Malignant tumours

The urethra is much more commonly involved secondarily by urothelial carcinoma of the bladder than by primary carcinoma. As with bladder cancer, secondary urethral involvement is more common in males, with a reported incidence of approximately 10-20%. This may take the form of papillary carcinoma, flat carcinoma in situ (which may extend into periurethral ducts) or prostatic stromal invasion. Distinction is important as the latter has a worse prognosis. The same diagnostic histological criteria apply as in the bladder. In females, total urethrectomy is usually performed as part of the cystectomy procedure but, in males, urethrectomy is only performed when separate biopsies show prostatic urethra involvement. Recurrence of urothelial carcinoma in the urethral stump following a urethra-sparing cystectomy may be treated by instillation of BCG immunotherapy or, if there is stromal invasion, transurethral resection or urethrectomy.

Primary urethral carcinoma is more common in females than males and affects mainly those over 50 years of age. Aetiological factors have not been clearly elucidated, although chronic inflammation may play a role. Most tumours arising proximally in the urethra are urothelial in type whereas distal lesions are more often squamous, reflecting the normal epithelial linings at these sites. Adenocarcinoma is seen in association with diverticula, prostatic adenocarcinoma or, in women, arising in periurethral glands. The clear cell variant should be distinguished from nephrogenic adenoma and spread of malignancy from the female genital tract or kidney. In males, primary urothelial carcinoma is usually treated by surgical excision, the extent of which depends on the location and stage of the tumour. Radiotherapy has the advantage of preserving the penis but has a higher rate of tumour recurrence and may result in urethral stricture. Primary urethral carcinoma in the female usually involves the proximal urethra and is locally advanced at presentation. Aggressive surgery, radiotherapy or a combination of both is often required for local control or palliation. Brachytherapy or adjuvant radiosensitising chemotherapy are other treatment options. Local excision is often adequate for distal urethral carcinoma in the female.

Prognosis: prognosis relates to anatomical location and pathological stage. Distal carcinomas have a better prognosis as they are often well-differentiated squamous cell or verrucous types and present earlier. Proximal tumours are more frequently high-grade and present at a later stage, hence prognosis is worse. In men, overall five-year survival rates are 60-70% for penile urethral carcinomas and only 20% for membranous/prostatic urethral lesions.

Other cancers: rare but include adenosquamous carcinoma, small cell carcinoma, malignant melanoma, lymphoma/leukaemia, embryonal rhabdomyosarcoma (in children), aggressive angiomyxoma (in women) and metastatic carcinoma.

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