Lymphoepithelioma-like carcinoma of the urinary tract: a clinicopathological study of 30 pure and mixed cases

Abstract

We studied 28 cases of lymphoepithelioma-like carcinoma of the bladder, one case in the renal pelvis, and one in the urethra. The mean age of the patients was 67.6 years with 21 (70%) males. Seventeen cases (56.7%) were pure with the remaining mixed with other patterns of carcinoma, including invasive urothelial carcinoma (n ¼ 10), invasive adenocarcinoma (n ¼ 3), and squamous cell carcinoma (n ¼ 2). The surface demonstrated carcinoma in situ (CIS) in six cases, noninvasive high-grade papillary urothelial carcinoma in three cases, and in situ adenocarcinoma in one case. In 19/30 (66%) cases, there was a heavy lymphocytic infiltrate and in the remaining 11/30 (34%) cases a mixed inflammatory infiltrate. None of the 26 cases labeled for EBV-encoded RNA by in situ hybridization. Tumor stages at presentation were: seven cases T1 (23%); 14 cases T2 (47%); seven cases T3 (23%); and two cases T4 (7%). Treatment consisted of radical cystectomy in 13/30 cases (43%); partial cystectomy in 4/30 cases (13%); nephrectomy in one case (3%), and transurethral resection often followed by radiation or chemotherapy in 12/30 (40%) cases. The mean follow up for patients without progression was 31 months. Eight of 27 cases with follow-up (30%) cases had tumor recurrence, with seven patients having metastases. In cases treated with cystectomy, the 5-year actuarial recurrence-free risk was 59% (62 and 57%, for pure and mixed cases, respectively). Lymphoepithelioma-like carcinoma, whether in pure or mixed form, has a similar prognosis to ordinary urothelial carcinoma when treated by cystectomy. Of the three pure cases treated by chemotherapy, two were free of disease at 4 and 65 months and the third had recurrent disease at 17 months. Given the association of lymphoepithelioma-like carcinoma with urothelial carcinoma in 47% of our cases and its propensity for multifocality, partial cystectomy would typically be ill advised for lymphoepithelioma-like carcinoma.

Lymphoepithelioma-like carcinoma of the urinary bladder: report of a rare case

“…Since then there have been only a few relatively small studies on LELC of urinary bladder 2–8. The largest study was reported by Tamas et al8 who described 17 pure 13 mixed LELC. Prior to that Lopez-Beltran et al5 reported 3 pure, 6 predominant and 4 focal LELCs of the urinary bladder.…”

“…Epstein-Barr virus (EBV) is frequently associated with lymphoepithelioma of the nasopharynx and LELC arising in the lung, stomach, thymus and salivary gland 1289. However, such an association has not been documented for LELC of the urinary bladder.…”

“…However, such an association has not been documented for LELC of the urinary bladder. Hybridization with EBV-encoded RNA (EBER) has been reported to be negative 589. It has been suggested that abnormalities of p53 regulation might be crucial in the pathogenesis of LELC of the urinary bladder 10.…”

“…Most patients present with painless hematuria and are stage T2-T3 at diagnosis 568. Histological features of LELC closely resemble lymphoepithelioma of the nasopharynx, with the tumor growing in nests, sheets or cords of large undifferentiated malignant epithelial cells within a dense inflammatory background, comprising mature lymphocytes, plasma cells, and rarely neutrophils and eosinophils.…”

“…Amin et al3 categorized the LELC of bladder into three subgroups as pure, predominant (more than 50% lymphoepithelial component) and focal (less than 50% lymphoepithelial component). Invasive urothelial carcinoma usually accompanies predominant or focal LELC 358. Though uncommon, the tumor may have accompanying adenocarcinoma and squamous cell carcinoma as well 58.…”