Ewing-like sarcoma bladder primary tumour: A case report and literature review.

Bladder primitive neuroectodermal tumors are extremely rare but are most frequent in older adult. 59-year-old man that complained of hematuria for the previous 24 h, urethral syndrome, and pain in the right renal fossa over the previous two weeks. No definitive management or treatment guidelines have been established. Hematuria is the most frequent symptom. Advanced age, metastasis, and incomplete tumor resection are determinants of a poor prognosis. Ewing-like bladder primary tumor is a rare entity with a poor prognosis, hence an aggressive treatment combining surgery and chemotherapy must be considered from the beginning.

High-flow priapism in pediatric population: Case series and review of the literature.

INTRODUCTION: Priapism is a prolonged erection that lasts longer than four hours. It is a rare pathology in the pediatric population, with an estimation of 0.3-1.5 per 100,000 children per year. The diagnostic sequence includes clinical history, physical examination and penile Doppler ultrasound (PDUS). Puncture of corpora cavernosa is not always necessary to establish the differential diagnosis between high-flow and low-flow priapism. The treatment of choice in pediatric age is not well defined. PATIENTS AND METHODS: Multicentric, retrospective and descriptive study including patients under 14 years with high-flow priapism between 2010 and 2020. RESULTS: A total of seven patients were diagnosed with high-flow priapism. None of them required puncture of the corpora cavernosa. Patients were treated with a conservative management, two patients required superselective arterial embolization due to persistent symptoms. CONCLUSIONS: High-flow priapism is a very rare entity in pediatric age; therefore, knowing the proper diagnosis and management is crucial. Currently, penile doppler ultrasound is enough for diagnosis in most cases and allows obviating the use of blood gas analysis. Children should be initially treated with a conservative management, reserving embolization for refractory cases.

High-flow Priapism in Pediatric Population: Case Series and Review of the Literature. / Priapismo de alto flujo en población pediátrica: serie de casos y revisión de la literatura.

INTRODUCTION: Priapism is a prolonged erection that lasts longer than four hours. It is a rare pathology in the pediatric population, with an estimation of 0.3-1.5 per 100,000 children per year. The diagnostic sequence includes clinical history, physical examination and penile Doppler ultrasound (PDUS). Puncture of corpora cavernosa is not always necessary to establish the differential diagnosis between high-flow and low-flow priapism. The treatment of choice in pediatric age is not well defined. PATIENTS AND METHODS: Multicentric, retrospective and descriptive study including patients under 14 years with high-flow priapism between 2010 and 2020. RESULTS: A total of seven patients were diagnosed with high-flow priapism. None of them required puncture of the corpora cavernosa. Patients were treated with a conservative management, two patients required superselective arterial embolization due to persistent symptoms. CONCLUSIONS: High-flow priapism is a very rare entity in pediatric age; therefore, knowing the proper diagnosis and management is crucial. Currently, penile doppler ultrasound is enough for diagnosis in most cases and allows obviating the use of blood gas analysis. Children should be initially treated with a conservative management, reserving embolization for refractory cases.

[TVA and TOA. New adjustable mesh for the treatment of female stress incontinence. Preliminaries results]. / TVA y TOA. Nuevas mallas ajustables de tensión en el tratamiento de la incontinencia urinaria de esfuerzo. Resultados preliminares.

OBJECTIVES: Evaluation of a new mesh for incontinence (TVA/TOA) which enables the degree of tension applied during surgery to be readjusted at the post-operative stage. PATIENTS AND METHOD: 62 patients treated with the TVA mesh and monitored over a 14-month period (SD 7.8, range 6-38). In 33 patients (53%) some other pelvic prolapse was corrected. Evaluation was carried out by clinical report, examination of bladder full of 250 saline solution, flowmetry and urinary residue. 40 patients filled in 4 quality of life questionnaires (QoL; ICIQ-SF; PGI-S; PGI-I). RESULTS: 42 patients were found to be objectively continent in the post-operative evaluation. Of these, it was necessary to reduce tension in 7 cases (11%) due to urinary obstruction (flow < 10 ml/sec and/or residue). The tension of the mesh was tightened in 20 patients (32%) due to continue with a certain degree of incontinence. All patients were discharged as continent and with no residue. In the last revision, 58 patients (93%) proved to be objectively continent and 4 (6.5%) showed a notable improvement in their incontinence. The Q(MAX) is 19.8 ml/sec (SD 9.8). The mictional urgency had disappeared or improved in 32 of the patients who had this prior to operation (76%) and had appeared in 3 of the patients who didn't (15%). The clinical report showed a high level of consensus with the ICIQ-SF survey (Kappa = 0.89) regarding stress incontinence, diminishing clearly (Kappa= 0.13) when urge incontinence was taken into account. 34 (85%) patients scored over 95 out of 110 in the QoL. 30 (75%) scored less than 6 in ICIQ-SF. 32 (80%) showed a perception of normality and 4 (10%) slight illness in the PGI-S. In the PGI-I 29 (72.5%) were much better and 11 (27.5%) quite a lot better. A relation exists between urgency and dismissed quality of life. CONCLUSION: With the TVA (trans-vaginal adjustable) mesh it is possible to adjust the tension originally applied during surgery at the post-operative stage, so that any defects or excesses can be corrected.

TVA y TOA. Nuevas mallas ajustables de tensión en el tratamiento de la incontinencia urinaria de esfuerzo. Resultados preliminares / TVA and TOA. New adjustable mesh for the treatment of female stress incontinence preliminaries resulted

Objetivos: Valoración de una nueva malla de incontinencia (TVA/TOA) que permite postoperatoriamente ajustar la tensión dada en quirófano. Pacientes y Métodos: 62 pacientes tratados con la malla TVA, seguimiento medio de 14 meses (DS 7,8, rango 6-38). En 33 pacientes (53%) se añadió corrección de algún prolapso. La valoración se ha realizado mediante historia clínica, exploración con vejiga llena con 250 cc de suero salino, flujometría y residuo. 40 pacientes han rellenado 4 cuestionarios de calidad de vida. (QoL; ICIQ-SF; PGI-S y PGI-I). Resultados: 42 pacientes eran continentes en la valoración post-operatoria. De estos, fue necesario disminuir la tensión en 7 (11%) por obstrucción urinaria (flujo< 10 ml/seg y/o residuo). En 20 pacientes (32%) se aumentó la tensión por continuar algún grado de incontinencia. Todos fueron dados de alta continentes y sin residuo. En la última revisión, 58 (93%) son totalmente continentes y 4 (6,5%) han mejorado notablemente su incontinencia. El QMAX es 19,8 ml/s (DS 9,8). La urgencia miccional ha desaparecido o mejorado en 32 (76%) de los pacientes que la presentaban preoperatoriamente y ha aparecido en 3 (15%) de los que no la presentaban. La historia clínica muestra un alto grado de concordancia con el cuestionario ICIQ-SF (Kappa= 0,89) en cuanto a incontinencia de esfuerzo se refiere, disminuyendo ostensiblemente (Kappa= 0,13) cuando se toma en consideración la incontinencia por urgencia. De los 40 pacientes que completaron los cuestionarios de calidad de vida, 34 (85%) tienen una puntuación superior a 95 sobre 110 en el QoL. 30 (75%) tiene una puntuación inferior a 6 en ICIQ-SF. 32 (80%) tienen una percepción de normalidad y 4 (10%) de enfermedad leve en el PGI-S. En el PGI-I 29 (72,5%) están mucho mejor y 11 (27,5%) bastante mejor. Existe relación directa entre urgencia miccional y pérdida de calidad de vida. Conclusión: La malla TVA (transvaginal ajustable) permite ajustar la tensión dada en quirófano, permitiendo corregir los defectos y los excesos

Objectives: Evaluation of a new mesh for incontinence (TVA/TOA) which enables the degree of tension applied during surgery to be readjusted at the post-operative stage. Patients and Method: 62 patients treated with the TVA mesh and monitored over a 14-month period (SD 7.8, range 6-38). In 33 patients (53%) some other pelvic prolapse was corrected. Evaluation was carried out by clinical report, examination of bladder full of 250 saline solution, flowmetry and urinary residue. 40 patients filled in 4 quality of life questionnaires (QoL; ICIQ-SF; PGI-S; PGI-I). Results: 42 patients were found to be objectively continent in the post-operative evaluation. Of these, it was necessary to reduce tension in 7 cases (11%) due to urinary obstruction (flow< 10 ml/sec and/or residue). The tension of the mesh was tightened in 20 patients (32%) due to continue with a certain degree of incontinence. All patients were discharged as continent and with no residue. In the last revision, 58 patients (93%) proved to be objectively continent and 4 (6.5%) showed a notable improvement in their incontinence. The QMAX is 19.8 ml/sec (SD 9.8). The mictional urgency had disappeared or improved in 32 of the patients who had this prior to operation (76%) and had appeared in 3 of the patients who didn’t (15%). The clinical report showed a high level of consensus with the ICIQ-SF survey (Kappa = 0.89) regarding stress incontinence, diminishing clearly (Kappa= 0.13) when urge incontinence was taken into account. 34 (85%) patients scored over 95 out of 110 in the QoL. 30 (75%) scored less than 6 in ICIQ-SF. 32 (80%) showed a perception of normality and 4 (10%) slight illness in the PGI-S. In the PGI-I 29 (72.5%) were much better and 11 (27.5%) quite a lot better. A relation exists between urgency and dismissed quality of life. Conclusion: With the TVA (trans-vaginal adjustable) mesh it is possible to adjust the tension originally applied during surgery at the post-operative stage, so that any defects or excesses can be corrected

[Survival prognostic factors valuation on a series of 202 patients with surgical treatment of renal cell carcinoma]. / Valoración de factores pronósticos de la supervivencia en una serie de 202 pacientes intervenidos por carcinoma de células renales.

OBJECTIVES: To describe renal cell carcinoma prognostic factors and set up the relationship with survival rates in this neoplasm. Likewise we show epidemiologic, clinical, diagnosis and therapeutic facts. MATERIAL AND METHOD: We review 202 patients underwent surgical treatment for renal cell carcinoma and the following features were recorded: gender, age and presenting symptoms, especially incidentally discovered tumors; tumor-related factors like TNM tumor stage, tumor grade and venous involvement: therapy-related recorded were surgical techniques and cytokine-based therapy. RESULTS: 60% of the patients showed organ-confined disease, 10% of patients with renal cell carcinoma presented with nodal positive disease and 7% with systemic metastases. 42% of patients presenting incidental tumor, with survival rates substantially better than that for symptomatic patients. 42% of patients with nodal positive disease presented systemic metastases at diagnosis, and 30% at surveillance. Systemic metastases presented a particularly poor prognosis for patients with renal cell carcinoma, with 12-months survival rates that 0%. Patients with cytokine-related therapy for metastatic disease presented 24-months survival rates that 20%. CONCLUSIONS: Renal cell carcinoma remains a major source of mortality, basically at advanced disease (nodal positive disease or systemic disease), without a clear improvement of survival rates despite the newer therapy modalities.

[Urologic findings in pseudoxanthoma elasticum: report one case]. / Manifestaciones urológicas del pseudoxantoma elástico: a propósito de un caso.

CASE REPORT: A 21-year-old woman presented with retinal angioid streaks and yellowish streak skin abnormalities in neck and axillary folds. Skin biopsy showed bluish-gray tangled masses of calcified elastic fibers in the mid- to lower dermis suggestive of Pseudoxanthoma elasticum (PXE). She consulted in Urology Department for lower urinary tract infection. Renal ultrasonography revealed multiple highly reflective foci in the corticomedullary junction. COMMENT: Pseudoxanthoma elasticum is a rare genetic disorder characterised by fragmentation and calcification of elastic fibers in the skin and media of arteries. Frequent manifestations include hypertension, angina pectoris, transient cerebral ischemic attacks, intermittent claudication, upper gastrointestinal bleeding, retinal angioid streaks and thickened skin. A characteristic appearance of highly reflective foci in the renal parenchyma have been reported in patients with PXE, but it's not specific for this syndrome. However, the presence of this structural pattern in a young patient with dermatological abnormalities should lead to the consideration of PXE in the differential diagnosis list. Besides ultrasonography findings, only a ureteral disruption case in a patient underwent ureteroscopic manipulation has been described to our knowledge. In our case, the true significance of these disease in recurrent urinary tract infection is uncertain.

Valoración de factores pronósticos de la supervivencia en una serie de 202 pacientes intervenidos por carcinoma de células renales / Survival prognostic factors valuation on a series of 202 patients with surgical treatment of renal cell carcinoma

Objetivos: Describir los principales factores pronósticos del carcinoma renal y estudiar su relación con la supervivencia especifica. Recoger datos demográficos, clínicos, diagnósticos y terapéuticos de los pacientes. Material y métodos: Se revisaron 202 casos de carcinomas renales operados entre 1988-2003. Las variables valoradas respecto al paciente fueron: edad, sexo y presentación clínica. Se investigó la importancia del incidentaloma. Con respecto al tumor se valoró: estadio TNM, grado histológico, tamaño y afectación venosa. Con respecto al tratamiento: principales vías de abordaje y técnicas quirúrgicas y terapia adyuvante inmunoterápica. Resultados: Un 60% de los pacientes presentó enfermedad órgano confinada, un 10% afectación nodal y un 7% enfermedad metastásica. Un 42% se presentaron incidentalmente, con una significativa mejor supervivencia respecto a tumores sintomáticos. Un 42% de los pacientes pN+ presentaron metástasis a distancia al diagnóstico y un tercio las desarrollaron durante el seguimiento. La supervivencia de los pacientes metastásicos sin tratamiento adyuvante fue inferior al año, mientras que un 20% de los tratados con inmunoterapia permanecían vivos a los 2 años. Conclusiones: El carcinoma renal es un tumor con alta mortalidad, fundamentalmente en sus estadios más avanzados (pN+ o pM+). Pese a las nuevas modalidades de tratamiento, no se ha logrado mejorar sustancialmente la supervivencia (AU)

Objectives: To describe renal cell carcinoma prognostic factors and set up the relationship with survival rates in this neoplasm. Likewise we show epidemiologic, clinical, diagnosis and therapeutic facts. Material and method: We review 202 patients underwent surgical treatment for renal cell carcinoma and the following features were recorded: gender, age and presenting symptoms, especially incidentally discovered tumors; tumor-related factors like TNM tumor stage, tumor grade and venous involvement; therapy-related recorded were surgical techniques and cytokine-based therapy. Results: 60% of the patients showed organ-confined disease, 10% of patients with renal cell carcinoma presented with nodal positive disease and 7% with systemic metastases. 42% of patients presenting incidental tumor, with survival rates substantially better than that for symptomatic patients. 42% of patients with nodal positive disease presented systemic metastases at diagnosis, and 30% at surveillance. Systemic metastases portend a particularly poor prognosis for patients with renal cell carcinoma, with 12-months survival rates that0%. Patients with cytokine-related therapy for metastatic disease presented 24-months survival rates that 20%.Conclusions: Renal cell carcinoma remains a major source of mortality, basically at advanced disease (nodal positive disease or systemic disease), without a clear improvement of survival rates despite the newer therapy modalities (AU)

Manifestaciones urológicas del pseudoxantoma elástico: a propósito de un caso / Urologic findings in pseudoxanthoma elasticum: report one case

Caso clínico: Mujer de 21 años con estrías angioides retinianas y estrías cutáneas en axilas y cuello. Tras biopsia cutánea con calcificaciones en las fibras elásticas de dermis, fue diagnosticada de Pseudoxantoma elástico. Remitida al Servicio de Urología por infección del tracto urinario inferior, en estudio ecográfico renal presentó múltiples focos hiperecogénicos de pequeño tamaño en la unión córtico-medular. Comentarios: El Pseudoxantoma elástico es una rara enfermedad genética que se caracteriza por la fragmentación y calcificación de las fibras elásticas de la piel y arterias. Las manifestaciones clínicas incluyen hipertensión, angor pectoris, ictus, claudicación intermitente, hemorragia digestiva alta, estrías angioides retinianas y estrías cutáneas. La ecografía se caracteriza por la presencia de múltiples focos hiperecogénicos a nivel del parénquima renal, pero este hallazgo no es específico del Pseudoxantoma elástico. Sin embargo, la presencia de este patrón ultrasonográfico en pacientes jóvenes con anomalías dermatológicas debe hacernos pensar en este síndrome dentro de las diferentes posibilidades diagnósticas. Aparte de los hallazgos ecográficos descritos, sólo una lesión ureteral en una paciente sometida a ureteroscopia ha sido descrita en relación al ámbito urológico de este síndrome. En nuestro caso, la relación entre esta enfermedad y la presencia de infección urinaria recurrente no esta clara

Case report: A 21-year-old woman presented with retinal angioid streaks and yellowish streak skin abnormalities in neck and axillary folds. Skin biopsy showed bluish-gray tangled masses of calcified elastic fibers in the mid-to lower dermis suggestive of Pseudoxanthoma elasticum (PXE). She consulted in Urology Department for lower urinary tract infection. Renal ultrasonography revealed multiple highly reflective foci in the corticomedullary junction. Comment: Pseudoxanthoma elasticum is a rare genetic disorder characterised by fragmentation and calcification of elastic fibers in the skin and media of arteries. Frequent manifestations include hypertension, angina pectoris, transient cerebral ischemic attacks, intermittent claudication, upper gastrointestinal bledding, retinal angioid streaks and thickened skin. A characteristic appearance of highly reflective foci in the renal parenchyma have been reported in patients with PXE, but it’s not specific for this syndrome. However, the presence of this structural pattern in a young patient with dermatological abnormalities should lead to the consideration of PXE in the differential diagnosis list. Besides ultrasonography findings, only a ureteral disruption case in a patient underwent ureteroscopic manipulation has been described to our knowledge. In our case, the true significance of these disease in recurrent urinary tract infection is uncertain