Carcinoma Ex Pleomorphic Adenoma in Lacrimal Gland: A Rare Malignant Neoplasm

Author(s):

Renuka Verma1, Nidhi Kaushik1*, Monika Dhankher1, Niharika Taunk1, Sonia Chhabra1, Rajeev Sen1

Carcinoma ex pleomorphic adenoma (CXPA) is a rare malignancy which arises from a previously benign pleomorphic adenoma (PA). It has high aggressive behavior and poor prognosis. It is often difficult to diagnose because of similar clinical presentation to benign tumors and various other histological variants. Histological diagnosis is the gold standard for making the diagnosis. This tumor is rare in salivary gland and even rarer in lacrimal gland with only 0.3% of all orbital tumorous lesions. Patients with PA of the lacrimal gland clinically present as insidiously enlarging painless swelling of the orbit and if transformed to CXPA bulbar enlargement, displacement, and proptosis are the chief presentation