Localized primary ureteral amyloidosis.

OBJECTIVE: To analyze a new case of primary localized amyloidosis of the ureter, describing the differential diagnosis with ureteral tumors and its pathogenic mechanism. METHOD: Description of the case of a male patient who presented clinical symptoms of renoureteral pain and hematuria and an image suspected of ureteral tumour undergoing laparoscopic nephroureterectomy. The pathological study confirmed amyloidosis of the ureter, AL by immunohistochemistry. RESULTS: This report further extends the slightly more than 50 published cases of primary localized amyloidosis of the ureter the clinical signs of which, pain and haematuria, are similar, showing stenosis and enlargement in an area of the ureteral wall on imaging techniques. In the majority of cases the diagnosis was obtained postoperatively, being necessary to exclude generalized and secondary affectation. CONCLUSIONS: Emphasis must be placed on the differential diagnosis of tumors of the ureter, especially in single-kidney patients, by using ureteroscopy and biopsy or preferably a careful cytological study. In our case a local inflammatory phenomenon could have been the basis of its pathogenesis.

Amiloidosis ureteral primaria localizada / Localized primary ureteral amyloidosis

OBJETIVO: Analizar un nuevo caso de amiloidosis ureteral primaria localizada, ahondando en el diagnóstico diferencial con los tumores ureterales y en su mecanismo patogénico.MÉTODO: Describimos el caso de un paciente varón, al que por clínica de dolor renoureteral y hematuria y sospecha por imagen de tumor ureteral, se practicó nefroureterectomía laparoscópica, confirmando el estudio histológico amiloidosis ureteral, AL por inmunohistoquímica. RESULTADOS: Viene a engrosar los escasos 50 casos publicados de amiloidosis ureteral primaria localizada, cuya manifestación clínica de dolor y hematuria es similar, encontrando estenosis y engrosamiento en una zona de la pared ureteral en técnicas de imagen. En la mayoría el diagnóstico fue postoperatorio, debiendo excluir afectación generalizada y secundaria.CONCLUSIONES: Hay que incidir en el diagnóstico diferencial con los tumores de uréter, sobre todo en pacientes monorrenos, mediante ureteroscopia y biopsia o preferible estudio exhaustivo citológico. En nuestro caso un fenómeno inflamatorio local, pudo ser la base de su patogénesis(AU)

OBJECTIVE: To analyze a new case of primary localized amyloidosis of the ureter, describing the differential diagnosis with ureteral tumors and its pathogenic mechanism.METHOD: Description of the case of a male patient who presented clinical symptoms of renoureteral pain and hematuria and an image suspected of ureteral tumour undergoing laparoscopic nephroureterectomy. The pathological study confirmed amyloidosis of the ureter, AL by immunohistochemistry.RESULTS: This report further extends the slightly more than 50 published cases of primary localized amyloidosis of the ureter the clinical signs of which , pain and haematuria, are similar, showing stenosis and enlargement in an area of the ureteral wall on imaging techniques. In the majority of cases the diagnosis was obtained postoperatively, being necessary to exclude generalized and secondary affectation.CONCLUSIONS: Emphasis must be placed on the differential diagnosis of tumors of the ureter, especially in single-kidney patients, by using ureteroscopy and biopsy or preferably a careful cytological study. In our case a local inflammatory phenomenon could have been the basis of its pathogenesis(AU)

[Secondary amyloidosis of the bladder and massive hematuria]. / Amiloidosis vesical secundaria y hematuria masiva.

OBJECTIVE: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. MATERIALS AND METHODS: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. RESULTS: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massive, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. CONCLUSIONS: Despite its rarity, as shown by the few cases reported, secondary amyloidosis of the bladder should be considered in patients already diagnosed with systemic amyloidosis and/or the conditions reported who require simple urethral catheterization.

Amiloidosis vesical secundaria y hematuria masiva / Secondary amyloidosis of the bladder and massive hematuria

Objetivo: Dar a conocer cuatro nuevos casos de amiloidosis vesical secundaria, cuya manifestación clínica es extraordinariamente rara, si atendemos a los escasos casos publicados que no llegan a la treintena. Material y métodos: Describimos los cuatro casos clínicos, manifestados todos ellos por hematuria, siendo en tres masiva y fulminante, con evolución fatal. Resultados: La amiloidosis vesical secundaria corresponde al tipo AA, más frecuente en mujeres y secundaria sobre todo a artritis reumatoide, pero también a espondilitis anquilopoyética y procesos inflamatorios crónicos de larga evolución. La hematuria es el síntoma fundamental, prácticamente único. El estudio patológico e inmunohistoquímico confirma el diagnóstico. Se da la circunstancia de que los tres casos de hematuria masiva y fatal presentaron una patología intercurrente que precisó practicar un sondaje uretral, siendo este el desencadenante. Conclusiones: A pesar de su rareza, confirmada por los pocos casos publicados, habrá que pensar en ella ante pacientes ya diagnosticados de amiloidosis sistémica y/o con las patologías descritas, que precisen un simple sondaje uretral(AU)

Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor(AU)

[Charles II: From spell to genitourinary pathology]. / Carlos II: del hechizo a su patología génito-urinaria.

OBJECTIVES: We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. METHODS: We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with. RESULTS: It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presented an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter's syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract. CONCLUSIONS: As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monastery.

Carlos II: Del hechizo a su patología génito-urinaria / Charles II: From spell to genitourinary pathology

OBJETIVO: Tratar de desentrañar la compleja patología del último rey de la dinastía de los Austrias, Carlos II, apodado El Hechizado, dentro de la cual la urogenital fue preponderante, impidiéndole cumplir con una de las finalidades de la institución monárquica cual es la transmisión a un descendiente y produciéndole una serie de complicaciones que le llevaron al fallecimiento.MÉTODOS: Revisamos las obras en las cuales se describe la vida del Rey, haciendo hincapié en sus antecedentes consanguíneos, en las dudas sobre su sexo en el momento del nacimiento, sus procesos patológicos, la repercusión en las Cortes Europeas, pero sobre todo en sus matrimonios y en la incapacidad para generar un heredero. Resaltamos como, siguiendo el pensamiento de la sociedad española del siglo XVII, hizo pensar que se encontraba hechizado. Sobrenombre con el que pasó a la historia.RESULTADO: Deducimos que pudo presentar un hipospadias posterior que junto con la monorquia y testículo atrófico, hace pensar que presentó un estado intersexual con genitales ambiguos. Su fenotipo físico inclina más hacia un hermafroditismo verdadero y sobre todo un varón XX, que hacia un síndrome de Klinefelter que ha sido el más atribuido. Es probable su asociación con un síndrome X frágil. Monorreno congénito muy posiblemente, su muerte se debió a una insuficiencia renal crónica producida por una glomerulopatía o una nefropatía intersticial a consecuencia de una litiasis renal más infecciones del tracto urinario recidivantes.CONCLUSIONES: Fruto de una reiterada política matrimonial endogámica, feneció en 1700 la dinastía de los Habsburgo en España encarnada en Carlos II, un monarca pluripatológico que sólo se libraría de especulaciones si se efectuaran estudios cromosómicos y genéticos de sus restos presentes en el monasterio de El Escorial(AU)

OBJECTIVES: We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. METHODS: We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with.RESULT: It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presen-ted an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter’s syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract.CONCLUSIONS: As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monaster(AU)

[Scrotal liposarcoma]. / Liposarcoma escrotal.

OBJECTIVE: To describe an additional case of scrotal liposarcoma and its treatment, which was distinct from conventional treatment. METHODS: The literature on paratesticular masses is reviewed with special reference to the liposarcomas. An additional case of liposarcoma is described which was treated by tumor excision without involvement of the testis. The diagnosis, classification and treatment of this condition are discussed. RESULTS: Liposarcoma is a very uncommon tumor and its diagnosis is based on the anatomopathological findings. Treatment is by orchidectomy with high ligation of the spermatic cord. However, the case described herein was treated only by resection of the tumor. Radiotherapy may be indicated in some cases. Chemotherapy does not appear to be useful in this type of tumor. CONCLUSION: In some cases of scrotal liposarcoma, the tumor can be resected without performing orchidectomy.

[Retroperitoneal liposarcoma in a patient with renal transplantation]. / Liposarcoma retroperitoneal en un paciente trasplantado renal.

A case of retroperitoneal sarcoma in a kidney transplanted patient is presented. We describe the presentation pattern and the aggressive outcome despite the surgical removal of 5.900 gr. retroperitoneal tumor, included native kidney. The patient died due multiple metastasis six months later. An interesting iconography shows the great volume of the tumor.

[Pelvic lipomatosis: contribution of a new case to the literature]. / Lipomatosis pelviana: aportación de un nuevo caso a la literatura.

OBJECTIVE: To describe an additional case of pelvic lipomatosis, a rare condition that can be encountered by the urologist. METHODS/RESULTS: A 66-year-old patient consulted for irritative voiding syndrome and fever. Patient evaluation by IVP and CT demonstrated pelvic lipomatosis. CONCLUSIONS: Pelvic lipomatosis is a disease with variable clinical and radiological features. Careful follow-up is warranted for prevention and treatment of possible complications.

Liposarcoma retroperitoneal en un paciente trasplantado renal / Retroperitoneal sarcoma in a kidney trasplanted patient

Presentamos un caso de liposarcoma retroperitoneal en un paciente trasplantado renal. Describimos la forma de presentación, y su evolución clínica, que fue particularmente agresiva tras la intervención quirúrgica, en la que se extirpó una masa retroperitoneal de 5.900 gramos que incluía riñón propio. El paciente falleció 6 meses después de la intervención por múltiples metástasis sistémicas. Asimismo mostramos una iconografía interesante por el volumen que alcanzó la masa hasta hacerse clínicamente evidente (AU)

[Renal carcinoma with vena cava invasion. A new case]. / Carcinoma renal con invasión de vena cava. Un nuevo caso.

It is well known that the renal carcinoma can progress to the renal vein and cava. The MRI is the most accurate study in order to assay the extension of the thrombus. The surgical management is based on the extension and location of the thrombus. New immunologic-based treatment is now being tested. We describe our experience with surgical management of the one case of renal carcinoma with cava vein thrombus. No evidence of progression is noted after 18 month follow up.

Carcinoma renal con invasión de vena cava. Un nuevo caso / Renal carcinoma with vena cava invasion. A case report

El tumor de células renales presenta una intensa predisposición a crecer hacia la vena renal y vena cava. Cuando existe trombo tumoral en la vena cava es fundamental delimitar su extensión, y para ello contamos con la R.M.N. como la exploración más precisa. La vía de acceso quirúrgico y la técnica utilizada para la exéresis viene radicalmente determinada por la localización y extensión del tumor. En los últimos años se ha abierto una nueva esperanza terapéutica con los tratamientos de base inmunológica. Presentamos un caso de tumor de células renales con trombo en la vena cava tratado quirúrgicamente, y con un seguimiento de 18 meses, sin evidencia de recidiva ni extensión a distancia (AU)

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[Albuginea plication in the treatment of penile curvature]. / Plicatura de la albugínea como tratamiento para la incurvación de pene.

Presentation of the results obtained by our team in the treatment of 22 cases of penial incurvation (11 congenital and 11 secondary to Peyronie's Disease) using the albuginea plication technique. Evaluation of post-operative results taking into account both subjective (extent of satisfaction, comfort during the post-operative etc.) and objective (post-operative complications, degree of correction, reincurvation or failure etc.) factors was done by a follow-up programme through the out-patients practices using a Questionnaire-Survey system. Presentation of results obtained and detailed discussion of each case. The conclusions were as follows: Albuginea plication is a safe technique that achieves total penis redressment, with no post-operative complications. The extent of satisfaction and success obtained exceed 85%, and can be increased by improving the pre-operative selection and providing correct and precise information on what can be resolved with this technique.