This case report documents a rare malignant transformation arising from unicentric Castleman disease (UCD) of the parotid gland—an entity typically known for its benign course. A 25-year-old male with previously excised UCD presented with a slowly progressive parotid swelling over four years, with cytology again suggesting recurrence. Definitive surgical management with total parotidectomy and modified radical neck dissection revealed an unexpected diagnosis of B-cell lymphoma on final histopathology.
This case underscores the importance of maintaining a high index of suspicion for malignant evolution even in histologically stable UCD, particularly when dealing with recurrent parotid masses. Early recognition and timely multidisciplinary referral remain pivotal for optimizing patient outcomes. To explore the full clinical insights and diagnostic nuances of this rare parotid…