Background: The benign lymphoepithelial lesion of the salivary/lacrimal glands is generally regarded as a lymphoproliferative disorder that may be associated with Sjögren syndrome. Although lymphomatous transformation in patients with Sjögren syndrome is well documented, few reports have appeared describing similar findings in patients with a benign lymphoepithelial lesion.
Methods: The authors report the unusual occurrence of malignant lymphoma involving the ocular adnexa in two patients with a histopathologically documented benign lymphoepithelial lesion of the parotid glands.
Results: Both patients developed B-cell lymphomas. The eyelid tumor in case 1 expressed IgM/kappa cell surface markers by flow cytometry, and the orbital/conjunctival masses in case 2 showed neoplastic nodules of B cells that were only immunoreactive to lambda light chains (avidin-biotin complex method).
Conclusion: To the authors' knowledge, this is the first report of ocular adnexal lymphoma arising in patients with a benign lymphoepithelial lesion of the parotid glands. This report establishes that ocular adnexal lymphoma may arise in a patient with a preexisting benign lymphoepithelial lesion.