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A benign lymphoepithelial lesion with malignant transformation developing into Sjögren's syndrome

SALIVARY gland enlargement may be an isolated finding or associated with a range of concomitant symptoms. When salivary gland .swelling is the only manifestation, with histologically a diffuse lymphoid infiltration and epimyoepithelial islands, it is termed benign lymphoepithelial lesion (BLL). When salivary and ocular symptoms are present without connective tissue disease it is called sicca syndrome. Sjogren's syndrome is the triad of keratoconjunctivitis sicca, xerostomia and a connective tissue disease. A case is presented in which the only manifestation of the disease was unilateral swelling of the parotid gland. The histology proved it to contain both BLL and a malignant lymphoma. This condition developed gradually into Sjogren's syndrome. Even though this complex of diseases (BLL, sicca syndrome and Sjogren's syndrome) is well known, such a development seems to be unusual and warrants description.