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García-Perdomo HA, Dávila-Raigoza AM, Summers E, Billingham L, Necchi A, Griffiths G, Spiess PE. Urethral cancer: a comprehensive review endorsed by the Global Society of Rare Genitourinary Tumours. BJU Int 2024; 134:175-184. [PMID: 38587299 DOI: 10.1111/bju.16334] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/09/2024]
Abstract
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.
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Affiliation(s)
- Herney Andrés García-Perdomo
- UROGIV Research Group, Department of Surgery, School of Medicine, Universidad del Valle, Cali, Colombia
- Division of Urology/Urooncology, Department of Surgery, School of Medicine, Universidad del Valle, Cali, Colombia
| | | | - Ellie Summers
- Cancer Research U.K., Clinical Trials Unit, Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK
| | - Lucinda Billingham
- Cancer Research U.K., Clinical Trials Unit, Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK
| | - Andrea Necchi
- Urological Research Institute, IRCCS Ospedale San Raffaele, Vita-Salute San Raffaele University, Milan, Italy
| | - Gareth Griffiths
- Cancer Research U.K., Southampton Clinical Trials Unit, University of Southampton, Southampton, UK
| | - Philippe E Spiess
- Department of Genitourinary Oncology and Tumor Biology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA
- Urology and Oncology, University of South Florida, Tampa, FL, USA
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Paighan NM, Goel HK, Makkar A. A case report on locally advanced squamous cell carcinoma in permanent perineal urethrostomy: A surgical conundrum!! Indian J Urol 2024; 40:197-199. [PMID: 39100612 PMCID: PMC11296581 DOI: 10.4103/iju.iju_106_24] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2024] [Revised: 05/15/2024] [Accepted: 06/13/2024] [Indexed: 08/06/2024] Open
Abstract
Primary urethral cancer is a rare condition often manifesting in the sixth decade of life and is typically associated with urethral stricture disease. A 65-year-old male, a known case of stricture urethra, underwent perineal urethrostomy 10 years back. He presented with a growth at the urethrostomy site and the biopsy revealed squamous cell carcinoma. Magnetic resonance imaging of the pelvis revealed that the lesion extended into the penile and prostatic urethra with possible involvement of the corpus spongiosum, bulbospongiosus, and the bladder neck. Radical cystectomy, penectomy, scrotectomy, and bilateral orchiectomy with ileal conduit were performed. At 18-months of follow-up, the patient is recurrence-free.
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Affiliation(s)
- Nitin Madanrao Paighan
- Department of Urology and Renal Transplantation, ABVIMS and Dr. RML Hospital, New Delhi, India
| | - Hemant Kumar Goel
- Department of Urology and Renal Transplantation, ABVIMS and Dr. RML Hospital, New Delhi, India
| | - Arun Makkar
- Department of Urology and Renal Transplantation, ABVIMS and Dr. RML Hospital, New Delhi, India
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March B, Palma CA, Leslie S, Watson G, Lee P, Austin KK, Solomon M, Jeffery N. Phallus Preservation for Locally Advanced Proximal Primary Urethral Carcinoma: Technique and Outcomes. Urology 2023; 173:198-203. [PMID: 36646175 DOI: 10.1016/j.urology.2022.12.034] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/05/2022] [Revised: 12/25/2022] [Accepted: 12/27/2022] [Indexed: 01/15/2023]
Abstract
INTRODUCTION To describe a novel method of penile sparing perineal urethrectomy for locally advanced proximal primary urethral cancers (PUC). TECHNICAL CONSIDERATIONS In mid-2021, 2 cases underwent pelvic exenterative surgery for pT3 and pT4 PUC. The procedure comprised of a complete urethrectomy, proximal penectomy, en bloc pubectomy and excision of pelvic diaphragm in both cases. One case included a wide excision of scrotum, whilst the other required a prostatectomy and abdominoperineal resection of the rectum to achieve complete tumor resection. A complete R0 resection was achieved in both cases. At 6 months follow up, there is no evidence of ischemic necrosis of the penis and cosmesis is satisfactory to both patients. We provide a comprehensive operative description of both cases, together with illustrations, and discuss the underlying principles of penile preservation in the surgical treatment of locally advanced proximal PUC. CONCLUSION Complete perineal urethrectomy with phallic preservation is feasible in men with locally advanced proximal bulbar urethral cancer in the absence of tumor invasion of the penile shaft. The remnant penis survives off arterial supply from the superficial penile arteries arising from the external pudendal arteries. Phallic preservation may benefit patient's psychological quality of life post-procedure.
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Affiliation(s)
- Brayden March
- Department of Urology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
| | - Catalina A Palma
- Department of Urology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
| | - Scott Leslie
- Department of Urology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Institute of Academic Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
| | - Geoffrey Watson
- Department of Anatomical Pathology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
| | - Peter Lee
- Department of Colorectal Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Surgical Outcomes Research Centre, Sydney Local Health District, Sydney, Australia
| | - Kirk Ks Austin
- Department of Colorectal Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Institute of Academic Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Surgical Outcomes Research Centre, Sydney Local Health District, Sydney, Australia
| | - Michael Solomon
- Department of Colorectal Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Institute of Academic Surgery, Royal Prince Alfred Hospital, Camperdown, NSW, Australia; Surgical Outcomes Research Centre, Sydney Local Health District, Sydney, Australia; Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
| | - Nicola Jeffery
- Department of Urology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia.
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Su H, Zhu Y, Ye D. A brief review on the diagnostic and therapeutic principles of primary urethral cancer. Asian J Urol 2022; 9:423-429. [PMID: 36381601 PMCID: PMC9643286 DOI: 10.1016/j.ajur.2022.04.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2021] [Revised: 02/19/2022] [Accepted: 04/05/2022] [Indexed: 11/23/2022] Open
Abstract
Objective Primary urethral carcinoma (PUC) is a rare malignant carcinoma but with limited therapeutic options. This review aims to provide an overview of the current strategies on this patient settings. Methods Recent literature ranging from January 1987 and December 2021 was assessed through PubMed search to assess the diagnostic and therapeutic principles of PUC. Results A complete of examination including cystoscopy, imaging, and biopsy should be conducted for these patients. Once diagnosed, the clinical decision of PUC should be made according to the tumor location, pathological pattern, and extent of the tumor. For patients with superficial and distal urethral lesions, organ sparing approaches or radical reconstructive procedures can be utilized. While for more advanced disease or nodal involvement, an optimal multimodal treatment strategy consisted of surgery and radiochemotherapy should be adopted. For patients with urothelial carcinoma of the prostate, the management including transurethral resection of the prostate followed by bacille Calmette-Guerin or radical cystoprostatectomy should depend on the infiltration depth of PUC. Conclusion A complete of examination is important for the diagnosis of PUC. The management of PUC should be determined by the location, pathological pattern, and extent of the tumor. More multi-institutional collaborations should be held to investigate better treatment modalities for PUC.
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Affiliation(s)
- Hengchuan Su
- Department of Urology, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, FudanUniversity, Shanghai, China
| | - Yao Zhu
- Department of Urology, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, FudanUniversity, Shanghai, China
| | - Dingwei Ye
- Department of Urology, Fudan University Shanghai Cancer Center, Shanghai, China
- Department of Oncology, Shanghai Medical College, FudanUniversity, Shanghai, China
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D'Amico MJ, Shumaker AD, Chung P. Urethral Cancer After Urethroplasty: A Case Report and Review of the Literature. Urology 2022; 169:218-225. [PMID: 35914585 DOI: 10.1016/j.urology.2022.07.032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2022] [Revised: 07/05/2022] [Accepted: 07/17/2022] [Indexed: 10/16/2022]
Abstract
OBJECTIVE To review and report data on transformation to urethral carcinoma after urethroplasty for urethral stricture disease. Primary urethral carcinoma is a rare entity, and guidelines lack high quality data from which to cite. Urethroplasty is a highly effective treatment for urethral stricture disease, though in rare cases complications may include development of urethral carcinoma. METHODS A systematic PubMed search was performed to identify all articles describing patients with urethral carcinoma after urethroplasty. Data were collected on the following parameters: patient age and sex, indication for urethroplasty, presentation of cancer, imaging, pathology, presence of metastasis, intervention, and outcome. RESULTS The final cohort included fourteen patients, thirteen from previously published cases and one from our institution. The median patient age at presentation was 60, most had endoscopic management prior to urethroplasty, and the majority presented with decreased urinary stream. All patients developed squamous cell carcinoma of the urethra. Patients underwent radical resection, lymph node dissection, chemotherapy, or radiotherapy, often in combination. A majority of patients had died at the time of case report. CONCLUSIONS Development of urethral SCC, particularly after urethroplasty, is a rarely encountered process. Patients and urologists must have a high index of suspicion and investigate symptoms such as fistula or lower urinary tract symptoms, even if these occur many months or even years after BMG. By compiling previously reported cases and adding an additional case to the literature, we hope that familiarity with this entity will lead to earlier recognition and diagnosis of disease.
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Affiliation(s)
- Maria J D'Amico
- Department of Urology, Thomas Jefferson University Hospital, Philadelphia, PA 19107, USA. Maria.D'
| | - Andrew D Shumaker
- Department of Urology, Thomas Jefferson University Hospital, Philadelphia, PA 19107, USA
| | - Paul Chung
- Department of Urology, Thomas Jefferson University Hospital, Philadelphia, PA 19107, USA
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Primary tumor surgery improves survival in non-metastatic primary urethral carcinoma patients: a large population-based investigation. BMC Cancer 2021; 21:857. [PMID: 34315433 PMCID: PMC8314574 DOI: 10.1186/s12885-021-08603-z] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2020] [Accepted: 07/15/2021] [Indexed: 11/10/2022] Open
Abstract
Background Primary urethral carcinoma (PUC) is a rare genitourinary malignancy with a relatively poor prognosis. The aim of this study was to examine the impact of surgery on survival of patients diagnosed with PUC. Methods A total of 1544 PUC patients diagnosed between 2004 and 2016 were identified based on the SEER database. The Kaplan-Meier estimate and the Fine and Gray competing risks analysis were performed to assess overall survival (OS) and cancer-specific mortality (CSM). The multivariate Cox regression model and competing risks regression model were used to identify independent risk factors of OS and cancer-specific survival (CSS). Results The 5-yr OS was significantly better in patients who received either local therapy (39.8%) or radical surgery (44.7%) compared to patients receiving no surgery of the primary site (21.5%) (p < 0.001). Both local therapy and radical surgery were each independently associated with decreased CSM, with predicted 5-yr cumulative incidence of 45.4 and 43.3%, respectively, compared to 64.7% for patients receiving no surgery of the primary site (p < 0.001). Multivariate analyses demonstrated that primary site surgery was independently associated with better OS (local therapy, p = 0.037; radical surgery, p < 0.001) and decreased CSM (p = 0.003). Similar results were noted regardless of age, sex, T stage, N stage, and AJCC prognostic groups based on subgroup analysis. However, patients with M1 disease who underwent primary site surgery did not exhibit any survival benefit. Conclusion Surgery for the primary tumor conferred a survival advantage in non-metastatic PUC patients. Supplementary Information The online version contains supplementary material available at 10.1186/s12885-021-08603-z.
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Farrell MR, Xu JT, Vanni AJ. Current Perspectives on the Diagnosis and Management of Primary Urethral Cancer: A Systematic Review. Res Rep Urol 2021; 13:325-334. [PMID: 34104638 PMCID: PMC8180270 DOI: 10.2147/rru.s264720] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2021] [Accepted: 04/28/2021] [Indexed: 11/23/2022] Open
Abstract
Primary urethral cancer (PUC) is a rare but highly aggressive malignancy that causes malignant urethral obstruction. We conducted a literature review using PubMed to identify original research studies that assessed the diagnosis and management of primary urethral cancer. PUC affects men more than women, is more common in African Americans than Caucasians, and is associated with history of chronic inflammation and irritation of the urinary tract. Patients suspected of PUC should undergo a complete work-up including cystoscopy, magnetic resonance imaging, and biopsy. In men and women, surgical monotherapy ranging from organ-sparing to more radical reconstructive procedures has adequate survival rates for early stage PUC and has been shown to be similarly as effective as radiation monotherapy, while multimodal therapy has become the standard of treatment for advanced stage PUC. Salvage surgery or radiation therapy has been linked with increased survival rates. Nodal involvement at the time of diagnosis is a negative prognosticator and should be treated with multimodal therapy. Further prospective studies with greater sample sizes and standardized clinical trials would allow for greater consistency in evaluating the different treatment modalities for PUC.
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Affiliation(s)
- M Ryan Farrell
- Center for Reconstructive Urologic Surgery, Lahey Hospital and Medical Center, Burlington, MA, USA
| | - Jonathan T Xu
- Center for Reconstructive Urologic Surgery, Lahey Hospital and Medical Center, Burlington, MA, USA
| | - Alex J Vanni
- Center for Reconstructive Urologic Surgery, Lahey Hospital and Medical Center, Burlington, MA, USA
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8
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European Association of Urology Guidelines on Primary Urethral Carcinoma-2020 Update. Eur Urol Oncol 2020; 3:424-432. [PMID: 32605889 DOI: 10.1016/j.euo.2020.06.003] [Citation(s) in RCA: 27] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2020] [Revised: 05/30/2020] [Accepted: 06/10/2020] [Indexed: 01/10/2023]
Abstract
CONTEXT Primary urethral carcinoma (PUC) is a rare cancer accounting for <1% of all genitourinary malignancies. OBJECTIVE To provide updated practical recommendations for the diagnosis and management of PUC. EVIDENCE ACQUISITION A systematic search interrogating Ovid (Medline), EMBASE, and the Cochrane Central Register of Controlled Trials and Cochrane Database of Systematic Reviews was performed. EVIDENCE SYNTHESIS Urothelial carcinoma of the urethra is the predominant histological type of PUC (54-65%), followed by squamous cell carcinoma (16-22%) and adenocarcinoma (10-16%). Diagnosis of PUC depends on urethrocystoscopy with biopsy and urinary cytology. Pathological staging and grading are based on the tumour, node, metastasis (TNM) classification and the 2016 World Health Organization grading systems. Local tumour extent and regional lymph nodes are assessed by magnetic resonance imaging, and the presence of distant metastases is assessed by computed tomography of the thorax/abdomen and pelvis. For all patients with localised distal tumours (≤T2N0M0), partial urethrectomy or urethra-sparing surgery is a valid treatment option, provided that negative intraoperative surgical margins can be achieved. Prostatic Ta-Tis-T1 PUC can be treated with repeat transurethral resection of the prostate and bacillus Calmette-Guérin. In prostatic or proximal ≥ T2N0 disease, neoadjuvant cisplatin-based chemotherapy should be considered prior to radical surgery. All patients with locally advanced disease (≥T3N0-2M0) should be discussed within a multidisciplinary team. In men with locally advanced squamous cell carcinoma, curative radiotherapy combined with radiosensitising chemotherapy can be offered for definitive treatment and genital preservation. In patients with local urethral recurrence, salvage surgery or radiotherapy can be offered. For patients with distant metastatic disease, systemic therapy based on tumour characteristics can be evaluated. CONCLUSIONS These updated European Association of Urology guidelines provide up-to-date guidance for the contemporary diagnosis and management of patients with suspected PUC. PATIENT SUMMARY Primary urethral carcinoma (PUC) is a very rare, but aggressive disease. These updated European Association of Urology guidelines provide evidence-based guidance for clinicians treating patients with PUC.
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Mano R, Vertosick EA, Sarcona J, Sjoberg DD, Benfante NE, Donahue TF, Herr HW, Donat SM, Bochner BH, Dalbagni G, Goh AC. Primary urethral cancer: treatment patterns and associated outcomes. BJU Int 2020; 126:359-366. [PMID: 32336001 DOI: 10.1111/bju.15095] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
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.
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Affiliation(s)
- Roy Mano
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA.,Department of Urology, Tel-Aviv Sourasky Medical Centre, Sackler School of Medicine, Tel-Aviv University, Tel Aviv-Yafo, Israel
| | - Emily A Vertosick
- Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Joseph Sarcona
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA.,Department of Urology, Lenox Hill Hospital, New York, NY, USA
| | - Daniel D Sjoberg
- Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Nicole E Benfante
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Timothy F Donahue
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Harry W Herr
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - S Machele Donat
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Bernard H Bochner
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Guido Dalbagni
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
| | - Alvin C Goh
- Department of Surgery, Urology Service, Memorial Sloan Kettering Cancer Centre, New York, NY, USA
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Shields LBE, Kalebasty AR. Personalized chemotherapy in clear cell adenocarcinoma of the urethra: A case report. World J Clin Oncol 2020. [DOI: 10.5306/wjco.v12.i4.243] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
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Shields LBE, Kalebasty AR. Personalized chemotherapy in clear cell adenocarcinoma of the urethra: A case report. World J Clin Oncol 2020; 11:243-249. [PMID: 32355644 PMCID: PMC7186237 DOI: 10.5306/wjco.v11.i4.243] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2019] [Revised: 03/06/2020] [Accepted: 03/12/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis. Clear cell carcinoma of the urethra represents less than 0.02% of all malignancies in women. Adenocarcinomas account for 10% of female urethral carcinomas, of which 40% are the clear cell variant. Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen. CASE SUMMARY A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria. An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis. A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra. Specialized genetic testing determined personalized chemotherapy. She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab. Following 3 cycles of paclitaxel and bevacizumab, she attained significant clinical improvement, and response by FDG-Positron emission tomography (PET) imaging showed a definite improvement in size and metabolic activity. She achieved complete response after 6 cycles of therapy by PET scan. The patient concluded 11 cycles of paclitaxel and bevacizumab, and a subsequent PET scan confirmed progression of metastatic disease. The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment. CONCLUSION We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations. Our unique case represents the safe and effective use of non-platinum-based chemotherapy in clear cell adenocarcinoma of the urethra.
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Affiliation(s)
- Lisa B E Shields
- Norton Neuroscience Institute, Norton Healthcare, Louisville, KY 40202, United States
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Neoadjuvant Radiation with Concurrent 5-FU Resulting in Complete Pathologic Response in Stage IIIB Squamous Cell Carcinoma of the Urethra. Case Rep Oncol Med 2020; 2020:7948538. [PMID: 32110455 PMCID: PMC7042525 DOI: 10.1155/2020/7948538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2019] [Accepted: 01/25/2020] [Indexed: 11/18/2022] Open
Abstract
Squamous cell carcinoma (SCC) of the urethra is a rare malignancy, comprising less than 1% of all malignancies. The annual age-adjusted incidence of urethral SCC is 4.3 per million in men and 1.5 per million in women. Due to the rarity of the disease, there are a limited number of prospective randomized controlled trials to evaluate the optimal management of locally advanced urethral SCC. Here, we present the case of a 47-year-old man with stage IIIB urethral squamous cell cancer that showed complete clinical and pathologic response to neoadjuvant chemoradiation with only 5-flurouracil after incomplete response to traditional chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP).
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13
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Current Disease Management of Primary Urethral Carcinoma. Eur Urol Focus 2019; 5:722-734. [DOI: 10.1016/j.euf.2019.07.001] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2019] [Revised: 06/06/2019] [Accepted: 07/02/2019] [Indexed: 12/19/2022]
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Singh T, Li QK, Song DY. Pathologic Complete Response After Chemoradiation of a Massive Primary Urethral Carcinoma. Adv Radiat Oncol 2019; 4:487-491. [PMID: 31360804 PMCID: PMC6639762 DOI: 10.1016/j.adro.2019.02.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2019] [Revised: 02/26/2019] [Accepted: 02/27/2019] [Indexed: 11/05/2022] Open
Affiliation(s)
- Tanmay Singh
- Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University, Baltimore, Maryland
| | - Qing Kay Li
- Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland
| | - Daniel Y Song
- Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University, Baltimore, Maryland
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15
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Son CH, Liauw SL, Hasan Y, Solanki AA. Optimizing the Role of Surgery and Radiation Therapy in Urethral Cancer Based on Histology and Disease Extent. Int J Radiat Oncol Biol Phys 2018; 102:304-313. [DOI: 10.1016/j.ijrobp.2018.06.007] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2018] [Revised: 05/17/2018] [Accepted: 06/05/2018] [Indexed: 11/29/2022]
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16
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Guo H, Peng X, Jin C, Wang L, Chen F, Sa Y. Lichen Sclerosus Accompanied by Urethral Squamous Cell Carcinoma: A Retrospective Study From a Urethral Referral Center. Am J Mens Health 2018; 12:1692-1699. [PMID: 29926751 PMCID: PMC6142166 DOI: 10.1177/1557988318782095] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
To evaluate the characteristics of lichen sclerosus (LS) accompanied by urethral squamous cell carcinoma (USCC) and to raise urologists' awareness about the early management of LS, a retrospective analysis was performed on the clinical features, diagnosis, treatment, and prognosis of 18 male genital LS accompanied by USCC patients who were referred to Shanghai Sixth People's Hospital between June 2000 and August 2014. All of the patients had a long-term history of LS, urethral strictures, and urethral dilatation. Seven patients are with distal (glanular or penile) USCC, 10 patients with proximal USCC, and one with entire USCC. The most common presentation, except for LS and urethral strictures, was periurethral abscess, followed by extraurethral mass, pelvic pain, urethrocutaneous fistula, hematuria, and bloody urethral discharge. All had primary surgical excision that was adapted to tumor location and extension. All of the USCC were positive for P53 and Ki-67. P16 was positive in four cases of human papillomavirus (HPV)-associated USCC and negative in 14 cases of HPV-independent USCC. Patients with distal USCC had a significant longer survival time than proximal USCC ( p < .05). LS should be treated early to prevent the disease progression. LS probably has some associations with USCC. Distal USCC has a relatively better prognosis than proximal USCC.
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Affiliation(s)
- Hailin Guo
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China.,3 Department of Urology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Xufeng Peng
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China
| | - Chongrui Jin
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China
| | - Lin Wang
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China.,3 Department of Urology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Fang Chen
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China.,3 Department of Urology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Yinglong Sa
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair Institute, Shanghai, China
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17
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Peng X, Guo H, Jin C, Wang L, Sa Y. Squamous Cell Carcinoma of the Bulbar Urethra Accompanied by Lichen Sclerosus: A Case Report. Am J Mens Health 2017; 12:493-497. [PMID: 29182032 PMCID: PMC5818129 DOI: 10.1177/1557988317743386] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Squamous cell carcinoma (SCC) of the bulbar urethra accompanied by lichen sclerosus (LS) is rarely reported. This study reports the case of a 56-year-old man with urethral squamous cell carcinoma (USCC) accompanied by a long history of genital LS. The man presented with a painful perineal mass and had a long-term history of urethral strictures and urethral dilatation. The patient developed a periurethral abscess that expanded to the perineum and formed an urethrocutaneousperineal fistula. An organ-sparing perineal resection and fistulectomy was performed according to the patient's wishes. During the operation, residue-like pus mixed with necrotic tissues drained out. A section of the prepuce and the necrotic tissues were sent for histological analysis. Hematoxylin and eosin (HE) staining of the excised prepuce revealed classical LS. HE and immunohistochemical (IHC) staining of the necrotic tissues showed well-differentiated USCC. IHC staining showed the USCC to be positive for P53 and Ki-67 and negative for P16, suggesting the USCC was probably associated with LS. The patient received high-dose chemotherapy and radiation therapy and died 10 months after surgery.
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Affiliation(s)
- Xufeng Peng
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair institute, Shanghai, China
| | - Hailin Guo
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair institute, Shanghai, China
| | - Chongrui Jin
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair institute, Shanghai, China
| | - Lin Wang
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair institute, Shanghai, China
| | - Yinglong Sa
- 1 Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.,2 Shanghai Eastern Urological Reconstruction and Repair institute, Shanghai, China
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18
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Multimodal management of primary adenocarcinoma of the female urethra: About four cases. Cancer Radiother 2016; 20:169-75. [DOI: 10.1016/j.canrad.2015.11.005] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2015] [Revised: 11/16/2015] [Accepted: 11/25/2015] [Indexed: 11/21/2022]
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19
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Lucarelli G, Spilotros M, Vavallo A, Palazzo S, Miacola C, Forte S, Matera M, Campagna M, Colamonico O, Schiralli F, Sebastiani F, Di Cosmo F, Bettocchi C, Di Lorenzo G, Buonerba C, Vincenti L, Ludovico G, Ditonno P, Battaglia M. A Challenging Surgical Approach to Locally Advanced Primary Urethral Carcinoma: A Case Report and Literature Review. Medicine (Baltimore) 2016; 95:e3642. [PMID: 27175683 PMCID: PMC4902525 DOI: 10.1097/md.0000000000003642] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Primary urethral carcinoma (PUC) is a rare and aggressive cancer, often underdetected and consequently unsatisfactorily treated. We report a case of advanced PUC, surgically treated with combined approaches.A 47-year-old man underwent transurethral resection of a urethral lesion with histological evidence of a poorly differentiated squamous cancer of the bulbomembranous urethra. Computed tomography (CT) and bone scans excluded metastatic spread of the disease but showed involvement of both corpora cavernosa (cT3N0M0). A radical surgical approach was advised, but the patient refused this and opted for chemotherapy. After 17 months the patient was referred to our department due to the evidence of a fistula in the scrotal area. CT scan showed bilateral metastatic disease in the inguinal, external iliac, and obturator lymph nodes as well as the involvement of both corpora cavernosa. Additionally, a fistula originating from the right corpus cavernosum extended to the scrotal skin. At this stage, the patient accepted the surgical treatment, consisting of different phases. Phase I: Radical extraperitoneal cystoprostatectomy with iliac-obturator lymph nodes dissection. Phase II: Creation of a urinary diversion through a Bricker ileal conduit. Phase III: Repositioning of the patient in lithotomic position for an overturned Y skin incision, total penectomy, fistula excision, and "en bloc" removal of surgical specimens including the bladder, through the perineal breach. Phase IV: Right inguinal lymphadenectomy.The procedure lasted 9-and-a-half hours, was complication-free, and intraoperative blood loss was 600 mL. The patient was discharged 8 days after surgery. Pathological examination documented a T4N2M0 tumor. The clinical situation was stable during the first 3 months postoperatively but then metastatic spread occurred, not responsive to adjuvant chemotherapy, which led to the patient's death 6 months after surgery.Patients with advanced stage tumors of the bulbomembranous urethra should be managed with radical surgery including the corporas up to the ischiatic tuberosity attachment, and membranous urethra in continuity with the prostate and bladder. Neo-adjuvant treatment may be advisable with the aim of improving the poor prognosis, even if the efficacy is not certain while it can delay the radical treatment of the disease.
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Affiliation(s)
- Giuseppe Lucarelli
- From the Department of Emergency and Organ Transplantation, Urology, Andrology and Kidney Transplantation Unit, University of Bari (GLucarelli, MS, AV, SP, CM, MM, SF, MC, OC, FSchiralli, FSebastiani, FD, CBettocchi, PD, MB); Division of General Surgery, Polyclinic Hospital (LV), Bari; Department of Clinical Medicine, Medical Oncology Unit, Federico II University, Naples (GD, CBuonerba); and Department of Urology, Minimally Invasive and Robotic Surgery Center "F. Miulli", Acquaviva della Fonti (GLudovico), Italy
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20
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Blick C, Sahdev V, Mitra A, Nigam R, Muneer A. The contemporary management of primary urethral carcinoma. JOURNAL OF CLINICAL UROLOGY 2015. [DOI: 10.1177/2051415815584129] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Primary urethral cancer is a rare malignancy; the most common histological subtype is squamous cell carcinoma. The aetiology of this cancer is similar to penile cancer and the human papilloma virus (HPV) is thought to be an important factor in tumourigenesis. Surgery with or without chemoradiotherapy is the accepted treatment for primary urethral cancer. Current practice supports penile-sparing surgery, to maximise functional and psychological outcomes. We have reviewed the literature to summarise the pathogenesis and management of primary urethral cancer.
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Affiliation(s)
| | - Varun Sahdev
- Department of Urology, University College Hospital, London, UK
| | - Anita Mitra
- Department of Urology, University College Hospital, London, UK
| | - Raj Nigam
- Department of Urology, Royal Surrey County Hospital, Guildford, UK
| | - Asif Muneer
- Department of Urology, University College Hospital, London, UK
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21
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Serrano N, Chang M, Leland Rogers C, Orton M, Mannino R, Grob M, Agarwal R, Moghanaki D. Patient autonomy and shared decision making in the management of urethral cancer. Pract Radiat Oncol 2015; 6:66-70. [PMID: 26577009 DOI: 10.1016/j.prro.2015.09.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2015] [Revised: 08/28/2015] [Accepted: 09/09/2015] [Indexed: 12/14/2022]
Affiliation(s)
- Nicholas Serrano
- Department of Radiation Oncology, Virginia Commonwealth University, Massey Cancer Center, Richmond, Virginia
| | - Michael Chang
- Department of Radiation Oncology, Virginia Commonwealth University, Massey Cancer Center, Richmond, Virginia; Radiation Oncology Service, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia
| | - C Leland Rogers
- Department of Radiation Oncology, Virginia Commonwealth University, Massey Cancer Center, Richmond, Virginia; Radiation Oncology Service, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia
| | - Matthew Orton
- Department of Radiation Oncology, Indiana University, IU Health Arnett Cancer Center, Lafayette, Indiana
| | - Rosemarie Mannino
- Hematology Oncology Section, Medical Service, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia; Division of Hematology, Oncology and Palliative Care, Virginia Commonwealth University, Massey Cancer Center, Richmond, Virginia
| | - Mayer Grob
- Department of Urology, Virginia Commonwealth University, Richmond, Virginia; Department of Urology, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia
| | - Rakesh Agarwal
- Department of Radiology, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia
| | - Drew Moghanaki
- Department of Radiation Oncology, Virginia Commonwealth University, Massey Cancer Center, Richmond, Virginia; Radiation Oncology Service, Hunter Holmes McGuire VA Medical Center, Richmond, Virginia.
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22
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Bagshaw HP, Williams NL, Huang YJ, Tward JD, Gaffney DK. Palladium interstitial implant in combination with external beam radiotherapy and chemotherapy for the definitive treatment of a female urethral carcinoma. Gynecol Oncol Rep 2015; 13:40-3. [PMID: 26425719 PMCID: PMC4563585 DOI: 10.1016/j.gore.2015.06.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2015] [Revised: 06/03/2015] [Accepted: 06/06/2015] [Indexed: 11/03/2022] Open
Abstract
Primary urethral cancer is a rare diagnosis, especially in females. This report presents the utilization of a palladium interstitial implant and a review of the retrospective data published on the management of female urethral cancer. Excellent local control and survival has been obtained with the use of a palladium interstitial implant in combination with external beam radiotherapy and concurrent chemotherapy. This modality represents a novel and effective way to treat primary urethral cancer in females.
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Affiliation(s)
- Hilary P Bagshaw
- Department of Radiation Oncology, University of Utah, Huntsman Cancer Hospital, United States
| | - Ned L Williams
- Department of Radiation Oncology, University of Utah, Huntsman Cancer Hospital, United States
| | - Y Jessica Huang
- Department of Radiation Oncology, University of Utah, Huntsman Cancer Hospital, United States
| | - Jonathan D Tward
- Department of Radiation Oncology, University of Utah, Huntsman Cancer Hospital, United States
| | - David K Gaffney
- Department of Radiation Oncology, University of Utah, Huntsman Cancer Hospital, United States
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23
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Ekin RG, Yildirim Z, Bayol U, Diniz G, Karaca C, Zorlu F. Primary squamous cell carcinoma of the urethral diverticulum mimicking prostate cancer: Case report and review of the literature. Can Urol Assoc J 2015; 9:E329-32. [PMID: 26029309 DOI: 10.5489/cuaj.2326] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
Primary urethral carcinomas are uncommon, with urothelial carcinoma as the most common subtype. Urethral diverticulum is also rarely seen in men. A 44-year-old male presented with voiding symptoms. Abdominoperineal resection, prostatectomy, bladder neck excision, and proximal urethral excision were performed. A pathological examination revealed a well-differentiated squamous cell carcinoma (SCC) located inside an urethral diverticulum. We report this unusual case because primary SCC of the male urethral diverticulum is extremely rare. To our knowledge, our patient is only the second reported case.
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Affiliation(s)
- Rahmi Gokhan Ekin
- Department of Urology, Tepecik Teaching and Research Hospital, Izmir, Turkey
| | - Zubeyde Yildirim
- Department of Pathology, Tepecik Teaching and Research Hospital, Izmir, Turkey
| | - Umit Bayol
- Department of Pathology, Tepecik Teaching and Research Hospital, Izmir, Turkey
| | - Gulden Diniz
- Department of Pathology, Tepecik Teaching and Research Hospital, Izmir, Turkey
| | - Cezmi Karaca
- Department of General Surgery, Tepecik Teaching and Research Hospital, Izmir, Turkey
| | - Ferruh Zorlu
- Department of Urology, Tepecik Teaching and Research Hospital, Izmir, Turkey
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24
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Kent M, Zinman L, Girshovich L, Sands J, Vanni A. Combined Chemoradiation as Primary Treatment for Invasive Male Urethral Cancer. J Urol 2015; 193:532-7. [DOI: 10.1016/j.juro.2014.07.105] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/23/2014] [Indexed: 12/22/2022]
Affiliation(s)
- Marissa Kent
- Department of Urology, Lahey Hospital and Medical Center, Burlington, Massachusetts
| | - Leonard Zinman
- Department of Urology, Lahey Hospital and Medical Center, Burlington, Massachusetts
| | - Lyubov Girshovich
- Department of Radiation Oncology, Lahey Hospital and Medical Center, Burlington, Massachusetts
| | - Jacob Sands
- Department of Oncology, Lahey Hospital and Medical Center, Burlington, Massachusetts
| | - Alex Vanni
- Department of Urology, Lahey Hospital and Medical Center, Burlington, Massachusetts
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25
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Tritschler S, Lellig K, Roosen A, Horng A, Stief C. [Organ and function preservation in urethral cancer]. Urologe A 2014; 53:1310-5. [PMID: 25113827 DOI: 10.1007/s00120-014-3555-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
Primary urethral carcinomas are rare tumors that can occur both in men and women. Histological patterns of these tumors are mixed, urothelial tumors occur as well as squamous cell tumors or adenocarcinomas.There are different clinical factors that define clinical prognosis, and the 1- and 5-year cancer-free survival is 75% and 54%. Therapy of locally limited disease is surgical resection, and organ-preserving treatment is possible if negative frozen sections prove complete surgical resection. However, in men a perineal urethrostomy might be necessary, and in women there is a high risk of urinary incontinence if more than 2 cm of the distal urethra is resected.In case of locally advanced tumors or tumors of the proximal urethra, a radical urethrectomy with supravesical urinrary diversion is necessary. In some cases neoadjuvant (radio-)chemotherapy may be an option.
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Affiliation(s)
- S Tritschler
- Urologische Klinik und Poliklinik, Klinikum Großhadern, Ludwig-Maximilians-Universität, München, Marchioninistraße 15, 81377, München, Deutschland,
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26
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Dayyani F, Hoffman K, Eifel P, Guo C, Vikram R, Pagliaro LC, Pettaway C. Management of advanced primary urethral carcinomas. BJU Int 2014; 114:25-31. [PMID: 24447439 DOI: 10.1111/bju.12630] [Citation(s) in RCA: 53] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Primary urethral carcinoma (PUC) is a rare malignancy accounting for <1% of genitourinary cancers, with a predilection for men and African-Americans. The sites and histology of urethral carcinoma vary by gender and anatomical location. Squamous cell carcinoma is most common among both genders but adenocarcinomas are noted in 15-35% of cases among women. Obstructive or irritative symptoms and haematuria are common modes of presentation. Clinical evaluation includes cystourethroscopy with biopsy and examination under anaesthesia. Magnetic resonance imaging provides a highly effective method to image the primary tumour while defıning the potential involvement of surrounding structures. Most tumours are localised, with regional metastases to nodal sites seen in up to 30% of cases in both genders, while distant metastases at presentation are rare (0-6%), 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 tumours (T1-2) and tumours involving the fossa navicularis or the penile urethra have a better prognosis than higher stage tumours (>T2 or N+) and lesions involving the bulbomembranous urethra. In women, in addition to stage and location, the size of the tumour has also prognostic implications. While surgery and radiation therapy (RT) are of benefit in early stage disease, advanced stage PUC requires multimodal treatment strategies to optimise local control and survival. These include induction chemotherapy followed by surgery or RT and concurrent chemoradiation with or without surgery. The latter strategy has been used successfully to treat other human papillomavirus-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.
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Affiliation(s)
- Farshid Dayyani
- Division of Hematology and Oncology, Banner MD Anderson Cancer Center, Gilbert, AZ, USA; Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
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27
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Iborra F, Murez T, Millet I, Serre I, Poinas G, Thuret R. Les cancers primitifs de l’urètre. ONCOLOGIE 2014. [DOI: 10.1007/s10269-014-2387-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
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28
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[Radiation therapy in locally advanced and/or relapsed urological tumors]. Urologia 2014; 80:212-24. [PMID: 24526598 DOI: 10.5301/ru.2013.11501] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/20/2013] [Indexed: 11/20/2022]
Abstract
Radiation therapy (RT) plays a fundamental role in the treatment of locally advanced and/or relapsed urological tumors, as well as in palliation, or as definitive treatment, and even where integrated into a multi-modal approach. In operated renal tumors, positive margins or extracapsular extension show a positive impact of postoperative RT, with a reduction of relapses between 100% and 30%, while, in the case of palliation, treatments with RT at high doses are preferred. In advanced cancers of the upper urinary tract, RT plays a limited role, even if it seems to increase the level of disease control locally and, with the combination of cisplatin, survival rates too. An important reduction in the recurrence is also observed in locally advanced tumors of the urethra, with a recurrence of 60% after surgery, 36% after RT and 25% after pairing of the two. In locally advanced tumors of the penis, RT shows poorer results than surgery, and the addition of postoperative RT does not seem to add any further outcome, except where, in the presence of a positive inguinal dissection, the postoperative RT reduces lymph node recurrences by 60%-11%. Interesting data for the preservation of the organ are reported with reference to the combination with chemotherapy. In the tumors of the testis, it is still disputable whether the treatment of residual masses after chemotherapy may be appropriate, with a view to a possible salvage radiotherapy. In the treatment of the prostate, the role of RT is consolidated and evolving with the progress of dose escalation, the association with hormonal therapy, new technologies, new possibilities of IMRT and proton therapy and various studies on multi-modal approaches (hormone therapy, surgery, radiotherapy, chemotherapy). Cystectomy is the gold standard for the treatment of locally advanced bladder cancer, even though there is a revived interest in multimodal treatments (transurethral resection, chemotherapy, RT) that may allow the organ preservation. Postoperative radiotherapy, which can reduce by 50% to 20%-5% local recurrences that are highly correlated with distance failure and with survival, should be revised in the light of modern RT techniques that can further increase local control levels and reduce the toxicity significantly.
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29
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Gakis G. Editorial Comment to Docetaxel, cisplatin and 5-fluorouracil chemotherapy with concurrent radiation for unresectable advanced urethral carcinoma. Int J Urol 2013; 21:424-5. [DOI: 10.1111/iju.12334] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Affiliation(s)
- Georgios Gakis
- Department of Urology; University Hospital Tübingen; Tübingen Germany
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30
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Itoh J, Mitsuzuka K, Kimura S, Tanaka T, Yamamuro T, Yamashita S, Yamada S, Saito H, Kaiho Y, Arai Y. Docetaxel, cisplatin and 5-fluorouracil chemotherapy with concurrent radiation for unresectable advanced urethral carcinoma. Int J Urol 2013; 21:422-4. [DOI: 10.1111/iju.12333] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2013] [Accepted: 09/19/2013] [Indexed: 11/26/2022]
Affiliation(s)
- Jun Itoh
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Koji Mitsuzuka
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Shingo Kimura
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Takaki Tanaka
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Taku Yamamuro
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Shinichi Yamashita
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Shigeyuki Yamada
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Hideo Saito
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Yasuhiro Kaiho
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
| | - Yoichi Arai
- Department of Urology; Tohoku University School of Medicine; Sendai Japan
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31
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Radical chemoradiotherapy for urethral squamous cell carcinoma: two case reports and a review of the literature. Case Rep Urol 2013; 2013:194690. [PMID: 23738187 PMCID: PMC3664474 DOI: 10.1155/2013/194690] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2013] [Accepted: 04/15/2013] [Indexed: 01/09/2023] Open
Abstract
Primary urethral squamous cell carcinoma is rare. Its management is particularly challenging owing to the paucity of evidence from randomised trials to inform practice. We report two male and female cases of squamous cell carcinoma of the urethra, which were treated with concomitant cisplatin and radiotherapy. These cases add to the body of case reports that have shown benefit for concomitant chemoradiotherapy in urethral squamous cell carcinoma. They also illustrate that single agent chemotherapy, namely, cisplatin, may be used successfully with limited toxicities.
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32
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Gakis G, Witjes JA, Compérat E, Cowan NC, De Santis M, Lebret T, Ribal MJ, Sherif AM. EAU guidelines on primary urethral carcinoma. Eur Urol 2013; 64:823-30. [PMID: 23582479 DOI: 10.1016/j.eururo.2013.03.044] [Citation(s) in RCA: 113] [Impact Index Per Article: 9.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2013] [Accepted: 03/23/2013] [Indexed: 12/15/2022]
Abstract
CONTEXT The European Association of Urology (EAU) Guidelines Group on Muscle-Invasive and Metastatic Bladder Cancer prepared these guidelines to deliver current evidence-based information on the diagnosis and treatment of patients with primary urethral carcinoma (UC). OBJECTIVE To review the current literature on the diagnosis and treatment of patients with primary UC and assess its level of scientific evidence. EVIDENCE ACQUISITION A systematic literature search was performed to identify studies reporting urethral malignancies. Medline was searched using the controlled vocabulary of the Medical Subject Headings database, along with a free-text protocol. EVIDENCE SYNTHESIS Primary UC is considered a rare cancer, accounting for <1% of all malignancies. Risk factors for survival include age, tumour stage and grade, nodal stage, presence of distant metastasis, histologic type, tumour size, tumour location, and modality of treatment. Pelvic magnetic resonance imaging is the preferred method to assess the local extent of urethral tumour; computed tomography of the thorax and abdomen should be used to assess distant metastasis. In localised anterior UC, urethra-sparing surgery is an alternative to primary urethrectomy in both sexes, provided negative surgical margins can be achieved. Patients with locally advanced UC should be discussed by a multidisciplinary team of urologists, radiation oncologists, and oncologists. Patients with noninvasive UC or carcinoma in situ of the prostatic urethra and prostatic ducts can be treated with a urethra-sparing approach with transurethral resection and bacillus Calmette-Guérin (BCG). Cystoprostatectomy with extended pelvic lymphadenectomy should be reserved for patients not responding to BCG or as a primary treatment option in patients with extensive ductal or stromal involvement. CONCLUSIONS The 2013 guidelines document on primary UC is the first publication on this topic by the EAU. It aims to increase awareness in the urologic community and provide scientific transparency to improve outcomes of this rare urogenital malignancy.
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Affiliation(s)
- Georgios Gakis
- Department of Urology, Eberhard-Karls University, Tübingen, Germany.
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33
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Dayyani F, Pettaway CA, Kamat AM, Munsell MF, Sircar K, Pagliaro LC. Retrospective analysis of survival outcomes and the role of cisplatin-based chemotherapy in patients with urethral carcinomas referred to medical oncologists. Urol Oncol 2012; 31:1171-7. [PMID: 22534087 DOI: 10.1016/j.urolonc.2012.01.011] [Citation(s) in RCA: 51] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2011] [Revised: 12/30/2011] [Accepted: 01/23/2012] [Indexed: 12/20/2022]
Abstract
OBJECTIVES Primary carcinomas of the urethra (PCU) are rare and often advanced when diagnosed. Treatment standards are lacking. We studied treatment response and survival in a cohort of patients with PCU, with emphasis on modern platinum-containing chemotherapy regimens plus surgery for advanced disease. MATERIALS AND METHODS This was a retrospective chart review of consecutive patients with PCU seen by medical oncologists at our institution over a recent 5-year period. Outcome was measured as best response to chemotherapy. Kaplan-Meier estimates were generated for survival and Cox proportional hazard was used for prognostic factors for survival. RESULTS The 44 patients (64% women) included had a median age at diagnosis of 66.5 years. The most prevalent histologic subtypes of PCU were squamous cell carcinoma and adenocarcinoma. At diagnosis, 43% already had lymph node-positive [lymph node (LN)+] disease, and 16% had distant metastases. The entire cohort's overall survival (OS) was 31.7 months. The response rate to platinum-containing neoadjuvant chemotherapy was 72%. Twenty-one patients with locally advanced or LN+ PCU underwent chemotherapy plus surgery. Their median OS from chemotherapy initiation was 25.6 months. Four of 9 patients (44%) with LN+ PCU at diagnosis were alive at our review, with a minimum follow-up of more than 3 years. CONCLUSIONS Modern platinum-containing regimens appear to be effective in advanced PCU. Preoperative chemotherapy is associated with prolonged disease-free survival in a subgroup of LN+ cases.
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Affiliation(s)
- Farshid Dayyani
- Department of Genitourinary Medical Oncology, Unit 1374, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030-3721, USA
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Reis LO, Ferreira F, Almeida M, Ferreira U. Urethral carcinoma: critical view on contemporary consecutive series. Med Oncol 2011; 28:1405-1410. [PMID: 20596803 DOI: 10.1007/s12032-010-9609-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2010] [Accepted: 06/18/2010] [Indexed: 10/19/2022]
Abstract
Urethral carcinoma is a rare cancer of the urinary tract. Although most cases are of squamous cell carcinomas, there are several reports in the literature about transitional cell carcinoma and adenocarcinomas whose origin remains controversial. While the diagnosis of this condition is essentially clinical, magnetic resonance imaging (MRI) is the examination of choice for its staging, and a pathological confirmation is still necessary. There is no consensus on the treatment of these tumors because of its low incidence. The following therapeutic options are currently available: surgical resection (often for small and distal urethral tumors); radiotherapy and chemotherapy, for larger tumors, seeking a functional preservation of the penis, bladder and/or vagina; or a combination of these therapies thereof in case of more extensive tumors. The classic treatment involves surgery aiming loco-regional disease control, and continent urinary derivation provides satisfactory functional results in cases of radical urethrectomy. Much still needs to be learned about urethral cancer, and molecular diagnostics tools and therapeutic targets are promissory. The key to better understanding urethral cancer is the pooling of data from a wide range of sources including international consortia.
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Affiliation(s)
- Leonardo Oliveira Reis
- Division of Urologic Oncology, University of Campinas (UNICAMP), R. Votorantim, 51, ap. 43, Campinas-SP, 13073-090, Brazil.
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Memon S, Craig Lynch A, Cleeve L, Murphy DG, Pohl MJ, Heriot AG. Squamous cell carcinoma of the bulbar urethra. J Clin Oncol 2011; 29:e733-5. [PMID: 21876078 DOI: 10.1200/jco.2011.36.5890] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Affiliation(s)
- Sameer Memon
- Peter MacCallum Cancer Centre, East Melbourne, Victoria, Australia
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Mitsuzuka K, Saito H, Arai Y. Primary invasive squamous cell carcinoma of male bulbar urethra completely responded to concurrent chemoradiation therapy. Int J Urol 2011; 18:675-6. [PMID: 21736628 DOI: 10.1111/j.1442-2042.2011.02813.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
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Wood HM, Angermeier KW. Anatomic Considerations of the Penis, Lymphatic Drainage, and Biopsy of the Sentinel Node. Urol Clin North Am 2010; 37:327-34. [DOI: 10.1016/j.ucl.2010.04.013] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Abstract
Urethral cancer is a rare but aggressive neoplasm. Early-stage distal lesions can be successfully treated with a single modality. Results for definitive radiotherapy using either or both external beam radiation therapy and brachytherapy have shown excellent cure rates in men and women. The primary advantage of radiotherapy is organ preservation. Advanced tumors, however, have poor outcomes with single modality treatment. Results have been improved using a combination of radiotherapy and chemotherapy, chiefly 5-fluorouracil and mitomycin C. Although literature is limited to case reports because of the rarity of the disease, the markedly improved results compared with older results of surgery with or without radiation warrant consideration.
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Affiliation(s)
- Bridget F Koontz
- Department of Radiation Oncology, Duke University Medical Center, Durham, NC 22710, USA.
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Abstract
Urethral cancer is an infrequent pathology, less than 1% of the genitourinary tumors. It is more frequent in women (4:1), in the sixth or seventh decade of life. The most frequent histology being squamous cell carcinoma. First signs and symptoms usually are more attributable to benign stricture disease, rather than malignicy. The interval between the onset of symptoms and diagnosis may be as long as three years. Therefore most of these tumors are locally advanced at the time of diagnosis with generally poor prognosis despite aggressive treatment. Therapeutic management varies with the stage and location of the lesion. Because of the rarity of this pathology, no consensus has been reached on treatment modalities, but seems to be that must be a multimodal one, including surgery, radiotherapy and chemotherapy. We present the case of an 80 year-old male, with a diagnosis of urethral squamous-cell cancer, locally advanced at the time of diagnosis. Surgery was not feasible. The patient underwent chemotherapy and radiotherapy with evidence of quick progression thereafter.
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Iborra F, Rigaud J, Bastide C, Mottet N. [Treatment of primary urethral carcinoma. Guidelines from the French Urological Association. Cancer committee]. Prog Urol 2009; 19:170-5. [PMID: 19268254 DOI: 10.1016/j.purol.2008.12.003] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2008] [Revised: 12/05/2008] [Accepted: 12/11/2008] [Indexed: 11/24/2022]
Abstract
The litterature dealing with the treatment of primary uretral carcinoma is very limited. Most of it is based on small series, case report or expert opinions. These guidelines are level IV. The treatment modality is mainly based on the lesion topography and not on the histology. For anterior T1 or 2 lesions, surgery is the most often used modality. In women, radiotherapy might be an attractive option. For more advanced lesions, the combination of radiotherapy and chemotherapy is the standard of care. The optimal protocol remains to be defined. Intradiverticular lesions in women are mainly adenocarcimoma. Surgery only is often inadequate.
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Affiliation(s)
- F Iborra
- Polyclinique Saint-Roch, Montpellier, France
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