Lymphoepithelioma-like, a variant of urothelial carcinoma of the urinary bladder: a case report and systematic review for optimal treatment modality for disease-free survival

Abstract

BackgroundLymphoepithelioma-like carcinoma (LELC) is a rare high-grade carcinoma that resembles nasopharyngeal lymphoepithelioma and can occur throughout the body. First reported in 1991, bladder LELC has an incidence of about 1% of all bladder carcinomas. Due to its rare occurrence, prognoses and ideal treatment guidelines have not been clearly defined.MethodsA PubMed search was performed using two terms, “lymphoepithelioma-like carcinoma” and “bladder.” Review articles, articles in foreign languages, expression studies, and studies not performed in the bladder were excluded. We report a case of LELC of the bladder including treatment and outcome and performed a systematic review of all 36 available English literatures from 1991 to 2016 including the present case to identify factors affecting disease-free survival.ResultsOne hundred forty cases of bladder LELC were analyzed. The mean age of the patients was 70.1 years ranging from 43 to 90 years with 72% males and 28% females. Pure LELC occurs most often at 46% followed by mixed LELC 28% and predominant LELC 26%. EBV testing was negative in all cases tested. Mean follow-up length for all cases was 33.8 months with no evidence of disease in 62.2%, while 11.1% died of disease, 10.4% alive with metastasis, and 8.2% died without disease. 5.0% of cases had recurrence at an average of 31.3 months. Prognosis is significantly favorable for patients presenting with pure or predominant forms of LELC compared to mixed type (p < 0.0001). The treatment significantly associated with the highest disease mortality and lowest disease-free survival was TURBT alone when compared to any multi-modality treatment (p < 0.01).ConclusionWe conclude that the best treatment modality associated with the highest disease-free survival is multi-modal treatment including radical cystectomy.

Lymphoepithelioma-Like Carcinoma of the Bladder: A Case Report of a Rare and Particular Variant of Urothelial Carcinoma

“…Carcinomas with similar histological features arising outside the nasopharynx have been called lymphoepithelioma-like carcinomas and have been reported in other organs including the prostate, breast, uterine cervix, skin, lung, trachea, thymus, stomach and salivary glands [ 6 – 8 ].…”

“…In this review the chief complaint was gross hematuria constantly present. The stage at diagnosis of those tumors was in 87% of cases T2–T3 stage [ 8 ].…”

Histologic Variants of Urothelial Carcinoma: Morphology, Molecular Features and Clinical Implications

“…The lymphoepithelioma-like carcinoma (LELC) variant is another rare variant of bladder cancer which resembles lymphoepithelioma of the nasopharynx, a tumor defined by both a prominent lymphoid infiltrate and ubiquitous Epstein-Barr Virus (EBV) positivity [43]. However, unlike lymphoepithelioma of the nasopharynx, LELC are universally EBV negative and designated as lymphoepithelioma-like [44].…”

Lymphoepithelioma like Carcinoma of the Bladder – “A Case” Revisited

“…In the urinary tract, they are typically, but uncommonly seen in the urinary bladder, although occasional cases have been reported in the renal pelvis, ureter and urethra [1,2].…”