previous medical history was significant for symptoms from the Sjögren's syndrome. She complained of constant thirst and she was waking up three to four times each night because of xerostomia. She was diagnosed with chronic gastritis, therefore a vagotomy was performed in 1987. In addition, she occasionally suffered from a Raynaud's phenomenon on the right index finger and she was taking levothyroxin because of hypothyroidism; both diseases are linked with Sjögren's syndrome. She did not show any presence of autoantibodies to Ro/SSA and La/SSB, but a biopsy of her lips in 1990 showed a lymphoplasmocytoid inflammation consistent with Sjögren's syndrome. Her daughter was disabled due to psoriatic arthritis. Our patient underwent an ophthalmologic examination, which was suspicious for a conjunctival tumor (Figs 1 and 2). An orbital magnetic resonance imaging scan revealed enlargement of the left lateral rectus muscle with an ill-defined border and anterior extension beyond the orbital septum. Mild enhancement was noted after intravenous gadolinium injection, and orbital manifestation of a lymphoma was suggested (Fig 3). The biopsy from this tumor showed an extranodal marginal zone mucosa-associated lymphoid tissue (MALT) –type B-lymphoma of the conjunctiva, monoclonality with rearrangement was shown. Further investigations such as computed tomography scan, upper endoscopy, and bone marrow trephine biopsy did not show any other tumor localization. The final diagnosis was an extranodal marginal zone B-cell lymphoma of MALT type of the right conjunctiva stage lAE. She was treated with radiotherapy only, 30 Gy in 15 fractions with electrons of 6 MEV energy. Two years later, she has still not relapsed.
"Red eye" can be caused by very different reasons, but when an underlying diagnosis of Sjögren's syndrome is known, every clinician will first interpret the red eye as a symptom of keratoconjunctivis. Parameters leading to another cause could include that it was unilateral only, chronic, and therapy-refractory. MALT-type lymphoma in the conjunctiva is well described in patients with Sjögren's syndrome.1,2 MALT is usually absent from normal conjunctiva.3 It has to be acquired before MALT-type lymphoma can develop. Acquired MALTs are associated with inflammatory conditions, like autoimmune diseases (Sjögren's syndrome, Hashimoto's thyroiditis) or infectious diseases (Helicobacter pylori gastritis, chronic borreliosis in the skin, Chlamydia psittaci).4,5 Lymphoma deriving from MALT seems to behave less aggressively than other types of extranodal lymphoma.6 This tumor tends to remain localized to mucosal surfaces rather than to disseminate. MALT lymphomas of the conjunctiva are often bilateral.7 But patients with bilateral involvement have similar prognoses than those with unilateral lesions, because those tumor cells seem to be unable to survive without conjunctival tissue. Therefore it is justified to also classify bilateral MALT lymphoma of the conjunctiva as stage lE. There are no randomized trials published comparing different treatment approaches for this rare tumor entity. However, there are a lot of case reports and series showing that carefully planned radiotherapy with lens shielding is effective and safe with local control of 90% to 100% of all patients.8,9 The contralateral eye remains a potential site of relapse and should be carefully monitored. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Hsi ED, Zukerberg LR, Schnitzer B, et al: Development of extrasalivary gland lymphoma in myoepithelial sialadenitis. Mod Pathol 8:817-824, 1995[Medline] 2. Hyiek E, Smith WJ, Isaacson PG: Primary B-cell lymphoma of salivary glands and its relationship to myoepithelial sialadenitis. Hum Pathol 19:766-776, 1988[Medline] 3. Wotherspoon AC, Hardman-Lea S, Isaacson PG: Mucosa-associated lymphoid tissue (MALT) in the human conjunctiva. J Pathol 174:33-37, 1994[CrossRef][Medline] 4. Isaacson PG: Extranodal lymphomas: The MALT concept. Verh Dtsch Ges Pathol 76:14-23, 1992[Medline] 5. Ferreri A, Guidoboni M, Ponzoni M, et al: Evidence for an association between chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst 96:586-594, 2004 6. Cogliatti SB, Schmid U, Schumacher U, et al: Primary B-cell gastric lymphoma: A clinicopathological study of 145 patients. Gastroenterology 101:1159-1170, 1991[Medline] 7. Hardman-Lea S, Kerr-Muir M, Wotherspoon AC, et al: Mucosal-associated lymphoid tissue lymphoma of the conjunctiva. Arch Ophthalmol 112:1207-1212, 1994 8. Tsang RW, Gospodarowicz MK, Pintilie M, et al: Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol 21:4157-4164, 2003 9. Xicoy B, Ribera JM, Arellano A, et al: Effectiveness of local radiotherapy in primary extranodal marginal zone B-cell lymphoma of MALT or MALT lymphoma of conjunctiva: Study of four cases. Leuk Lymphoma 43:1975-1977, 2002[Medline]
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Copyright © 2006 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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