Primary urethral carcinoma: Results from a single center experience

Primary urethral carcinoma (PUC) is a rare neoplastic disease arising in the urethra, without any evidence of a previous or synchronous carcinoma of the entire urinary tract. Since rare diseases are often incorrectly diagnosed and managed, the aim of this study was to analyze the experience of a single urology center in the treatment of PUC, focusing on neoplasms arising from the male anterior urethra.

Medical records of patients with neoplasms at the level of the penile and bulbar urethra who presented at our tertiary referral center between January 1988 and December 2018 were retrospectively reviewed. Patients with carcinoma of the prostatic urethra were excluded. The diagnosis was obtained with the aid of urethroscopy and lesion biopsy. Local staging was performed by means of contrast-enhanced MRI in selected patients. Staging was achieved by clinical examination, ultrasonography, and CT scan. Radical surgery (radical cystectomy + total penectomy + bilateral inguinal lymphadenectomy) was proposed to patients with ≥T2 tumors or cN+ with a good performance status, proximal tumor and without severe comorbidities. In case of nodal involvement, neoadjuvant chemotherapy was additionally offered. Patients with localized disease (

A total of 13 male patients with anterior PUC were studied retrospectively. Total penectomy was performed in 7 cases, while partial urethrectomy was performed in 5 cases, and partial penectomy in 1 case. Of the 7 patients who underwent total penectomy, 5 underwent radical cystectomy with bilateral inguinal lymphadenectomy. Three patients were treated with neoadjuvant systemic chemotherapy, of which 2 were cT3N+ and 1 was cT3N0. The predominant histologic subtype was squamous cell carcinoma (SCC), found in 7 patients (53.8%), while urothelial carcinoma (UC) was present in 5 patients (38.5%) and a malignant fibro-histiocytoma in 1 case. Most patients were diagnosed at advanced stages of the disease, with T2 tumors in 15.4% and T3 tumors in 46.2% of the cases. Median follow-up was 24 months (range 1-294). After surgical treatment, 8 patients (61.5%) presented disease recurrence after a median of 6.3 months, with a 5-year RFS of 58% ± 14%. The 5-year OS rate was 50% ± 14% while the 5-year CSS rate was 66% ± 13%.

Given its rarity, urethral carcinoma should be treated in a referral center. Treatment options include a radical approach or penile-preserving surgery (urethrectomy or partial penectomy) in cases of localized disease. Due to the high recurrence rate, strict follow-up is mandatory.