Bladder Lymphoepithelioma-Like Carcinoma: Case Report and Literature Review

Abstract

Urinary Lymphoepithelioma-like carcinoma are rare tumours classified according to Lymphoepithelioma component as pure (100%), predominant (≥50%) or focal (<50%), that is correlated with prognosis.

We present here an original case of a 54 year-old man who was diagnosed with a high-grade T2 transitional cell carcinoma associated with LELC >50%. Four cycles of neoadjuvant gemcitabine and platinum-based chemotherapy were carried out with a good tolerance. After completed chemotherapy, the patient underwent a radical cyst prostatectomy with lymph nodes dissection and orthotropic urinary diversion. One year after surgery the patient remains free from relapse.

It’s difficult to define the optimal strategy, literature reporting only small series. Nevertheless, the benefit of chemotherapy is certain.

The outcome is good in the pure and predominant forms and poorer in focal subtypes.

Keywords: Bladder cancer; Lympho-epithelial; Carcinoma; Chemotherapy

Introduction

Lymphoepithelioma-Like Carcinoma (LELC) is a rare tumour, which has a close link to Epstein-Barr virus. (EBV) It’s commonly found in nasal pharyngea, stomach, cervix, lung, hepatobiliary tract and ovary [1].

Its occurrence in the urinary system is very rare. LELC of the urinary bladder was first described by Zuckerberg in 1991 [2]. It represents between 0,4 and 1,3% of all bladder cancers. These tumours are classified according to Lymphoepithelioma component as pure (100%), predominant (≥50%) or focal (<50%) [3].

We present here an original case of a 54 year-old man who was diagnosed with a LELC of the urinary bladder and discuss its management regarding the lake of data in the literature.

Case Report

A 54 year-old Caucasian man presented few weeks history of haematuria associated with urinary frequency and dysuria. He had no medical history. He underwent surgery for discal hernia and appendicectomy a long time ago. He was a smoker since he was 15 year-old and stopped for fifteen years.

He underwent transurethral resection for its bladder tumour.

The results of histological examination confirmed a highgrade T2 transitional cell carcinoma with LELC >50%. On immunohistochemical staining the CKAE1/AE3, p53, was positive and CK7 and CD20 were negative.

In order to classify this tumor, the patient got a CT scan of the chest and the abdomens as well as a bone scan showing no evidence of loco regional extension or metastases. Blood tests showed a moderate anemia and normal kidney and hepatic functions. The tumor was classified according TNM classification of urinary bladder cancer (2009) as a stage T2b, N0, M0.

After discussion of the case during a multidisciplinary GU round, treatment with neoadjuvant gemcitabine and platinium-based chemotherapy was carried out. The patient received four cycles with a good tolerance (no grade 3 or more toxicity).

After completed chemotherapy, the patient underwent a radical cystoprostatectomy with lymph nodes dissection and orthotopic urinary diversion.

The final pathological evaluation of the tumour was predominant transitional cell carcinoma with LELC, classified ypT2R0pN0 tumour, and a low-risk Gleason 6 prostate adenocarcinoma.

The patient is under close observation with regular clinical and radiologic follow-up. He is for now considering in remission for 6 months.

Discussion

Lymphoepithelioma-like carcinoma of the bladder is a rare variant, often manifesting in T2-T3 (usually muscle-invasive) stages and occurring in male patients of 60 year-old.

They are revealed most of the time by haematuria, generally accompanied with urgency.

These tumours have a favorable prognosis with a five-year survival of 59%, achieving 62% in the pure type, compared to transitional cell carcinoma [4].

They respond better to chemotherapy than transitional cell carcinoma. The exact pathogenesis of this tumour is not well established. Epstein-Barr virus is frequently associated with Lymphoepithelioma of the nasopharynx but has not been found in the LELC of the urinary bladder. Abnormality of p53 regulation might be a part of the pathogenesis [5]. These tumours are characterized by a prominent lymphocytic infiltration. They may occur in an association with transitional cell carcinoma.

    

    

    Figure 1: Figure A: Immunohistochemistry for CD3.
Figure B: Hematein Eosin Safran X10.
Figure C: Keratin.