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.

BACKGROUND AND OBJECTIVES: Lymphoepithelioma-like carcinoma (LELC) is an undifferentiated epithelial tumor with a dense inflammatory infiltrate that resembles the lymphoepithelioma of the nasopharinx occurring in other sites. Primary LELC of the bladder (LELCB) was first reported by Zukerberg et al in 1991. The incidence of LELCB is 0.4%-1.3% of all bladder carcinomas. The mean age at diagnosis is 69 years. Of the patient population 69% are men. Herein we report on one more case of primary predominant LELCB and review all the English literature concerning this subject after performing a pooled analysis of the cases recorded in the English literature including the present one.
MATERIALS AND METHODS: The reports of 43 patients including the present case of primary LELCB from the English literature were collected from 1991 to 2002. Patients were evaluated for age, sex, primary and adjuvant treatments, clinical staging, follow-up and outcome, and disease related survival. The overall patient population was separated into 3 groups according to the LELCB classification of Amin.
RESULTS: The overall patient population included 31 males and 12 females. Average age was 68.4 years (range 52-84). LELCB histological subtypes resulted pure in 17 cases (40%), predominant in 16 (37%) and focal in 10 (23%). Mean follow-up was 37.7 months (range 0-216). Outcome resulted as follows: 26 patients (62%) did not show evidence of diasease, 11 (26%) died of disease, 1 (2%) was alive with metastases, and 4 (10%) died for causes unrelated to the primary disease. Survival rate related to specific disease resulted 71%. Mean follow-up was 48.1 in the first group (pure LELCB), 32 in the second (predominant LELCB), and 30.3 in the third one (focal LELCB). Patients with not evidence 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 the second, and 9 (90%) in the third one. Patients who died for disease not related to the primary tumor 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 in group 2. Survival rate related to specific disease 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 performed include both deep transurethral resection of the tumor (TUR-B) as well as partial or radical cystectomy, with or without adjuvant treatments including systemic chemotherapy and radiotherapy. The prognosis is favorable for patients presenting with the pure and predominant forms with a diploid DNA pattern and very poor for patients presenting with focal LELCB. Bladder salvage therapy by performing both TUR-B alone or combined with adjuvant systemic chemotherapy may be a reasonable option for patients with pure or predominant LELCB, while radical surgery with adjuvant systemic therapy may be indicated for focal muscle invasive LELCB.