Sjogren's syndrome and bilateral MALT-related lymphomas of the parotid gland

Sjogren's syndrome is an important autoimmune disease in the head and neck. Patients have an increased arrival risk of up to 6% per year for developin g B-cell lymphomas, including mucosa-associated lymphoid tissue (MALT) lymp homas. The following case report shows this relation and the difficulty of differentiating clinically recurrent swelling of the parotid gland in Sjogr en's syndrome from malignant lymphoma. A 64-year-old woman had a 2-year his tory of indolent, recurrent swelling of both parotid glands. Blood examinat ion showed elevated ESR and a hypergammaglobulinemia. Immunosupressive ther apy produced no improvement. Two years after the diagnosis of Sjogren's syn drome, swelling of the left parotid gland persisted. Superficial parotidect omy of the left side was performed and histopathological examination reveal ed a MALT-related lymphoma. Subsequent parotidectomy of the right side also showed infiltration of the gland by a MALT lymphoma. Postoperative radiati on therapy was given. During the follow-up period no recurrence or systemic disease was detected. Patients with Sjogren's syndrome should be examined regularly by the otolaryngologist. Ifa lymphoma cannot be ruled out,open bi opsy must be considered for histological diagnosis. Prognostic factors for developing a lymphoma are possibly a high ESR and hypergammaglobulinemia. F urther prognostic factors have to be evaluated.