Primary lymphoepithelioma-like carcinoma of the urinary bladder: Report of one case with review and update of the literature after a pooled analysis of 43 patients

Abstract

Background and objectives:Lymphoepithelioma-like carcinoma (LELC) is anundifferentiated epithelial tumor with a denseinflammatory infiltrate that resembles thelymphoepithelioma of the nasopharinx occurringin other sites. Primary LELC of the bladder(LELCB) was first reported by Zukerberg et alin 1991. The incidence of LELCB is 0.4%–1.3% of all bladder carcinomas. The mean ageat diagnosis is 69 years. Of the patientpopulation 69% are men. Herein we report onone more case of primary predominant LELCB andreview all the English literature concerningthis subject after performing a pooled analysisof the cases recorded in the Eglish literatureincluding the present one.Materials and methods: The reports of 43patients including the present case of primaryLELCB from the English literature werecollected from 1991 to 2002. Patients wereevaluated for age, sex, primary and adjuvanttreatments, clinical staging, follow-up andoutcome, and disease related survival. Theoverall patient population was separated into 3groups according to the LELCB classification ofAmin. Results: The overall patientpopulation included 31 males and 12 females.Average age was 68.4 years (range 52–84). LELCBhistological subtypes resulted pure in 17cases(40%), predominant in 16 (37%) and focal in10 (23%). Mean follow-up was 37.7 months(range 0–216). Outcome resulted as follows: 26patients (62%) did not show evidence ofdiasease (62%), 11 (26%) died of disease, 1(2%) was alive with metastases, and 4 (10%)died for causes unrelated to the primarydisease. Survival rate related to specificdisease resulted 71%. Mean follow-up was 48.1in the first group (pure LELCB), 32 in thesecond (predominant LELCB), and 30.3 in thethird one (focal LELCB). Patients with notevidence of disease were 13 (81%) in group 1,13 (82%) in group 2, and 0 in group 3.Patients who died of their disease resulted 1(6%) in the first group, 1 (6%) in thesecond, and 9 (90%) in the third one. Patientswho died for disease not related to the primarytumor were 2 (13%) in the first group, 1 (6%)in the second, and 1 (10%) in the third one.One patient (6%) was alive with metastases ingroup 2. Survival rate related to specificdisease resulted 93% in the first group, 93%in the second one, and 0% in the third one.Conclusions:To date, there are no clear guide lines for the treatment of LELCB. Treatments performedinclude both deep transurethral resection ofthe tumor (TUR-B) as well as partial or radicalcystectomy, with or without adjuvant treatmentsincluding systemic chemotherapy andradiotherapy. The prognosis is favorable forpatients presenting with the pure andpredominant forms with a diploid DNA patternand very poor for patients presenting withfocal LELCB. Bladder salvage therapy byperforming both TUR-B alone or combined withadjuvant systemic chemotherapy may be areasonable option for patients with pure orpredominant LELCB, while radical surgery withadjuvant systemic therapy may be indicated forfocal muscle invasive LELCB.