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Education - Case Histories |
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| Lymphoepithelial-like carcinoma of the urinary bladder - Recognition has diagnostic and clinical implications | ||
| MH Winkler, PVS Kumar, DA Gillatt, D Patterson and DS Peat 11 February 2000 |
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| Lymphoepithelioma is an undifferentiated malignant epithelial tumour of the nasopharynx that is histologically distinctive by virtue of a prominent lymphoid infiltrate suggestive of malignant lymphoma(1, 2). Tumours of similar histological type have been described in the lung(3), stomach(4), skin(5), cervix(6), thymus(7) and bladder(8). Lymphoepithelial-like carcinoma (LELC) is a term proposed for these distinctive malignant epithelial neoplasms. Only 13 cases of LELC of the bladder have been described(8,9,10). Distinguishing between LELC of the bladder and malignant lymphoma is imperative and has clinical implication(1, 10). | ||
| Clinical History | ||
| We report the cases of 2 patients who were treated for LELC of the bladder aged 57 and 76. Both cases presented with haematuria. Case 1 was diagnosed with pT1 G3 TCC at presentation. He underwent three further transurethral resections for recurrences and proceeded to radical cystectomy. The tumour had recurred as a pT2b G3 pure LELC. Case 2 presented with a pT2 G3 TCC with squamoid and LELC component and was treated with radical cystectomy. Both patients are well at 20 and 18 month follow up. | ||
| Morphology and Immunohistochemistry | ||
| The LELC component in both tumours was indistinguishable from lymphoepithelioma of the nasopharynx and was characterized by nests of undifferentiated malignant cells with ill-defined cytoplasmic borders, prominent nucleoli, and numerous mitoses growing in a typical syncytial pattern (Figure 1 and 2). |
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| The neoplastic cells showed intense positivity for cytokeratin (AE1/AE3) (Figure 3) and the lymphoid infiltrate comprised mixed B and T cells (CD 3 and CD 79a) with many admixed CD68 positive macrophages. | ||
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| Discussion | ||
Awareness of this rare variant of bladder carcinoma is necessary to avoid erroneous diagnosis and to identify patients with a better prognosis (10). It is imperative to make the distinction from malignant lymphoma in its pure form. Immunohistochemistry for cytokeratin is invaluable to establish the origin of the tumor (2, 9). Primary lymphoma of the bladder is extremely rare and usually advanced and poses a different therapeutic challenge(11). A secondary lymphoma of the bladder should prompt the clinician to search for a primary site elsewhere. A common diagnosis in scant bladder biopsies is chronic cystitis and a dense lymphoid infiltrate should alert the pathologist to search for neoplastic cells, as it is possible to pass these off as reactive histiocytes and overlook the LELC pattern(8). This tumor has a strong association with EBV in nasopharyngeal carcinomas and the common etiology has been shown in other anatomical sites(12). This has not been investigated in the bladder. Based on the reported sensitivity to chemotherapy of lymphoepitheliomas of the nasopharynx(13) (Figure 4), pure LELC of the bladder appear to respond to chemotherapy. |
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| Dinney et al.(1993)(10) treated three muscle invasive LELC of the bladder with cisplatin-based chemotherapy with the intent of salvaging the bladder. All three patients did not show signs of recurrence after 6 years of follow-up. The favorable outcome of pure LELC is explained in part by the strong host immune response against the tumour cells(8). Tumours with predominant TCC and LELC behave more like high grade TCCs and have an unfavorable outcome(9). Our patients had their bladders removed, as the evidence for bladder preservation is at best circumstantial and therefore should not be the basis for any treatment decisions. However, it provides the potential for treatment of residual and recurrent disease. | ||
| References 1. Carbone A, Micheau C. Pitfalls in the microscopic diagnosis of undifferentiated carcinoma of the nasopharyngeal type (lymphoepithelioma). Cancer 1982, 50:1344-9 2. Oppedal BR, Bohler PJ, Marton PF, Brandzaeg P. Carcinoma of the nasopharynx. Histological examination with supplementary immunohistochemistry. Histopathology 1987, 11:1161 3. Butler AE, Colby TV, Weiss L, Lombard C. Lymphoepithelioma-like carcinoma of the lung. Amer J Path 1989, 13;632 4. Watanabe H, Enjoji M, Imai T. Gastric carcinoma with lymphoid stroma. Its morphological characteristics and prognostic correlations. Cancer 1976, 38:232 5. Swanson SA, Cooper PH, Mills SE, Wick MR. Lymphoepithelioma-like carcinoma of the skin. Mod Path 1988, 1:359 6. Mills SE, Austin MB, Randall ME. Lymphoepithelioma-like carcinoma of the uterine cervix. A distinctive, undifferentiated carcinoma with inflammatory stroma. Amer J Surg Path 1985, 9:883 7. Wick MR, Scheithauer BW, Weiland LH, Bernatz PE. Primary thymic carcinomas. Amer J Surg Path 1982, 6:613 8. Zuckerberg LR, Harris NL, Young RH. Carcinomas of the urinary bladder simulating malignant lymphoma. A report of five cases. Amer J Surg Path 1991, 15:569-576 9. Amin MB, Ro JY, Lee KM, Ordonez NG, Dinney CB et al. Lymphoepithelioma-like carcinoma of the urinary bladder. Amer J Surg Path 1994, 18:466-473 10. Dinney CP, Ro JY, Babaian RJ, Johnson DE. Lymphoepithelioma of the bladder: a clinicopathological study of 3 cases. J Urol 1993;149:840-842 11. Simpson RHW, Bridger JE, Anthony PP, et al. Malignant lymphoma of the lower urinary tract: a clinicopathologic study with review of the literature. Br J Urol 1991, 85:254-60 12. Pittaluga S, Wong MP, Chung LP, Loke SL. Clonal Epstein-Barr virus in lymphoepithelioma-like carcinoma of the lung. Amer J Surg Path 1993, 17:678-682 13. Rahima M, Rakowsky E, Barzilay J, Sidi J. Carcinoma of the nasopharynx. An analysis of 91 cases and comparison of differing treatment approaches. Cancer 1986, 58:843-9 |
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