Papillary cystadenoma of the epididymis (PCE) is a rare benign epithelial tumor remarkable for its association with von Hippel-Lindau disease. A 12-year-old boy consulted for a progressive enlargement of the left testicle. At time of surgery, the whole epididymis was enlarged. Pathologic diagnosis was PCE with a focus on borderline malignancy. Scrotal left epididymectomy was performed. von Hippel-Lindau disease screening was negative. No relapse has been detected 2 years later. In case of atypical clinical examination of a hydrocele, unusual presentations such as PCE should be considered. The main differential diagnoses were adenomatoid tumor, nonpapillary cystadenoma, and metastatic clear cell renal carcinoma.
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