Urethral cancer: a comprehensive review endorsed by the Global Society of Rare Genitourinary Tumours.

OBJECTIVE: To determine the effectiveness and adverse effects of urethrectomy alone or as part of multimodal therapy (MMT). METHODS: A comprehensive search was conducted across MEDLINE (OVID), EMBASE, LILACS and the Cochrane Central Register of Controlled Trials (CENTRAL) databases, from their inception to the present date. The study cohort comprised individuals aged 16 years and older diagnosed with urethral tumours at any stage who underwent either isolated urethrectomy or urethrectomy as an integral component of MMT. RESULTS: Ninety-two studies comprising 25 480 patients met the inclusion criteria. Surgical outcomes for urethral cancer vary considerably, with 5-year overall survival (OS) ranging from 10% to 68% based on disease extent, approach, and gender. Radiotherapy (RT) alone provides 5-year OS of approximately 40%. Combined regimens provide better outcomes compared to single modalities, including reduced recurrence and enhanced survival. However, trimodal therapy showed survival benefits only for urothelial subtypes, indicating the need to tailor management according to cancer type. MMT with neoadjuvant chemotherapy prior to surgery demonstrated the most consistent survival gains. CONCLUSIONS: The management of urethral cancer demands a nuanced, personalised approach, accounting for factors such as tumour location, sex, and tumour stage. MMT combining surgery, chemotherapy and RT has shown the ability to enhance outcomes in advanced disease. More extensive collaborative studies through specialised centres are imperative to advance evidence-based protocols and refine treatment in order to improve survival.

[A Case of Small Cell Carcinoma of the Urethra].

We report a case of small cell carcinoma of the urethra with inguinal lymph node metastases. A 50- year-old female patient presented with gross hematuria. Cystoscopy and computed tomography (CT) revealed a tumor surrounding the urethra and an inguinal lymphadenopathy. Biopsy of the urethral tumor demonstrated small cell carcinoma. Four courses of chemotherapy with etoposide and cisplatin, followed by 66 Gy of irradiation achieved complete remission. Unfortunately, 14 months later, positroemission-CT scan revealed recurrence of inguinal lymph node metastases. Although seven courses of chemotherapy with nogitecan were carried out, a new metastatic bone tumor developed. Amrubicin was administered as a third-line treatment, but was canceled after one course because of side effects. The patient died at 39 months after diagnosis. Small cell carcinoma of urethra with metastases has extremely poor prognosis, as is demonstrated by this case.

Evaluation and management of urethral and periurethral masses in women.

PURPOSE OF REVIEW: Female periurethral masses are an uncommon occurrence. The purpose of this review is to describe etiologies of female urethral and periurethral masses and to provide an update on diagnosis and management. RECENT FINDINGS: The most common causes of periurethral and urethral masses in women are urethral caruncles, urethral diverticula, and Skene's gland cysts. Urethral meatal lesions such as urethral caruncles and prolapse can be managed conservatively with topical estrogen therapy and close follow-up or should be excised in the setting of thrombosis, significant or recurrent bleeding, acute urinary retention, or persistent pain. Benign periurethral gland masses, such as Skene's gland cysts, Gartner's duct cysts, and Mullerian duct cysts, remain rare. Recent case series reveal a high rate of surgical management of these lesions with few complications. Urethral malignancy or malignant transformation of benign etiologies are even rarer but can be aggressive in nature and should be treated promptly. SUMMARY: Nonspecific urinary and vaginal symptoms as well as similar physical presentations make diagnosis of urethral and periurethral lesions in females difficult. Magnetic resonance imaging is useful for differentiation of periurethral masses. The decision for conservative or surgical management is typically guided by patient symptom bother, as well as concern for urethral malignancy.

Clinical features and oncological outcomes of primary female urethral cancer.

PURPOSE: Investigation of the clinical features and treatment outcomes of primary female urethral cancer (FUC) at a single institution. MATERIALS AND METHODS: We retrospectively reviewed 32 FUC patients during 1997-2017. We investigated preoperative risk factors predicting overall (overall survival [OS]) and recurrence-free survival (RFS) and reviewed clinical features, treatment modality, and oncologic outcomes according to pathology. The median follow-up duration and age was 56 months (range: 4-229) and 61 years (range: 15-82), respectively. RESULTS: The median OS and RFS were 70 and 16 months, respectively. A total of 19 (59.4%) patients received systemic chemotherapy, including 14 (43.8%) who received radiation therapy. Further, 22 patients (68.8%) underwent surgery. On univariate analysis, >T2, N+, and tumor size ≥3 cm were associated with poorer OS. There were 15 cases of distant metastasis and five local recurrences. Outcomes were poorest in adenocarcinoma (AC), moderate in clear cell carcinoma and transitional cell carcinoma, and best in squamous cell carcinoma (SCC). CONCLUSION: Female urethral lesions should be carefully examined to exclude FUC. Distal urethral SCC was responsive to surgical excision, but proximal urethral AC had poor oncological outcome even after extensive treatment. Due to the heterogeneity and poor prognosis of FUC, multimodal treatment is mandatory.

Primary clear cell adenocarcinoma of female urinary tract: a case report and literature review.

BACKGROUND: Primary clear cell adenocarcinoma of the urethra is extremely rare, reported only in single case reports, and its histological origin is not clear. There is no standard treatment for CCAU at present, and surgery is still the main treatment for CCAU without distant metastasis. CASE PRESENTATION: A 67-year-old female complained of gross hematuria with frequent micturition and urgency. No urethral diverticulum was found by cystoscopy or MRI, and the mass grew around the urethra. Urethral and anterior pelvic viscera resection was performed. Clear cell adenocarcinoma was confirmed by immunohistochemistry after the operation, and no recurrence or metastasis was found after one year of follow-up. CONCLUSION: CCAU is very rare, and most cases originate from the urethral diverticulum and some may also originate from tissues around the urethra. For CCAU patients without distant metastasis, the main treatment is still surgery, and radiotherapy and chemotherapy can be performed for patients with distant metastasis. Gene detection may provide guidance for the precise chemotherapy of CCAU.

Immunotherapy combined with high- and low-dose radiation to all sites leads to complete clearance of disease in a patient with metastatic vaginal melanoma.

A 73-year-old woman with metastatic vaginal mucosal melanoma that had progressed on ipilimumab and nivolumab experienced clinical and radiographic complete response to dual checkpoint inhibitor immunotherapy given in combination with high-dose plus low-dose radiation. General characteristics and treatment options in this disease are highlighted.

Primary urethral cancer: treatment patterns and associated outcomes.

OBJECTIVES: To evaluate treatment patterns and associated outcomes of patients with urethral cancer. PATIENTS AND METHODS: After obtaining institutional review board approval we identified 165 patients treated for primary urethral cancer between 1956 and 2017. Treatment included monotherapy (surgery or radiation), dual therapy (surgery+radiation, surgery+chemotherapy, or chemotherapy+radiation) or triple therapy (surgery+radiation+chemotherapy). Rates of different treatments were described by treatment year. The association between treatment type and outcomes was evaluated with multivariable Cox regression models, adjusting for disease characteristics. RESULTS: The study cohort included 74 men and 91 women, with a median age of 61 years. Common histologies were squamous cell (36%), urothelial (27%) and adenocarcinoma (25%). At presentation, 72% of patients had invasive disease, 24% had nodal involvement, and 5% had metastases. Treatment included monotherapy (57%), dual therapy (21%), and triple therapy (10%). The use of monotherapy decreased over time, while rates of dual therapy remained consistent, and rates of triple therapy increased. The median follow-up was 4.7 years. Estimated 5-year local recurrence-free, disease-specific and overall survival were 51%, 48% and 41%, respectively. Monotherapy was associated with decreased local recurrence-free survival after adjusting for stage, histology, sex and year of treatment (P = 0.017). There was no evidence that treatment type was associated with distant recurrence, cancer-specific or overall survival. CONCLUSIONS: We found preliminary evidence that multimodal therapy, more commonly used in recent years, was of benefit in patients with primary urethral cancer. This finding should be confirmed in further studies involving multiple centres because of the low incidence of the disease.