First case of nodular dermatofibrosis with cystic renal disease in a beagle: An atypical case without FLCN gene mutation.

This report presents the first case of nodular dermatofibrosis with renal cysts (NDRC) in a beagle. In this atypical case, the gene mutation associated with the disease was not present, the renal cysts showed dynamic changes in size and number, and the patient has greatly surpassed the NDRC life expectation.

A clinical review of congenital hepatic fibrosis diagnosed in adulthood: presentation, complications, and outcomes.

INTRODUCTION AND OBJECTIVES: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. MATERIALS AND METHODS: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. RESULTS: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). CONCLUSIONS: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.

Detección de mutaciones del gen de HNF1B en niños con malformaciones congénitas renales y del tracto urinario / Detection of mutations of the HNF1B gene in children with congenital anomalies of the kidney and urinary tract

Resumen: Introducción: Las anomalías congénitas del riñón y del tracto urinario se originan de alteraciones genéticas, en su mayoría desconocidas. Las mutaciones en el gen que codifica para el factor hepatocitario nuclear 1B (HNF1B), son la causa monogénica más frecuentemente descrita. Se desconocen datos en Chile y Latinoamérica. Objetivo: Determinar la presencia de variantes del gen HNF1B en niños chilenos con anomalías congénitas del riñón y/o tracto urinario y sus características clínicas. Pacientes y Mé todo: Estudio descriptivo con pacientes entre 10 meses y 17 años, consultantes en Unidad de Nefrología Hospital Luis Calvo Mackenna, período abril - diciembre 2016, portadores de displasia renal quística, displasia/hipoplasia renal no quística y/o riñón en herradura. Se determinaron variantes de HNF1B mediante secuenciación de exones 1, 2, 3 y 4; previa extracción y amplificación de DNA. Se utilizaron enzimas de restricción para definir si variantes eran homo o heterocigotas. Familiares di rectos de casos índices se estudiaron con secuenciación del exón afectado. Resultados: Se incluyeron 32 pacientes, 43,75% varones, mediana edad 11 años. El 65,6% displasia/hipoplasia renal no quística, 31,25% displasia renal quística y 3,15% riñón en herradura. En 2 pacientes (6,25%) se detectó una misma variante genética heterocigota en exón 4, posición 1027 (C1027T), no descrita anteriormente. El estudio de familiares determinó la variante en 3 de 5 individuos, todos sin anomalías nefrouro- lógicas congénitas. Conclusiones: Confirmamos la presencia de una variante genética heterocigota del gen HNF1B, no descrita previamente, dando inicio a la búsqueda de este tipo de mutaciones en nuestro medio, lo cual nos permite aproximarnos al conocimiento de causalidad, determinación de compromiso extrarrenal y consejo genético.

Abstract Introduction: Congenital anomalies of the kidney and urinary tract are caused by genetic alterations mostly unknown. Mutations in the gene that codes for hepatocyte nuclear factor 1B (HNF1B) are the most frequently described monogenic causes. Data are unknown in Chile and Latin America. Objective: To determine the presence of variants of the HNF1B gene in Chilean children with conge nital anomalies of the kidney and/or the urinary tract and their clinical characteristics. Patients and Method: Descriptive study with children aged 10 months to 17 years, patients of the Calvo Mackenna Hospital Nephrology Unit, with cystic renal dysplasia, non cystic renal dysplasia/hypoplasia, horses hoe kidney between April and December 2016. HNF1B variants were determined by sequencing of exons 1, 2, 3 and 4 after DNA extraction and amplification. Restriction enzymes were used to define if the variants were homo or heterozygous. Direct family members of index cases were studied with sequencing of the affected exon. Results: 32 patients were included, 43.75% males, median age 11 years. 65.6% of them had non-cystic renal dysplasia, 31.25% cystic renal dysplasia, and 3.15% hor seshoe kidney. In two patients (6.25%) the same heterozygous genetic variant was detected in exon 4, position 1027 (C1027T), not previously described. The study of relatives found the same variant in three out of five individuals, all without congenital nephro-urological anomalies. Conclusions: We confirmed the presence of a not previously described heterozygous genetic variant of the HNF1B gene. This work initiates the search for this type of mutations in our region which allows us to ap proach the knowledge of causality, determination of extrarenal involvement, and genetic counseling.

Malignancy Rate, Histologic Grade, and Progression of Bosniak Category III and IV Complex Renal Cystic Lesions.

OBJECTIVE: The primary purpose of this study is to determine the malignancy rate, histologic grade, and initial stage of surgically treated complex renal cysts classified as Bosniak category III or IV. For nonsurgical lesions, a secondary objective was to evaluate lesion progression on follow-up examinations. MATERIALS AND METHODS: We searched our database for cystic lesions classified as Bosniak III or IV category on CT or MRI from January 2008 to April 2016. Surgically resected lesions, per category, were correlated with information on pathologic reports to obtain malignancy rates. For malignant lesions, histologic grade and initial stage were evaluated. Imaging follow-up of at least 2 years was used to evaluate progression of clinically followed lesions. RESULTS: We included 86 lesions in 85 patients in the final analysis. Of the 60 surgically resected lesions (70%), 46 (77%) were malignant and 14 (23%) were benign. Malignancy rates were 72% for Bosniak category III lesions and 86% for Bosniak category IV lesions. Most malignant cysts were early-stage (pT1) cysts with low histologic grades (89% of Bosniak III lesions and 91% of Bosniak IV lesions). Follow-up studies of the surgically resected lesions did not show local recurrence, metastasis, or lymph node enlargement. Among patients with lesions managed by watchful waiting (n = 26), all lesions remained unchanged in terms of size and complexity after at least 2-years of follow-up. CONCLUSION: Although high malignancy rates were observed for both Bosniak category III and IV lesions, our results suggest that such malignant cysts are usually early-stage tumors with a low histologic grade. Lesions that underwent follow-up remained unchanged on control examinations. These findings may indicate low aggressiveness of these lesions, supporting the idea that more conservative approaches may be used.

Carcinomas renales múltiples como manifestación inicial del Síndrome de Von Hippel Lindau. Presentación de caso / Multiple renal carcinoma as a clinical manifestation of Von Hippel Lindau Syndrome. Case presentation

Introducción: El Síndrome de Von Hippel Lindau es una afección neoplásica multisistémica, heredada de manera autosómica dominante y con alta penetrancia. Su expresividad clínica es muy diversa,oscilando la incidencia entre 1/35000 y 1/36000 nacidos vivos. Esta enfermedad usualmente se diagnostica entre los 20 y 30 años, pero los síntomas pueden aparecer en la infancia. La lesión clínica inicial más común y precoz es el hemangioblastoma de la retina y/o del sistema nervioso central. Objetivo: Presentar un caso de un paciente con carcinomas renales múltiples como manifestación inicial de un Síndrome de Von Hippel Lindau. Presentación del Caso: Paciente masculino de 59 años, con antecedentes de salud, quien acude a urgencias por cuadro febril de 3 días de evolución, que fue interpretado como Dengue; se le realizó, dentro de los complementarios, ultrasonido abdominal, donde se descubrió masa sólida a nivel del polo superior del riñón derecho, asociado a existencia de otra en polo inferior de este mismo riñón, así como de 2 más en el contralateral. Además, se encontraron varios quistes pancreáticos y 2 renales izquierdos corticales. El paciente fue intervenido quirúrgicamente; se corroboró el diagnóstico de carcinomas renales de células claras. El examen oftalmológico reveló la presencia de un hemangioblastoma retiniano derecho; en tanto la tomografía computarizada simple de cráneo y la resonancia magnética espinal no mostraron alteraciones. Conclusiones: Este síndrome es una rara, pero grave afección genética, caracterizada por un alto riesgo de desarrollar enfermedades neoplásicas, lo que hace que sea aún más importante conocerlo, para poder identificar y tratar a tiempo sus temidas complicaciones(AU)

Introduction: Von Hippel Lindau Syndrome is a multisystem neoplastic affection, which is inherited as an autosomal dominant trait, with high penetrance. Its clinical expressivity is very diverse, ranging its incidence between 1/35000 and 1/36000 born alive. This disease is usually diagnosed between the 20 and 30 years of age, but its symptoms can appear in childhood. The most common and early initial clinical lesion is the hemangioblastoma of the retina and/or central nervous system. Objective:To present a case of a patient with multiple renal carcinoma as initial manifestation of Von Hippel Lindau Syndrome. Case presentation: 59 years old male patient with a history of good health who comes to the Emergency Room because of febrile clinical state of 3 days´ evolution, that was interpreted as dengue. Abdominal ultrasound was included in the complementary studies, in which a solid mass at level of upper pole of right kidney was observed, associated with the existence of another one in lower pole of the same kidney, as well as two others in contralateral. Also, multiple pancreatic cysts and two left cortical renal ones were found. The patient underwent surgery, and the diagnosis of clear cells renal carcinoma was corroborated. The ophthalmological exam revealed the presence of a right retinal hemangioblastoma whereas the plain skull CT-scan, and the magnetic resonance imaging of the lumbar spine did not show any alterations. Conclusions:This syndrome is a rare, but a serious genetic affection, characterized by a high risk to develop neoplastic diseases; that´s one reason why it is very important to know about it in order to identify, and treat its feared complications in time(AU)

Minimally Complex Renal Cysts: Outcomes and Ultrasound Evaluation Compared with Contrast-Enhanced Cross-Sectional Imaging Bosniak Classification.

We correlated contrast-enhanced cross-sectional imaging and outcomes to assess the reproducibility of ultrasonographic criteria for renal minimally complex (MC) cysts. From 2003 to 2015, 143 cysts were described as complex or MC by ultrasound (US). After exclusions, 98 US studies were retrospectively evaluated and compared with computed tomography (CT)/magnetic resonance imaging (MRI). At sonography, 51 were MC cysts and 47 were complexes according to two independent observers. Inter-observer agreement for US was 0.704 and 0.745 for CT/MRI. Of 51 cysts classified as MC by US, 38 were Bosniak I/II and 6 were Bosniak IIF by CT/MRI. In 7, there were no cross-sectional images; however, they were stable for at least 2 y. Of 47 complex cysts, 9 were Bosniak II, 22 Bosniak IIF, 8 Bosniak III and 8 Bosniak IV. No Bosniak III/IV cysts by CT/MRI were classified as MC by US. Our results indicate that US offers reproducible criteria for MC cysts and may be used alone for these lesions.

MRI evaluation of complex renal cysts using the Bosniak classification: a comparison to CT.

PURPOSE: The purpose of this study was to compare the ability of magnetic resonance imaging (MRI) and computed tomography (CT) to discriminate between benign and malignant cystic renal lesions utilizing the Bosniak classification. MATERIALS AND METHODS: We retrospectively searched our Radiological Information System using renal/kidney cysts as entries. The search retrieved 2929 patients and 525 complex renal cysts. After exclusions, 42 complex cysts, from 37 patients, with CT and MRI, up to six months apart, were included. Surgery and pathology report and follow-up of at least 24 months were used as a standard of reference. RESULTS: The mean age of patients was 51.4 years, ranging from 11 to 82 years old. Twenty-nine lesions were classified as Bosniak I, II or II-F by CT and/or MRI and 13 as Bosniak III or IV, by one of the methods. The interobserver agreement for Bosniak classification for CT was 0.87 and 0.93 for MRI. Fifteen lesions had higher Bosniak categories on MRI, included six with change in management. Only two lesions had a higher category on CT, one with change in management. The frequency of malignancy for Bosniak III was 50 % (2/4) for CT and 20% for MRI (1/5), as Bosniak upgrades by MRI resulted in surgery for benign lesions. Both methods had 100 % frequency of malignancy for category 4. CONCLUSION: MRI led to category migration and management change of complex renal cysts in a significant proportion of cases, likely due to its superior soft tissue and contrast resolution. The impact of MRI on detection and outcomes of malignant complex renal cysts still requires further investigation.

Safety and feasibility of radiofrequency ablation for treatment of Bosniak IV renal cysts

ABSTRACT Purpose To describe our initial experience with radiofrequency ablation (RFA) of Bosniak IV renal cysts. Materials and Methods From 2010 to 2014, 154 renal tumor cases were treated with percutaneous thermal ablation, of which 10 cases (6.4%) from nine patients were complex renal cysts and were treated with radiofrequency ablation. Results All complex cysts were classified as Bosniak IV (four women and five men; mean age: 63.6 yrs, range: 33–83 years). One patient had a single kidney. Lesion size ranged from 1.5 to 4.1cm (mean: 2.5cm) and biopsy was performed on four cysts immediately before the procedure, all of which were malignant (two clear cell and two papillary carcinoma). Mean volume reduction of complex cysts was 25% (range: 10–40%). No patients required retreatment with RFA and no immediate or late complications were observed. The follow-up of Bosniak IV cysts had a median of 27 months (interquartile range [IQR], 23 to 38) and no recurrence or significant loss of renal function were observed. Conclusions Mid-term follow-up of the cases in our database suggests that image-guided percutaneous RFA can treat Bosniak IV cysts with very low complication rates and satisfactorily maintain renal function.

Radioterapia de intensidade modulada (IMRT) para pacientes do SUS: análise de 508 tratamentos em dois anos de instalação da técnica / Intensity modulated radiotherapy (IMRT) for patients of the Brazilian unified health system (SUS): an analysis of 508 treatments two years after the technique implementation

Objetivo: A oferta de radioterapia de alta tecnologia para população atendida pelo Sistema Único de Saúde (SUS) é limitada, por não pertencer ao rol de procedimentos e, muitas vezes, pela capacidade instalada frente à demanda e dificuldade de retenção de recursos humanos especializados. Dessa forma, o acesso à radioterapia de intensidade modulada (IMRT) é restrito a poucos serviços no Brasil. Pretendemos apresentar as características dos primeiros 508 tratamentos de IMRT durante os primeiros anos de instalação da técnica em um hospital universitário. Materiais e Métodos: Foram analisados 508 tratamentos de IMRT, de maio de 2011 a setembro de 2013, que completaram a radioterapia. A técnica empregada foi multilâminas estático. Resultados: De um total de 4.233 pacientes tratados no período, 12,5% realizaram IMRT. As principais indicações foram para crânio, cabeça e pescoço, e próstata. Aproximadamente 30% das radioterapias de crânio e 50% das de próstata foram por IMRT. A toxicidade total foi 4%. Conclusão: Em razão das restrições de acesso à radioterapia e da não cobertura deste procedimento, as indicações de IMRT para pacientes do SUS devem ser apoiadas nos protocolos clínicos das instituições em acordo com sua realidade, com especial atenção à redução da toxicidade. .

The Bosniak classification for renal cysts was developed in the late 1980s in an attempt to standardize the description and management of complex cystic renal lesions. Alterations were made to such a classification in the 1990s and, the last one, in 2005. Currently, five categories of cystic renal lesions are defined - namely, I, II, II-F, III and IV –, according to their degree of complexity and likelihood of malignancy. Despite being initially described for computed tomography, this classification has been also utilized with some advantages also for magnetic resonance imaging. The present article reviews the different phases of this classification, its diagnostic efficacy and the most controversial features of its use.

Classificação de Bosniak para cistos renais complexos: histórico e análise crítica / Bosniak classification for complex renal cysts: history and critical analysis

A classificação de Bosniak para cistos renais surgiu na década de 1980 para tentar padronizar a descrição e condutas em relação às lesões renais císticas complexas. Esta classificação sofreu alterações na década de 1990 e, a última, em 2005. Atualmente, são definidas cinco categorias de lesões císticas renais – I, II, II-F, III e IV –, de acordo com o grau de complexidade e maior probabilidade de malignidade. Apesar de inicialmente ter sido descrita para a tomografia computadorizada, esta classificação é utilizada, com algumas vantagens, também na ressonância magnética. O presente artigo revisa as diferentes fases desta classificação, sua eficácia diagnóstica e os aspectos mais controversos de sua utilização.

The Bosniak classification for renal cysts was developed in the late 1980s in an attempt to standardize the description and management of complex cystic renal lesions. Alterations were made to such a classification in the 1990s and, the last one, in 2005. Currently, five categories of cystic renal lesions are defined - namely, I, II, II-F, III and IV –, according to their degree of complexity and likelihood of malignancy. Despite being initially described for computed tomography, this classification has been also utilized with some advantages also for magnetic resonance imaging. The present article reviews the different phases of this classification, its diagnostic efficacy and the most controversial features of its use.

Enfermedad renal quística adquirida y su relación con variables clínicas, demográficas y antropométricas en pacientes dialíticos / Acquired renal cystic disease and its relationship to clinical, demographic and anthropometric variables in patients on hemodialysis

Introducción: la enfermedad renal crónica (ERC) se asocia frecuentemente con enfermedad renal quística adquirida (ERQA). En el orden clínico, esta última suele ser asintomática, pero puede complicarse con carcinoma renal. Objetivo: identificar la frecuencia de presentación de ERQA y la posible relación de la longitud renal, el número y tamaño de los quistes, con características de pacientes en hemodiálisis. Métodos: se realizó un estudio analítico, transversal, en el cual se incluyó a todos los pacientes del Servicio de Hemodiálisis del Instituto de Nefrología, en tratamiento en junio de 2012. Fueron excluidos los que tenían enfermedad quística como causa de ERC, los nefrectomizados y aquellos con mala ventana ecográfica. La ecografía renal se realizó con equipo Toshiba Nemio XG de alta resolución y transductor convexo de 3,5 MHz. La información fue procesada de forma automatizada mediante el paquete estadístico SPSS, versión 15.0. Para identificar las posibles relaciones entre las variables fueron utilizados los test de Wilcoxon-Mann-Whitney y de Kruskal-Wallis. También se empleó el coeficiente de correlación lineal de Pearson (r). Resultados: el 38,4 por ciento de los pacientes presentó ERQA. Se encontró asociación del número de quistes con el tiempo en hemodiálisis, la dosis de eritropoyetina utilizada y las cifras de hemoglobina (p< 0,05 en los 3 casos). Entre tiempo en hemodiálisis y número de quistes, la relación fue directa y moderada (r= 0,60). Conclusiones: la frecuencia de ERQA es elevada en pacientes en hemodiálisis. El número de quistes aumenta con el tiempo en tratamiento y los pacientes con mayor número de quistes poseen valores más elevados de hemoglobina y requieren de dosis más bajas de eritropoyetina...

Introduction: chronic kidney disease (CKD) is frequently associated with acquired renal cystic disease (ARCD). The latter condition is usually clinically asymptomatic, but it may complicate with renal carcinoma. Objective: identify the frequency of presentation of ARCD and the possible relationship of renal length and the number and size of cysts, to characteristics of patients on hemodialysis. Methods: an analytical cross-sectional study was conducted of all the patients on hemodialysis at the Institute of Nephrology in June 2012. Patients with cystic disease as the cause of CKD, nephrectomized patients and those with a poor echographic window were not included. Renal echography was performed with high resolution Toshiba Nemio XG equipment and a 3.5 MHz convex transducer. Data was processed with the statistical software SPSS version 15.0. The Wilcoxon-Mann-Whitney and Kruskal-Wallis tests were used for identification of the possible relationships between variables. The Pearson linear correlation coefficient (r) was also used. Results: 38.4 percent of the patients had ARCD. An association was found between the number of cysts and the time on hemodialysis, the doses of erythropoietin used, and hemoglobin values (p< 0.05 in the three cases). The relationship between time on hemodialysis and number of cysts was direct and moderate (r= 0.60). Conclusions: the frequency of ARCD was high among patients on hemodialysis. The number of cysts increased with treatment time. Patients with a greater number of cysts have higher hemoglobin values and require smaller doses of erythropoietin...

Bosniak classification of renal cystic lesions according to multidetector computed tomography findings / Classificação de Bosniak das lesões císticas renais segundo achados na tomografia computadorizada multidetectores

Renal cystic lesions are usually diagnosed in the radiologists' practice and therefore their characterization is crucial to determine the clinical approach to be adopted and prognosis. The Bosniak classification based on computed tomography findings has allowed for standardization and categorization of lesions in increasing order of malignancy (I, II, IIF, III and IV) in a simple and accurate way. The present iconographic essay developed with multidetector computed tomography images of selected cases from the archives of the authors' institution, is aimed at describing imaging findings that can help in the diagnosis of renal cysts.

As lesões renais císticas são diagnósticos frequentes na prática do médico radiologista, sendo sua caracterização fundamental na determinação da conduta e prognóstico. A classificação de Bosniak permitiu, por meio de tomografia computadorizada, uniformizar e categorizar tais lesões em ordem crescente de malignidade (I, II, IIF, III e IV), sendo esta avaliação realizada de maneira simples e precisa. Este ensaio iconográfico realizado com tomografia computadorizada multidetectores, de casos selecionados dos arquivos do nosso serviço, tem como objetivo demonstrar achados de imagem que possam auxiliar no reconhecimento dos principais aspectos diagnósticos dos cistos renais.

Intensity modulated radiotherapy (IMRT) for patients of the Brazilian unified health system (SUS): an analysis of 508 treatments two years after the technique implementation.

The Bosniak classification for renal cysts was developed in the late 1980s in an attempt to standardize the description and management of complex cystic renal lesions. Alterations were made to such a classification in the 1990s and, the last one, in 2005. Currently, five categories of cystic renal lesions are defined - namely, I, II, II-F, III and IV -, according to their degree of complexity and likelihood of malignancy. Despite being initially described for computed tomography, this classification has been also utilized with some advantages also for magnetic resonance imaging. The present article reviews the different phases of this classification, its diagnostic efficacy and the most controversial features of its use.

OBJETIVO: A oferta de radioterapia de alta tecnologia para população atendida pelo Sistema Único de Saúde (SUS) é limitada, por não pertencer ao rol de procedimentos e, muitas vezes, pela capacidade instalada frente à demanda e dificuldade de retenção de recursos humanos especializados. Dessa forma, o acesso à radioterapia de intensidade modulada (IMRT) é restrito a poucos serviços no Brasil. Pretendemos apresentar as características dos primeiros 508 tratamentos de IMRT durante os primeiros anos de instalação da técnica em um hospital universitário. MATERIAIS E MÉTODOS: Foram analisados 508 tratamentos de IMRT, de maio de 2011 a setembro de 2013, que completaram a radioterapia. A técnica empregada foi multilâminas estático. RESULTADOS: De um total de 4.233 pacientes tratados no período, 12,5% realizaram IMRT. As principais indicações foram para crânio, cabeça e pescoço, e próstata. Aproximadamente 30% das radioterapias de crânio e 50% das de próstata foram por IMRT. A toxicidade total foi 4%. CONCLUSÃO: Em razão das restrições de acesso à radioterapia e da não cobertura deste procedimento, as indicações de IMRT para pacientes do SUS devem ser apoiadas nos protocolos clínicos das instituições em acordo com sua realidade, com especial atenção à redução da toxicidade.

Bosniak classification for complex renal cysts: history and critical analysis.

The Bosniak classification for renal cysts was developed in the late 1980s in an attempt to standardize the description and management of complex cystic renal lesions. Alterations were made to such a classification in the 1990s and, the last one, in 2005. Currently, five categories of cystic renal lesions are defined - namely, I, II, II-F, III and IV -, according to their degree of complexity and likelihood of malignancy. Despite being initially described for computed tomography, this classification has been also utilized with some advantages also for magnetic resonance imaging. The present article reviews the different phases of this classification, its diagnostic efficacy and the most controversial features of its use.

A classificação de Bosniak para cistos renais surgiu na década de 1980 para tentar padronizar a descrição e condutas em relação às lesões renais císticas complexas. Esta classificação sofreu alterações na década de 1990 e, a última, em 2005. Atualmente, são definidas cinco categorias de lesões císticas renais ­ I, II, II-F, III e IV ­, de acordo com o grau de complexidade e maior probabilidade de malignidade. Apesar de inicialmente ter sido descrita para a tomografia computadorizada, esta classificação é utilizada, com algumas vantagens, também na ressonância magnética. O presente artigo revisa as diferentes fases desta classificação, sua eficácia diagnóstica e os aspectos mais controversos de sua utilização.

Quiste renal gigante. Presentación de un caso / Giant renal cyst. Presentation of a case

En el riñón frecuentemente se desarrollan quistes. Dentro de la diversidad de quistes renales, el simple puede presentarse dentro del riñón o en la superficie de este pudiendo, ser único o múltiple, unilateral o bilateral y más frecuente en el izquierdo. Generalmente se manifiestan asintomático y pueden alcanzar gran tamaño, denominándose quistes gigantes. Se presenta un paciente de 66 años que ingresa por dolor lumbar hace un año, que empeora a pesar del tratamiento médico para una presunta sacrolumbalgia. En el abdomen se aprecia marcado aumento de volumen, sobre todo hacia el hemiabdomen superior y se palpa gran masa tumoral que ocupa la totalidad del hemiabdomen superior, renitente y no dolorosa. Se realizó tomografía axial computarizada de abdomen, apreciándose imagen hipodensa multitabicada que ocupa todo el hemiabdomen izquierdo con desplazamiento de asas, riñón izquierdo y músculo psoas. Se concluye como quiste gigante multitabicado de riñón izquierdo. Se realizó laparotomía paramediana izquierda supra e infraumbilical y se detecta gran quiste renal del polo superior del riñón izquierdo que sobrepasa la línea media desplazando la arteria aorta a la derecha de la columna vertebral. Se realizó nefrectomía izquierda. Es raro, que un quiste renal simple gigante, sobrepase la línea media y tenga compresión de tantos órganos intraabdominales; este es un hecho poco descrito en la literatura médica. El examen físico superficial y limitado al órgano que produce los síntomas que motivan la consulta, limita el diagnóstico precoz de muchas enfermedades como se demuestra en este paciente. Este es uno de los errores más frecuentes que se aprecian al aplicar el método clínico.

Frequently, cysts develop in the kidney. Among the diversity of renal cysts, the simple one could be located inside the kidney or in its surface and may be single or multiple, unilateral or bilateral, and more frequent in the left kidney. Generally they are asymptomatic and can reach a great size, called giant cysts. We present a patient aged 66 years, entering the hospital with lumbar pain of one year that gets worse despite the medical treatment for a presumed sacrolumbal pain. It was found a remarked increase of the abdomen volume, mainly in the direction of the upper hemiabdomen and it feels a big tumoral mass filling the totality of the upper abdomen, renitent and no painful. An abdominal computer tomography was carried out, finding a multi partitioned hypodense image filling all the left hemiabdomen with displacement of loops, left kidney and psoas muscle. We arrived to the conclusion that it was a giant multi partitioned cyst of the left kidney. A left paramedian supra and infra umbilical laparotomy was carried out finding a renal cyst of the upper pole of the left kidney surpassing media line and displacing aortal artery to the right of the spinal column. A left nephrectomy was carried out. It is rare a simple renal giant cyst surpassing the media line and compressing so many intra abdominal organs; this is a fact scarcely described in the medical literature. The surface physical examination restricted to the organ producing the symptoms that motivate the consultation, interferes the precocious diagnosis of many diseases as it is shown in this patient. This is one of the most frequent mistakes committed when applying the clinical method.

Cystic adnexal mass in a 16-year-old female: Ovarian pathology or complication of a Müllerian anomaly?

Patient: Female, 16 Final Diagnosis: Pelvic mass Symptoms: None Medication: None Clinical Procedure: CT • MRI Specialty: Diagnostic radiology • pediatrics. OBJECTIVE: Unusual presentation of unknown etiology, Rare disease, Mistake in diagnosis. BACKGROUND: Müllerian anomalies encompass a wide variety of malformations in the female genital tract, usually associated with renal and anorectal malformations. Of these anomalies, approximately 11% are uterus didelphys, which occurs when midline fusion of the müllerian ducts is arrested to a variable extent. CASE REPORT: We report the case of a 16-year-old female with uterine didelphys, jejunal malrotation, hematometra, hematosalpinx, and bilateral subcentimeter homogenous circular cystic-like renal lesions, who initially presented with left lower quadrant abdominal pain, non-bloody vomiting, and a history of irregular menstrual periods. Initial CT was confusing for an adnexal cystic mass, but further imaging disclosed the above müllerian anomalies. CONCLUSIONS: Müllerian anomalies may mimic other, more common, adnexal lesions; thus, adequate evaluation of suspicious cystic adnexal masses with multiple and advanced imaging modalities such as MRI is essential for adequate diagnosis and management.

Enfermedad quística renal en la edad pediátrica / Kidney cystic disease in pediatric age

Cystic diseases of the kidney include a width range of pathologies, representing a diagnostic challenge for both clinician and radiologist. There are a many classifications for these pathologies, which can confuse the images interpretation. With emphasis in diagnosis images, with the ultrasound as the most important, the main characteristics of the most frequent cystic renal diseases are reviewed: autosomal recessive and dominant polycystic kidney disease, glomerulocystic kidneys, tuberous sclerosis, juvenile nephronophthisis, sponge kidney, simple cysts, multicystic dysplastic kidney, cystic dysplastic kidney, multilocular cystic renal tumor and acquired cystic kidney disease.

Las enfermedades quísticas renales incluyen una variada gama de patologías y representan un desafío diagnóstico tanto para el clínico como para el radiólogo. Existen diversas clasificaciones, que puede crear confusión en la interpretación de las imágenes. Con especial énfasis en el diagnóstico por imágenes, donde el ultrasonido juega un rol preponderante, se revisan las principales características de las entidades más frecuentes: enfermedad renal poliquística autosómica recesiva y dominante, enfermedad renal glomeruloquística, esclerosis tuberosa, nefronoptisis juvenil, riñon en esponja medular, quistes simples, riñon displásico multiquístico, displasia renal quística, tumor renal quístico multilocular y enfermedad renal quística adquirida.