Primary lymphoepithelioma-like carcinoma of the ureter

Lymphoepithelioma-like carcinoma (LELC) is an uncommon tumor of the urinary tract. We present a case of LELC involving the ureter of a 71-year-old male patient with gross hematuria. On biopsy, the patient was diagnosed with invasive poorly differentiated carcinoma, with a final diagnosis of LELC on subsequent nephroureterectomy. On resection, the neoplasm was solitary and consisted of undifferentiated neoplastic cells demonstrating a syncytial growth pattern, vesicular nuclei, prominent nucleoli, and an admixed polyclonal lymphoid infiltrate. Immunohistochemistry showed that the tumor cells were positive for Cam5.2 and CK7; focally positive for cytokeratinAE1/3, EMA, CK20, and p63; and negative for CK903. Epstein-Barr virus in situ hybridization was negative. No disease progression was noted at 5-month follow-up. Only 6 previous cases of LELC involving the ureter have been reported in the literature. We document an additional example of this uncommon entity and present a comprehensive review of LELC involving the ureter.