Consequences of delay in screening, monitoring, and treatment of angiomyolipoma and tuberous sclerosis: A case report
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BACKGROUND: Tuberous sclerosis complex (TSC) is a multisystem disorder that results in tumor growth in various organs. TSC can affect the kidneys in the form of renal angiomyolipomas and cysts that can lead to chronic kidney disease. CASE PRESENTATION: A 38-year-old woman was referred to Kennedy Krieger Institute for comprehensive TSC management. Before referral, the patient had gone most of her life without a definite diagnosis of TSC despite visually-prominent signs such as forehead plaques, facial angiofibromas, and ungual fibromas. Eventually, complications of the disease led to the patient requiring hemodialysis at age 34 and a complete bilateral nephrectomy at age 36. However, the patient was not diagnosed with TSC until an evaluation at the National Institutes of Health at age 37. After becoming a patient at our clinic, a multidisciplinary approach was taken to provide comprehensive care by including various disciplines such as nephrology, neurology, pulmonology, ophthalmology, dentistry, dermatology, and cardiology. DISCUSSION AND CONCLUSION: TSC consensus recommendations aid in diagnosis, monitoring, and treatment of TSC and its associated manifestations, including those involving the kidneys. Our case underscores the importance of early identification of TSC to prevent future complications and promotes use of a multidisciplinary team to provide comprehensive care.
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A case of refractory chylous ascites after nephrectomy successfully treated with percutaneous obliteration using adhesive glue.

Here we report a case of uncontrollable chylous ascites that developed after nephrectomy and was successfully treated with percutaneous obliteration of the lymphocele-like extravasation using ethiodized oil during lymphangiography. Under computed tomographic and fluoroscopic guidance, an N-butyl cyanoacrylate-ethiodized oil mixture was used with metallic coils to obliterate the extralymphatic leakage site. The volume of intraperitoneal drainage decreased steadily over the next 5 days, and the tube was removed. Percutaneous obliteration can be characterized as filling of the leakage site from outside the lymph vessel with no flow disruption, which contrasts with the conventional embolization approach via the cisterna chyli.

Masa ocupante renal sólida de hasta 4 cm: análisis de la metodología diagnóstica, estadificación TNM y tratamiento quirúrgico / Solid renal mass up to 4 cm. Analysis of the diagnostic procedures, TNM staging and surgical treatment

Objetivos: Analizar retrospectivamente tumores renales operados con un tamaño máximo de 4 cm (T1a) para determinar si la metodología diagnóstica fue adecuada, el valor de la estadificación TNM de la UICC 2002 sobre el pronóstico y evaluar el tratamiento quirúrgico de elección. Material y método: Se estudiaron 78 unidades renales de 74 pacientes operados (4 casos bilaterales sincrónicos) desde 1984 a 2005 en el Servicio de Urología del Hospital Alemán. Se analizó la distribución etaria, sexo, forma de presentación (incidental y sintomático), metodología diagnóstica, variables de laboratorio, tratamiento quirúrgico (cirugía parcial o radical), histopatología, grado de malignidad según Fuhrman, tamaño tumoral, bilateralidad, multicentricidad estadificación TNM, evolución y supervivencia con la curva de Kaplan Meier. Resultados: Se estudiaron 78 unidades renales de un total de 192, 40,62%. La edad media fue 58,72 años. Sexo masculino 69% y femenino 31%. La forma de presentación fue 91,90% incidentales y sintomáticos el 8,10% (Hematuria en 5 y MTS pulmonar en 1). El diagnóstico fue por ecografía y tomografía computarizada en 63 casos, en 4 se agregó resonancia nuclear magnética (RNM) y en 7 ecografía y RNM. Arteriografía en 11 casos, 3 informaron tumor y 8 fueron normales. Biopsia en 5 casos, todas positivas para carcinoma células claras. El laboratorio sólo halló alterada la eritrosedimentación en 12 casos. El tratamiento quirúrgico fue cirugía radical en 35 unidades renales (44,87%) y conservadora en 43 unidades (55,13%). La histopatología fue carcinoma a células claras(CC) 79,48%, carcinoma papilar 1,28%, angiomiolipoma (AML) 8,97%, oncocitoma 7,69% y adenoma 2,56%. El grado de malignidad fue Fuhrman 1(76,19%), 2(20,63%) y 3(3,18%). Bilaterales 4 en 2 sendos CC, 1 CC y AML y 1 CC y adenoma. Tamaño tumoral medio 2,93 cm. Estadios T1a 96,82%, T3a 1,59% y T3aM+1,59%. Se obtuvieron datos de la evolución en 54 de 61 tumores malignos, de los cuales 50 casos se hallaban libres de enfermedad, 3 fallecidos por progresión a los 18, 33 y 82 meses, respectivamente, todos ellos tumores sintomáticos y 1 fallecido por otra causa. La supervivencia con un tiempo medio de seguimiento de 52,25 meses fue 94%. Conclusiones: 1) Con la ecografía y la TC se obtuvo un alto índice de definición diagnóstica para tumores renales sólidos. La biopsia en ciertos casos contribuyó a lograr una correcta táctica de tratamiento. 2) La cirugía conservadora fue el tratamiento de elección en tumores hasta en 4 cm correctamente seleccionados. Creemos que la estadificación TNM debería contemplar en este tamaño tumoral la forma de presentación para mejorar su valor en el pronóstico

Objectives: We perform a retrospective review of renal tumors operated with a maximum size of 4 cm to determine if the diagnostic methodology was adequate, the TMN staging prognostic accuracy (UICC 2002) and the goal surgical treatment. Material and method: Between 1984 to 2005, 78 renal units form 74 patients (4 bilateral synchronous) operated at the Service of Urology of the Hospital Alemán de Buenos Aires. Age distribution, sex, presentation form (incidental and symptomatic), diagnostic methodology, laboratory variables, surgical treatment (partial or radical surgery), histopathology, Fuhrman grade, tumor size, bilaterality, multicentricity, TNM staging, evolution and survival (Kaplan Meier) were analyzed. Results: 78 renal units from a total of 192, 40.62% were analyzed. The median age was 58.72 years. 69% were male and 31% female. The presentation form was 91.90 % incidental and symptomatic 8.10% (Hematuria in 5 and lung metastasis in 1). The diagnosis was performing with ultrasound and CT scan 63 cases, in 4 was also used resonance magnetic imaging (RMI) and in 7 ultrasound and RMI. Arteriography was used in 11 cases, 3 showed tumor and 8 were normal. Biopsy was performing in 5 cases, all positive for clear cells carcinoma. Globular sedimentation was the only one laboratory abnormality in 12 cases. Surgical treatment was radical surgery in 35 renal units (44.87%) and conservative surgery in 43 units (55.13%). Pathology clear cells carcinoma (CCC) 79.48%, papillary carcinoma 1.28%, angiomyolipoma (AML) 8.97%, oncocytoma 7.69% and adenoma 2.56%. The Fuhrman grade was 1 in 76.19%; 2 in 20.63% and 3 in 3.18%. Bilateral tumor were found in 4 cases 2 CCC, 1 CCC and AML and 1 CCC and adenoma. Tumor median size was 2.93 cm. Staging was T1a 96.82%, T3a 1.59% and T3aM+ 1.59%. Follow-up could be made in 54 of 61cases. At median follow-up of 52.25 months, 50 cases were disease free, 3 died by progression at 18, 33 and 82 months and all of them were symptomatic tumors, 1 died by a non related cause. Survival rate was 94%. Conclusions: 1)Ultrasound and CT scan obtained a highest diagnostic accuracy for solid renal mass. Biopsy in selective cases could contribute to achieve a correct treatment strategy. 2) Conservative surgery was the goal treatment in selected tumors up to 4 cm. and we believe that TNM staging should contemplate the presentation form to improve the prognostic value

Renal angiomyolipoma: selective arterial embolization--effectiveness and changes in angiomyogenic components in long-term follow-up.

PURPOSE: To evaluate the efficacy of selective arterial embolization in symptomatic renal angiomyolipoma (AML) and the change in angiomyogenic components during long-term follow-up after embolization. MATERIALS AND METHODS: Fourteen adult patients with symptomatic AMLs underwent 16 selective arterial embolizations. The embolic materials used were absolute alcohol with (n = 5) or without (n = 3) iodized oil, Gianturco coils (n = 4), and polyvinyl alcohol foam powder with gelatin sponge (n = 2). Follow-up ultrasonography and computed tomography (CT) were performed in six and 14 patients, respectively. The effectiveness of selective arterial embolization was evaluated on the basis of the area of the angiomyogenic components in the AML on initial and follow-up images and clinical improvement. RESULTS: All patients showed devascularization of the tumor on the postembolization angiograms. In 13 patients, clinical symptoms disappeared. The follow-up period was 7-72 months (mean, 33 months). One patient underwent nephrectomy at 7 months after embolization because of a large cystic lesion found at 1 month. In long-term CT follow-up (> or =12 months) in 12 patients, nearly all angiomyogenic components disappeared, but fatty components partially shrank with liquefactive necrosis in tumors. CONCLUSION: Selective arterial embolization is an effective and safe treatment of AML. The angiomyomatous components crucial for the prevention of bleeding were very sensitive to the embolization.

Renal angiomyolipoma: diagnosis with B-ultrasonography, CT scanning, DSA and its interventional treatment.

From 1989, 15 cases of renal angiomyolipoma (AML) have been diagnosed by ultrasonography. CT scanning and digital subtraction angiography (DSA) at our hospital. In 8 patients with uneven hyperechoes on B-mode ultrasonography (B-US) (8/15) and 7 with low density of fat on CT scanning (7/12) accurate diagnosis was established preoperatively. DSA revealed the "berry-like" pseudoaneurysms in the arterial phase (14 cases), the defined lucent area in the nephrogram phase (10 cases) and the "onion-peel appearances" during venous phases (8 cases), correct diagnosis was achieved in all patients. 8 cases were surgically treated and 7 treated by subselective embolization of renal artery. Effects in all cases were good. The diagnostic value of B-US, CT scanning, DSA and interventional treatment of AML was discussed. It was believed that the diagnosis with DSA was a technique with high specificity, and embolization therapy was simple and effective for AML.