Lacrimal gland carcinoma ex pleomorphic adenoma with chronic lymphocytic leukemia infiltration

Rita Couceiro,1 Cláudia Loureiro,1 Pedro Luís,2 Dolores López-Presa,2 Helena Proença,1 Ana Fonseca,1 Manuel Monteiro-Grillo11Department of Ophthalmology, Hospital de Santa Maria, Lisbon, Portugal; 2Department of Pathology, Hospital de Santa Maria, Lisbon, PortugalPurpose: To report a rare case of lacrimal gland carcinoma ex pleomorphic adenoma (Ca ex PA) with chronic B-cell lymphocytic leukemia (B-CLL) infiltration in a patient without a previous diagnosis of B-CLL.Patient and methods: We report a 66-year-old woman who presented with recent worsening of a long-standing right eye proptosis. Sequential orbital computed tomography imaging was performed over the course of 2 years, and biopsy specimens were analyzed.Results: Initial computed tomography scans revealed a lacrimal gland lesion with stable dimensions for more than 1 year and no malignancy features on incisional biopsy. Subsequently, lesion volume growth and bone erosion were documented on orbital computed tomography. Lateral orbitectomy and lacrimal gland resection were performed. Pathology and immunohistochemistry detected Ca ex PA with B-CLL infiltration.Conclusion: This case highlights the importance of persistent investigation of clinically suspicious orbital lesions. To our knowledge, this is the first description of a case of lacrimal gland Ca ex PA with B-CLL infiltration.Keywords: ex pleomorphic adenoma, chronic lymphocytic leukemia, lacrimal gland malignancy, proptosi