A 60-year-old woman came to the Emergency Department complaining of a vaginal formation. The urologist suspected a urethral caruncle: the patient was discharged with vaginal oestrogen cream to relieve symptoms and a follow-up was suggested. After two months the patient returned to the Emergency Department since the mass was increasing in volume and complaining of dysuria and haematuria. Ultrasound, contrast-enhanced computed tomography, and contrast-enhanced magnetic resonance revealed a mass arising from the mucosa and involving the vulva and the urethra, suspicious of malignancy. We present a challenging diagnosis of an infiltrative and rapidly progressive primary vulval amelanotic melanoma with a complete imaging evaluation and a confirmed histological diagnosis.

• Klíčová slova
• AM, Amelanotic melanoma, Amelanotic melanoma vulva, CT, Diagnostic procedure, Gynecologic tumor, MRI, PVAM, Vulval melanoma, Vulvar cancer,
• MeSH
• amelanotický melanom * diagnóza   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory močové trubice * diagnóza   patologie   MeSH
• nádory vulvy * patologie   diagnóza   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

INTRODUCTION: The optimal management of the urethra in patients planned for radical cystectomy (RC) remains unclear. We sought to evaluate the impact of urethrectomy on perioperative and oncological outcomes in patients treated with RC for non-metastatic urothelial carcinoma of the bladder (UCB). MATERIALS AND METHODS: We assessed the retrospective data from patients treated with RC for UCB of five European University Hospitals. Associations of urethrectomy with progression-free (PFS), cancer-free (CSS), and overall (OS) survivals were assessed in univariable and multivariable Cox regression models. We performed a subgroup analysis in patients at high risk for urethral recurrence (UR) (urethral invasion and/or bladder neck invasion and/or multifocality and/or prostatic urethra involvement). RESULTS: A total of 887 non-metastatic UCB patients were included. Among them, 146 patients underwent urethrectomy at the time of RC. Urethrectomy was performed more often in patients with urethral invasion, T3/4 tumor stage, CIS, positive frozen section analysis of the urethra, and those who received neoadjuvant chemotherapy, underwent robotic RC, and/or received an ileal conduit urinary diversion (all p < 0.001). Estimated blood loss and the postoperative complication rate were comparable between patients who received an urethrectomy and those who did not. Urethrectomy during RC was not associated with PFS (HR 0.83, p = 0.17), CSS (HR 0.93, p = 0.67), or OS (HR 1.08, p = 0.58). In the subgroup of 276 patients at high risk for UR, urethrectomy at the time of RC decreased the risk of progression (HR 0.58, p = 0.04). CONCLUSION: In our study, urethrectomy at the time of RC seems to benefit only patients at high risk for UR. Adequate risk assessment of UCB patients' history may allow for better clinical decision-making and patient counseling.

• Klíčová slova
• Bladder cancer, Radical cystectomy, Urethral recurrence, Urethrectomy,
• MeSH
• cystektomie MeSH
• karcinom z přechodných buněk * patologie   chirurgie   MeSH
• lidé MeSH
• močový měchýř chirurgie   MeSH
• nádory močové trubice * patologie   chirurgie   MeSH
• nádory močového měchýře * patologie   chirurgie   MeSH
• retrospektivní studie MeSH
• uretra patologie   chirurgie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Spindle cell lipoma, cellular angiofibroma and mammary myofibroblastoma are mesenchymal tumours that have overlapping morphological and immunophenotypic features. Aberrations in chromosome 13q14 have been identified as a recurrent feature. We report a unique case of a 69-year-old woman who metachronously developed all three tumours. She developed a peri-urethral and a recurrent peri-vaginal cellular angiofibroma at age 54 and 57, respectively, a spindle cell lipoma at age 62 and a mammary myofibroblastoma at age 69. Dual-colour interphase fluorescent in situ hybridisation (FISH) revealed losses of RB1 and FOXO1 (13q14LOH [loss of heterozygosity]) within neoplastic cells. There was also loss of retinoblastoma (Rb) protein expression. To our knowledge, this is the first report of these three tumours arising in the same patient. The genetic link between these tumours supports the hypothesis that they may arise from the same progenitor cells. However, further research is required to elucidate the precise pathogenetic link.

• Klíčová slova
• Cellular angiofibroma, FISH, Fluorescence in situ hybridisation, Mammary myofibroblastoma, RB1, Spindle cell lipoma,
• MeSH
• angiofibrom genetika   patologie   MeSH
• fenotyp MeSH
• forkhead box protein O1 genetika   MeSH
• genetická predispozice k nemoci MeSH
• hybridizace in situ fluorescenční MeSH
• lidé MeSH
• lidské chromozomy, pár 14 * MeSH
• lipom genetika   patologie   MeSH
• nádorové biomarkery genetika   MeSH
• nádory močové trubice genetika   patologie   MeSH
• nádory prsu genetika   patologie   MeSH
• nádory vaginy genetika   patologie   MeSH
• nádory ze svalové tkáně genetika   patologie   MeSH
• sekundární malignity genetika   patologie   MeSH
• senioři MeSH
• ubikvitinligasy genetika   MeSH
• vazebné proteiny retinoblastomu genetika   MeSH
• ztráta heterozygozity * MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• forkhead box protein O1 MeSH
• FOXO1 protein, human MeSH Prohlížeč
• nádorové biomarkery MeSH
• RB1 protein, human MeSH Prohlížeč
• ubikvitinligasy MeSH
• vazebné proteiny retinoblastomu MeSH

PURPOSE: We assessed contemporary incidence rates and trends of primary urethral cancer. METHODS: We identified urethral cancer patients within Surveillance, Epidemiology and End Results registry (SEER, 2004-2016). Age-standardized incidence rates per 1,000,000 (ASR) were calculated. Log linear regression analyses were used to compute average annual percent change (AAPC). RESULTS: From 2004 to 2016, 1907 patients with urethral cancer were diagnosed (ASR 1.69; AAPC: -0.98%, p = 0.3). ASR rates were higher in males than in females (2.70 vs. 0.55), respectively and did not change over the time (both p = 0.3). Highest incidence rates were recorded in respectively ≥75 (0.77), 55-74 (0.71) and ≤54 (0.19) years of age categories, in that order. African Americans exhibited highest incidence rate (3.33) followed by Caucasians (1.72), other race groups (1.57) and Hispanics (1.57), in that order. A significant decrease occurred over time in Hispanics, but not in other race groups. In African Americans, male and female sex-stratified incidence rates were higher than in any other race group. Urothelial histological subtype exhibited highest incidence rate (0.92), followed by squamous cell carcinoma (0.41), adenocarcinoma (0.29) and other histologies (0.20). In stage stratified analyses, T1N0M0 stage exhibited highest incidence rate. However, it decreased over time (-3.00%, p = 0.02) in favor of T1-4N1-2M0 stage (+ 2.11%, p = 0.02). CONCLUSION: Urethral cancer is rare. Its incidence rates are highest in males, elderly patients, African Americans and in urothelial histological subtype. Most urethral cancer cases are T1N0M0, but over time, the incidence of T1N0M0 decreased in favor of T1-4N1-2M0.

• Klíčová slova
• Histology, Incidence rate, Race, Region, Time trend, Urethral cancer,
• MeSH
• adenokarcinom diagnóza   epidemiologie   MeSH
• dospělí MeSH
• incidence MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory močové trubice diagnóza   epidemiologie   patologie   MeSH
• program SEER trendy   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• sexuální faktory MeSH
• spinocelulární karcinom diagnóza   epidemiologie   MeSH
• stupeň nádoru MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Primary urethral adenocarcinomas are very rare neoplasms accounting for <10% of all urethral carcinomas. Site of their origin is unclear, but they seem to arise from Skene's paraurethral glands, which is the female homologue of the male prostate. The aim of this article is to report the first case of Skene's gland adenocarcinoma in which a molecular genetic profiling was performed. The patient was a 73-year-old woman with a polypoid lesion sized 3 × 2 cm located at the interface between the bladder neck and the proximal urethra. Transurethral resection was performed and small tissue fragments with positive margins were obtained. Histology revealed an epithelial neoplasm consisting of cribriform structures located in the subepithelial connective tissue of the bladder wall and proximal urethra. The lesion showed positive immunohistochemical staining with prostate specific antigen, prostatic acid phosphatase, NKX3.1, and alpha-methylacyl-CoA racemase. Using the Illumina TruSight Tumor 170 next-generation sequencing assay, a mutation and loss of heterozygosity of the phosphatase and tensin homologue (PTEN) gene was detected. No fusion in any of the examined genes was found using this assay as well as FusionPlex Solid Tumor Kit and FusionPlex Sarcoma kit assays from ArcherDX. Given the rarity of Skene's gland adenocarcinoma, it is uncertain whether the same grading and prognostic criteria that are currently used for prostatic cancer apply here as well. It is also unclear what treatment strategy should be applied, but according to the available literature, it seems that local excision or wide surgical resection could represent sufficient therapeutic modalities.

• Klíčová slova
• PTEN gene, Skene’s gland adenocarcinoma, molecular genetics, next-generation sequencing, paraurethral glands,
• MeSH
• adenokarcinom diagnóza   genetika   patologie   MeSH
• fosfohydroláza PTEN genetika   MeSH
• lidé MeSH
• mutace MeSH
• nádorové biomarkery genetika   MeSH
• nádory močové trubice diagnóza   genetika   patologie   MeSH
• senioři MeSH
• uretra patologie   MeSH
• ztráta heterozygozity MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• fosfohydroláza PTEN MeSH
• nádorové biomarkery MeSH
• PTEN protein, human MeSH Prohlížeč

OBJECTIVE: To test the effect of race/ethnicity on histological subtype, stage at presentation, and cancer specific mortality (CSM) in urethral cancer patients. MATERIAL AND METHODS: Stratified analyses (Surveillance, Epidemiology and End Results [2004-2016]) tested the effect of race/ethnicity on histology and stage. Cumulative incidence-plots and multivariable competing-risks regression models (CRR), addressed CSM, after matching for TNM-stage, histology, age, and gender. RESULTS: Of 1,904 urethral cancer patients, 71% were Caucasian, 16% African American, 7% Hispanic and 5% other. African Americans were younger (66 years) than Caucasians (73 years) and Hispanics (74 years). In African Americans, adenocarcinoma (25%) and squamous cell carcinoma (SCC; 29%) were more frequent than in Caucasians (12% and 23%) or Hispanics (15% and 20%). African Americans with adenocarcinoma exhibited higher stage than other adenocarcinoma patients. In CRR, African Americans (35%) and Hispanics (29%) exhibited highest and second highest 3-year CSM, even after matching. After further multivariable adjustment of matched CRRs, CSM was higher in Hispanics (HR: 1.93, P= 0.03) and in African Americans (Hazard ratio 1.35, P= 0.07), relative to Caucasians. CONCLUSION: Race/ethnicity impacts important differences on urethral cancer patients. African American race/ethnicity predisposes to higher rate of SCC and adenocarcinoma. Moreover, African Americans are younger and present with higher stage at diagnoses. Finally, even after most detailed matching for stage, age, gender, and adjustment for treatment and systemic therapy and socioeconomic status, African Americans and Hispanics exhibit higher CSM than Caucasians.

• Klíčová slova
• African American, Hispanic, Mortality, Race, SEER database, Urethral cancer,
• MeSH
• běloši statistika a číselné údaje   MeSH
• černoši nebo Afroameričané statistika a číselné údaje   MeSH
• Hispánci a Latinoameričané statistika a číselné údaje   MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití MeSH
• nádory močové trubice klasifikace   mortalita   patologie   MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• staging nádorů MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: To evaluate the impact of salvage therapy (ST) on overall survival (OS) in recurrent primary urethral cancer (PUC). PATIENTS: A series of 139 patients (96 men, 43 women; median age = 66, interquartile range: 57-77) were diagnosed with PUC at 10 referral centers between 1993 and 2012. The modality of ST of recurrence (salvage surgery vs. radiotherapy) was recorded. Kaplan-Meier analysis with log-rank was used to estimate the impact of ST on OS (median follow-up = 21, interquartile range: 5-48). RESULTS: The 3-year OS for patients free of any recurrence (I), with solitary or concomitant urethral recurrence (II), and nonurethral recurrence (III) was 86.5%, 74.5%, and 48.2%, respectively (P = 0.002 for I vs. III and II vs. III; P = 0.55 for I vs. II). In the 80 patients with recurrences, the modality of primary treatment of recurrence was salvage surgery in 30 (37.5%), salvage radiotherapy (RT) in 8 (10.0%), and salvage surgery plus RT in 5 (6.3%) whereas 37 patients did not receive ST for recurrence (46.3%). In patients with recurrences, those who underwent salvage surgery or RT-based ST had similar 3-year OS (84.9%, 71.6%) compared to patients without recurrence (86.7%, P = 0.65), and exhibited superior 3-year OS compared to patients who did not undergo ST (38.0%, P<0.001 compared to surgery, P = 0.045 to RT-based ST, P = 0.29 for surgery vs. RT-based ST). CONCLUSIONS: In this study, patients who underwent ST for recurrent PUC demonstrated improved OS compared to those who did not receive ST and exhibited similar survival to those who never developed recurrence after primary treatment.

• Klíčová slova
• Primary urethral carcinoma, Radiotherapy, Recurrence, Salvage, Surgery,
• MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru patologie   MeSH
• míra přežití MeSH
• nádory močové trubice mortalita   patologie   radioterapie   MeSH
• prognóza MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• záchranná terapie metody   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

PURPOSE: To evaluate risk factors for survival in a large international cohort of patients with primary urethral cancer (PUC). METHODS: A series of 154 patients (109 men, 45 women) were diagnosed with PUC in ten referral centers between 1993 and 2012. Kaplan-Meier analysis with log-rank test was used to investigate various potential prognostic factors for recurrence-free (RFS) and overall survival (OS). Multivariate models were constructed to evaluate independent risk factors for recurrence and death. RESULTS: Median age at definitive treatment was 66 years (IQR 58-76). Histology was urothelial carcinoma in 72 (47 %), squamous cell carcinoma in 46 (30 %), adenocarcinoma in 17 (11 %), and mixed and other histology in 11 (7 %) and nine (6 %), respectively. A high degree of concordance between clinical and pathologic nodal staging (cN+/cN0 vs. pN+/pN0; p < 0.001) was noted. For clinical nodal staging, the corresponding sensitivity, specificity, and overall accuracy for predicting pathologic nodal stage were 92.8, 92.3, and 92.4 %, respectively. In multivariable Cox-regression analysis for patients staged cM0 at initial diagnosis, RFS was significantly associated with clinical nodal stage (p < 0.001), tumor location (p < 0.001), and age (p = 0.001), whereas clinical nodal stage was the only independent predictor for OS (p = 0.026). CONCLUSIONS: These data suggest that clinical nodal stage is a critical parameter for outcomes in PUC.

• Klíčová slova
• Clinical, Nodal stage, Primary urethral carcinoma, Prognostic, Risk factors, Survival,
• MeSH
• adenokarcinom mortalita   patologie   terapie   MeSH
• Kaplanův-Meierův odhad MeSH
• karcinom z přechodných buněk mortalita   patologie   terapie   MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití MeSH
• nádory močové trubice mortalita   patologie   terapie   MeSH
• přežití bez známek nemoci MeSH
• prognóza MeSH
• proporcionální rizikové modely MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• spinocelulární karcinom mortalita   patologie   terapie   MeSH
• staging nádorů MeSH
• věkové faktory MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH

• MeSH
• karcinom patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• melanom patologie   MeSH
• nádor z thekálních buněk patologie   MeSH
• nádory močové trubice patologie   chirurgie   MeSH
• papilární karcinom patologie   MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH