Primary urethral carcinoma (PUC) is a rare malignancy accounting for less than 1% of genitourinary cancers, with a predilection for men and African Americans. The sites and histology of urethral carcinoma vary by gender and anatomic location. Squamous cell carcinoma is most common among both genders but adenocarcinomas are noted in 15% to 35% of cases among women. Obstructive or irritative symptoms and hematuria are common modes of presentation. Clinical evaluation includes cystourethroscopy with biopsy and examination under anesthesia. Magnetic resonance imaging provides a highly effective method to image the primary tumor while defıning the potential involvement of surrounding structures. Most tumors are localized, with regional metastases to nodal sites seen in up to 30% of cases in both genders, while distant metastases at presentation are rare (0 to 6%), but occur in up to 40% of cases with recurrent disease. Among men, the two most important prognostic factors are disease location and stage. Low-stage tumors (T1-2) and tumors involving the fossa navicularis or the penile urethra have a better prognosis than higher-stage tumors (>T2 or N+) and lesions involving the bulbomembranous urethra. In women, in addition to stage and location, the size of the tumor has also prognostic implications. While Surgery and radiotherapy are of benefit in early stage disease, advanced stage PUC requires multimodal treatment strategies to optimize local control and survival. These include induction chemotherapy followed by surgery or radiotherapy and concurrent chemoradiation with or without surgery. The latter strategy has been used successfully to treat other HPV-related cancers of the vagina, cervix and anus and may be of value in achieving organ preservation. Given the rarity of PUC, prospective multi-institutional studies are needed to better define the optimal treatment approach for this disease entity.
Written by:
Dayyani F, Hoffman K, Eifel P, Guo C, Vikram R, Pagliaro LC, Pettaway C Are you the author?
Division of Hematology and Oncology, Banner MD Anderson Cancer Center, Gilbert, AZ; Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
Reference: BJU Int. 2014 Jan 21 (Epub ahead of print)
doi: 10.1111/bju.12630
PubMed Abstract
PMID: 24447439
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