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Lymphoma of the parotid gland in Sjogren's syndrome.

Sjogren's syndrome is a chronic autoimmune disorder that manifests as a lymphocytic infiltration of the exocrine glands, particularly the salivary and lacrimal glands. The hallmark of Sjogren's syndrome is the sicca complex of xerostomia and keratoconjunctivitis. (1) Its association with lymphoma is well documented; this severe complication of primary Sjogren's syndrome occurs in 5 to 10% of patients who are followed for more than 10 years. (2, 3) Lymphomas that develop in patients with Sjogren's syndrome are classified as mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas are sub-classified as either low-grade or high-grade. (4)

We evaluated a 60-year-old woman who had experienced an insidious and slowly progressive development of dry eyes and dry mouth. She exhibited no evidence of underlying rheumatoid arthritis. Clinically, a soft-tissue mass was identified in the parotid area. Computed tomography (CT) at the level of the parotid glands showed a nodular appearance of both parotid glands, indicating dilation of the acini (figure, A). Also noted was a low-density soft-tissue mass in the right parotid gland that measured approximately 3 cm at its greater dimension (figure, B); this lesion was histologically proven to be a lymphoma.

[FIGURE OMITTED]

The pathophysiology of lymphoma in Sjogren's syndrome remains unknown. To date, there is no argument favoring a viral infection or deregulation of a unique oncogene or antioncogene. (2) CT and magnetic resonance imaging can demonstrate findings consistent with Sjogren's syndrome. In such cases, the parotid glands have a punctate or nodular appearance that represents globular collections. These findings, however, are nonspecific; they are also seen in chronic sialadenitis and granulomatous diseases. (4)

References

(1.) Talal N. Sjogren's syndrome In: Schumacher HR, Jr., ed. Primer on the Rheumatic Diseases. 9th ed. Atlanta: Arthritis Foundation, 1988:136-8.

(2.) Mariette X. [Gougerot-Sjogren syndrome. Risk of lymphoma]. Presse Med 1999;28:1214-18.

(3.) Voulgarelis M, Moutsopoulos HM. Lymphoproliferation in autoimmunity and Siogren's syndrome. Curr Rheumatol Rep 2003;5:317-23.

(4.) Som PM, Brandwein MS. Salivary glands: Anatomy and pathology. In: Som PM, Curtin HD, eds. Head and Neck Imaging. 4th ed., vol. 2, St. Louis: Mosby, 2003:2005-133.

>From the Department of Radiology, Louisiana State University Health Sciences Center, New Orleans.
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Title Annotation:Imaging Clinic
Author:Ramirez, Guadalupe
Publication:Ear, Nose and Throat Journal
Geographic Code:1USA
Date:Mar 1, 2004
Words:358
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