Hallazgo necrópsico de nefroblastoma asociado a síndrome metabólico experimental / Necropsic finding of nephroblastoma associated to experimental metabolic syndrome

Introducción: El síndrome metabólico presenta entre sus manifestaciones la obesidad, la cual se encuentra relacionada con el desarrollo de cáncer. Sin embargo, no habíamos encontrado en cuatro años ningún caso de neoplasias malignas en animales con síndrome metabólico. Objetivo: Describir el desarrollo de un tumor maligno a nivel renal en modelo experimental de síndrome metabólico. Métodos: El biomodelo experimental se logró por la aplicación de una solución de sacarosa al 35 por ciento, durante 20 semanas a 50 ratas machos Wistar destetados. El diagnóstico de nefroblastoma se realizó mediante necropsia con observación morfológica de la lesión renal. Resultados: Confirmado el síndrome metabólico se detectó en un caso, una masa palpable en abdomen. En la inspección macroscópica se observó un tumor en el polo inferior del riñón derecho, color pardo grisáceo, con hemorragia y cambios quísticos. Histológicamente se observaron alteraciones propias de un nefroblastoma mixto con componentes del blastema, mesenquimal y epitelial. Conclusiones: Se describe por vez primera, en estudio anatomopatológico, la presencia de un caso de nefroblastoma en rata con síndrome metabólico experimental(AU)

Introduction: One of the manifestations of metabolic syndrome is obesity, which is in turn related to the development of cancer. However, in four years we had not found any case of malignant neoplasms in animals with metabolic syndrome. Objective: Describe the development of a malignant renal tumor in an experimental metabolic syndrome model. Methods: The experimental biomodel was made applying a 35 percent saccharose solution to 50 male weaned Wistar rats for 20 weeks. The diagnosis of nephroblastoma was achieved by necropsy with morphological observation of the renal lesion. Results: Upon metabolic syndrome confirmation, a palpable mass was detected in the abdomen of one of the cases. Macroscopic observation revealed a grayish brown tumor in the lower pole of the right kidney with hemorrhaging and cystic changes. Histological examination found alterations typical of mixed nephroblastoma with blastema, mesenchymal and epithelial components. Conclusions: This is the first time a description is provided in an anatomopathological study of a case of nephroblastoma in a rat with experimental metabolic syndrome(AU)

Intra-tumor genetic heterogeneity in Wilms tumor samples

SUMMARY Childhood renal tumors account for ~7% of all childhood cancers, and most cases are embryonic Wilms' tumors (WT). Children with WT are usually treated by either COG or SIOP. The later treats the children using preoperative chemotherapy, but both have around 90% of overall survival in five years. WT is a genetically heterogeneous group with a low prevalence of known somatic alterations. Only around 30% of the cases present mutation in known genes, and there is a relatively high degree of intra-tumor genetic heterogeneity (ITGH). Besides potentially having an impact on the clinical outcome of patients, ITGH may interfere with the search for molecular markers that are prospectively being tested by COG and SIOP. In this review, we present the proposal of the current UMBRELLA SIOP Study 2017/Brazilian Renal Tumor Group that requires the multi-sampling collection of each tumor to better evaluate possible molecular markers, as well as to understand WT biology

RESUMO Os tumores renais pediátricos correspondem a aproximadamente 7% de todos os tumores infantis, sendo o mais frequente o tumor de Wilms (TW). Crianças com TW são geralmente tratadas seguindo dois distintos protocolos terapêuticos (COG ou SIOP), sendo que no último, os pacientes recebem tratamento quimioterápico pré-operatório. Ambos apresentam sobrevida global em cinco anos em torno de 90%. TW é geneticamente heterogêneo, apresentando baixa prevalência de alterações somáticas conhecidas, com cerca de 30% dos casos apresentando mutações em genes conhecidos e um alto grau de heterogeneidade genética intratumoral (HGIT). Além de potencialmente ter um impacto sobre o desfecho clínico dos pacientes, a HGIT pode interferir na busca de marcadores moleculares que estão sendo testados prospectivamente pelos grupos COG e Siop. Nesta revisão, apresentamos a proposta do atual estudo Umbrella Siop 2017/Grupo de Tumores Renais Brasileiros (GTRB), que orienta a coleta de três diferentes regiões do tumor para melhor avaliar possíveis marcadores moleculares, bem como para compreender a biologia do TW.

Nephroblastoma in a black-tufted marmoset (Callithrix penicillata) / Nefroblastoma em um sagui-de-tufos-pretos (Callithrix penicillata)

A renal nephroblastoma is described in a free-living black-tufted marmoset (Callithrix penicillata) in Central Brazil. The monkey was found dead and subjected to necropsy. Gross anatomic changes consisted of a ruptured left kidney, which was almost completely effaced by a white to yellow, partially encapsulated friable mass. The left ureter was distended due to obstruction by a red, spherical, 2mm in diameter friable mass. The urinary bladder was also distended. Histologically the renal and ureteral masses consisted of a triphasic embryonal neoplasm composed of embryonic epithelium forming glomeruli and tubules, polygonal blastemal cells, and a mesenchymal stroma. The embryonic epithelium exhibited rare nuclear immunoreactivity for WT-1, whereas blastemal cells exhibited robust cytoplasmic and rare nuclear immunoreactivity for WT-1; blastemal cells were also immunoreactive for vimentin. No immunoreactivity was detected for pan-cytokeratin (AE1/AE3), actin, and desmin. Morphological and immunohistochemical features of the present neoplasm are consistent with those described for renal nephroblastoma.(AU)

Descreve-se um caso de nefroblastoma maligno em um sagui de vida livre no Brasil Central. O macaco foi encontrado morto e encaminhado para necropsia. Na macroscopia, o rim esquerdo apresentava-se rompido e o parênquima estava substituído por um tecido neoplásico friável, parcialmente encapsulado e de superfície natural branca e de corte amarela. O ureter esquerdo apresentava-se distendido devido à obstrução por uma massa friável, vermelha, esférica, de 2mm de diâmetro. Histologicamente, as massas renal e ureteral consistiam de uma neoplasia embrionária composta por três populaçõies de células neoplásicas, composta por epitélio embrionário formando glomérulos e túbulos, células blastemais poligonais e um estroma mesenquimal. O epitélio embrionário exibiu imunorreactividade nuclear rara para WT-1, enquanto que as células blastemais exibiram imunorreactividade nuclear citoplasmática e rara para WT-1; As células blastemais também foram imunorreativas à vimentina. Nenhuma imunorreatividade foi detectada para pan-citoqueratina (AE1/AE3), actina e desmina. As características morfológicas e imuno-histoquímicas da presente neoplasia são consistentes com as descritas para o nefroblastoma renal.(AU)

Metástases pulmonares em crianças: estamos operando desnecessariamente? / Pulmonary metastases in children: are we operating unnecessarily?

RESUMO Objetivo: determinar, em pacientes pediátricos portadores de neoplasias malignas, as características de nódulos pulmonares identificados à tomografia computadorizada, capazes de diferenciar nódulos benignos de metástases. Métodos: estudo retrospectivo de pacientes submetidos a ressecções pulmonares de nódulos diagnosticados como metástases em um período de sete anos. Achados de tomografia e da cirurgia, assim como resultados dos exames anatomopatológicos foram comparados. Resultados: nove pacientes, submetidos a 11 intervenções cirúrgicas, foram estudados. Entre as variáveis estudadas, apenas o tamanho do nódulo, maior do que 12,5mm provou ser estatisticamente significante para predizer malignidade. Conclusão: esse estudo sugere que, entre as características tomográficas de nódulos pulmonares de crianças portadoras de neoplasias malignas, apenas o tamanho da lesão foi preditor de malignidade.

ABSTRACT Objective: to determine, in pediatric patients with malignant neoplasms, the characteristics of pulmonary nodules identified on computed tomography, as well as the possibility of differentiating benign lesions from metastases. Methods: we conducted a retrospective study of patients submitted to pulmonary resections of nodules diagnosed as metastases in a period of seven years. We compared computed tomography and surgery findings, as well as results of anatomopathological examinations. Results: we studied nine patients submitted to 11 surgical interventions. Among the studied variables, only nodule size greater than 12.5mm proved to be statistically significant to predict malignancy. Conclusion: among the tomographic characteristics of pulmonary nodules in children with malignant neoplasms, only the size of the lesion was a predictor of malignancy.

Descrição de casos de nefroblastoma diagnosticados em um laboratório de anatomia patológica da cidade de São Paulo, Brasil / Description of Nephroblastoma Cases Diagnosed in a Laboratory of Pathologylocated at Sao Paulo City, Brazil / Descripción de los Casos de Nefroblastoma Diagnosticados en un Laboratorio dePatología de la Ciudad de São Paulo, Brasil

Introdução: As neoplasias renais correspondem a 2% dos tumores malignos nos seres humanos. Entre os tumores renais de maior prevalência, o nefroblastoma, também conhecido como tumor de Wilms, é a neoplasia maligna sólida mais comum em crianças. Objetivo: Descrever 15 casos de nefroblastoma (tumor de Wilms) diagnosticados em um laboratório especializado em Anatomia Patológica, localizado na região central da cidade de São Paulo, no período de abril de 2003 a agosto de 2011. Método: Estudo descritivo, retrospectivo, no qual foram avaliados prontuários de pacientes que realizaram exames para diagnóstico de doenças renais. Foram levantados laudos de amostras renais obtidas por nefrectomia ou biópsia, diagnóstico anatomopatológico macro e microscópico, sexo e idade dos indivíduos acometidos, visando a caracterizar a população portadora de tumor de Wilms. Resultados: Em uma amostra de 2.277 pacientes, 15 tiveram seu diagnóstico positivo para nefroblastoma (tumor de Wilms), sendo a maior porcentagem, 67% dos casos, de indivíduos do sexo masculino. Em 46,67% dos casos, o tumor foi classificado com estadiamento I.Conclusão: Conhecer a população normalmente acometida pelo nefroblastoma pode contribuir para um diagnóstico precoce e, consequentemente, melhorar o prognóstico do indivíduo acometido.

Tumores renais e adrenais com invasão cardíaca: resultados cirúrgicos imediatos em 14 pacientes / Renal and adrenal tumors with cardiac invasion: immediate surgical results in 14 patients / Tumores renales y adrenales con invasión cardiaca: resultados quirúrgicos inmediatos en 14 pacientes

FUNDAMENTO: A ressecção do trombo tumoral em veia cava inferior (VCI) e átrio direito (AD) aumenta a sobrevida do paciente com câncer renal/supra-renal. OBJETIVO: Avaliar a conduta cirúrgica do trombo da VCI e AD no tratamento dos tumores renais e supra-renais. MÉTODOS:De janeiro de 1997 a junho de 2007 foram avaliados, retrospectivamente, 14 pacientes tratados cirurgicamente para retirada de trombo em VCI e/ou AD decorrente de tumor renal ou supra-renal. Desses, 64,2% eram do sexo masculino, e havia 42,8% de casos de tumor de Wilms (TW), 28,5% de adenocarcinoma de supra-renal (AS) e 28,5% de carcinoma de células claras (CC), com idades médias de 4,5, 60,5 e 2,5 anos, respectivamente. Aspectos epidemiológicos e parâmetros intra e pós-operatórios hospitalar foram avaliados. RESULTADOS: Em todos os casos encontrou-se trombo tumoral em VCI supra-hepática, e em 62,4% o trombo invadiu o AD. A trombectomia foi realizada com o emprego da circulação extracorpórea associada à hipotermia profunda e parada circulatória total em 85,7% dos casos e moderada no restante. Ligou-se a VCI em 7,1% dos pacientes, e reconstruiu-se por rafia em 92,9%. Os tempos de intubação orotraqueal e internação variaram conforme o tipo de tumor. Ocorreram dois óbitos hospitalares no grupo de AS, por parada cardiorrespiratória intra-operatória. CONCLUSÃO: Existe maior número de casos de trombo tumoral em VCI e AD decorrente de TW. Os casos de AS evoluem com mais complicações no pós-operatório, e o prognóstico no pós-operatório hospitalar dos pacientes com TW é melhor.

BACKGROUND: The resection of tumor thrombus of the inferior vena cava (IVC) and right atrium (RA) increases the survival rate of patients with renal/adrenal cancer. OBJECTIVE: To evaluate the surgical procedure in cases of IVC and RA in the treatment of renal and adrenal tumors. METHODS: Fourteen patients undergoing surgical intervention (during the period) between January 1997 and June 2007, for resection of IVC and/or RA thrombus due to renal or adrenal tumors, were retrospectively evaluated. The patients (64.2% male) presented with Wilms' tumor, clear cell carcinoma and adrenal adenocarcinoma, and had mean age of 4.5, 60.5 and 2.5 years, respectively. Epidemiological characteristics and intra- and postoperative parameters were evaluated. RESULTS: Suprahepatic IVC tumor thrombus were observed in all the patients, and in 62.4% of them the thrombus invaded the RA. Thrombectomy was performed with extracorporeal circulation with deep hypothermia and total circulatory arrest in 85.7%, with mild hypothermia in the remaining cases. The inferior vena cava was ligated in 7.1% of the cases, and reconstruction with suture was performed in 92.9% of the patients. The duration of orotracheal intubation and length of hospital stay were different, according to the tumor type. Two deaths, due to intraoperative cardiorespiratory arrest, were seen among patients with adrenal adenocarcinoma. CONCLUSION: IVC and RA tumor thrombi are more frequent in patients with Wilms' tumor. More postoperative complications are seen in patients with adrenal adenocarcinoma, and the postoperative prognosis is better for patients with Wilms' tumor.

FUNDAMENTO: La resección del trombo tumoral en vena cava inferior (VCI) y atrio derecho (AD) aumenta la sobrevida del paciente con cáncer renal/ suprarrenal. OBJETIVO: Evaluar la conducta quirúrgica frente al trombo de la VCI y AD en el tratamiento de los tumores renales y suprarrenales. MÉTODOS: De enero de 1997 a junio de 2007, se evaluaron, retrospectivamente, a 14 pacientes tratados quirúrgicamente para retirada de trombo en VCI y/o AD que transcurre de tumor renal o suprarrenal. De estos, el 64,2% eran del sexo masculino; había el 42,8% de casos de tumor de Wilms (TW), el 28,5% de adenocarcinoma suprarrenal (ACS) y el 28,5% de carcinoma de células claras (CC), con edades promedio de 4,5, 60,5 y 2,5 años, respectivamente. Se evaluaron los aspectos epidemiológicos y también los parámetros hospitalarios intra y postoperatorios. RESULTADOS: En todos los casos se encontró trombo tumoral en VCI suprahepática, y en el 62,4% el trombo invadió el AD. Se realizó la trombectomia con el empleo de la circulación extracorpórea asociada a la hipotermia profunda; se verificó paro circulatorio total en el 85,7% de los casos, mientras que se mantuvo moderada en el restante del grupo. Se procedió a la ligadura de la VCI en el 7,1% de los pacientes, y se la reconstruyó por rafia en el 92,9%. Los tiempos de intubación orotraqueal e internación variaron conforme el tipo de tumor. Ocurrieron dos óbitos hospitalarios en el grupo de ACS, provocados por paro cardiorrespiratorio intraoperatorio. CONCLUSIÓN: Existe mayor número de casos de trombo tumoral en VCI y AD que transcurre de TW. Los casos de ACS evolucionan con más complicaciones en el período postoperatorio, mientras que el pronóstico en el postoperatorio hospitalario de los pacientes con TW resulta mejor.

Lactante con masa abdominal: teratoma renal vs. nefroblastoma teratoide / Lactant with abdominal mass: teratoma vs. nebroblastoma teratoid

El riñón es uno de los sitios menos comunes de localización de teratomas y otros tumores germinales. Los teratomas extragonadales son más frecuentes en la región sacro coccígea y en el mediastino. Hace once años en el Instituto Autónomo Hospital Universitario de los Andes, Mérida, Venezuela, fue atendida una lactante femenina de 3 meses de edad, con masa abdominal de crecimiento progresivo. La radiografía y el ultrasonido abdominal mostraron masa tumoral izquierda, con calcificaciones e imágenes de aspecto anecoico. La exploración quirúrgica demostró neoplasia quística con compromiso del riñón izquierdo. El estudio anatomopatológico reportó riñón izquierdo con neoplasia quística, multiloculada y luz ocupada por abundante líquido amarillo claro, material sebáceo, sangre y pelos. Quistes revestidos por tejidos maduros tipo epitelio plano estratificado con calcificación, queratina y epitelio mono-estratificado ciliado respiratorio, cartílago y tejido adiposo. El teratoma maduro renal es una neoplasia benigna muy infrecuente pero de buen pronóstico. El diagnóstico diferencial debe realizarse con varias neoplasias benignas y malignas de ubicación en retroperitoneo. Durante la infancia el diagnóstico diferencial más importante es con el nefroblastoma teratoide. El tratamiento de elección es el quirúrgico. La evolución del paciente motivo de este trabajo transcurrido diez años es satisfactoria.

Very rarely teratomas and tumors derived from germ cell can arise within the kidney. Extragonadal teratomas are most frequent in the mediastinum and sacrococcygeal region. In the University Hospital of The Andes Mérida, Venezuela was treated a three month old female infant with an abdominal mass. The X-ray films and the ultrasound study showed a big retroperitoneal tumor of left kidney and it was heterogeneous and had amorphous calcifications. In the surgery procedure, a multicystic tumor within the kidney parenchyma was found; the light was occupied with clear yellow liquid and fat, sebaceous and mucinous materials, hair, and blood. A cyst was involved with mature cells type stratificated with calcification queratine and epithelium mono-estratificated, ciliate respiratory, cartilage and sebaceous tissues. The teratoma renal is an uncommon neoplasm with a benign clinical course and a good prognosis. The differential diagnosis should be with some neoplasm retroperitoneal benign and malignant. During the infancy the histopathologic differential diagnosis with teratoid nephroblastoma is a mandatory. Surgery procedure should thus be the first choice for treatment. Ten years later, the evolution of the patient motive of studied in these work was satisfactory, actually she has no evidence of any tumor recurrence.

Imunoexpressão de WT1, ß-cateinina, E-caderina e APC em tumor de Wilms esporádico / Expression of WT1, p53, Beta-catenina, E-cadherina and APC in sporadic Wilms tumor

O tumor de Wilms é uma neoplasia embrionária, constituída classicamente por três componentes histológicos: blastema, epitélio e mesênquima (estroma), os quais podem ser encontrados em diferentes proporções em cada tumor. ... Este estudo tem como principal objetivo determinar a freqüência da expressão das proteínas WT1, p53, Beta-catenina, E-caderina e APC nos diferentes componentes histológicos do tumor de Wilms, assim como correlacionar estes achados com o prognóstico. Para estudar a expressão dos marcadores, foi utilizado a imunoistoquímica, através da construção de Tissue Macroarray (TMA), o qual permite a análise de muitas amostras teciduais em uma única lâmina, otimizando custo e tempo. Nos nossos resultados, WT1 e p53 foram mais freqüentemente expressos no componente blastema (WT1: 97% e p53: 63% dos casos) e epitélio (WT1: 87% e p53: 58% dos casos), quando comparados com o estroma (WT1: 13% e p53: 22% dos casos) e com o tecido renal não tumoral (ausência de expressão em todos os casos estudados). ... A imunoexpressão nuclear ocorreu principalmente nos componentes blastematoso e estromal. Houve imunoexpressão de E-caderina na membrana em 47% dos casos, em citoplasma em 44% e em núcleo em 12%. O acúmulo em núcleo foi encontrado principalmente nos componentes blastema e mesênquima, 21 e 25% respectivamente. A imunoexpressão do APC ocorreu em núcleo em 95% dos tumores avaliados, em citoplasma em 9% e nenhum apresentou expressão em membrana. Nos componentes blastematoso e epitelial houve imunopositividade nuclear em 100% dos casos avaliados. No componente mesenquimal a positividade nuclear ocorreu em 89% dos casos. Os pacientes com estádios precoces tiveram mais freqüentemente positividade do WT1. Não houve associação dos outros fatores clínicos, epidemiológicos e evolução dos pacientes com expressão dos marcadores estudados.

Wilms tumor is a triphasic malignant neoplasm compromised of variable proportions of epithelial, blastemal and mesenchymal (stromal) elements. Different components have different clinical behavior. Patients with tumors predominant epithelial and/or stromal has more frequently low stage while predominant blastema has advanced disease. This study was undertaken to evaluate the expression of WT1, beta-catenins, E-cadherin and APC in Wilms tumor and correlate this expression with clinical characteristics, histologic cell type and prognosis. We studied the immunohistochemical expression of WT1, p53, beta-catenins, E-cadherin in different component of Wilms tumor using a tissue array. WT1 and p53 were more often expressed on blastema component (WT1: 97% and p53: 63% of cases) and on epithelial (WT1: 87% and p53: 58% of cases), when compare with the stromal component (WT1: 13% and p53: 22% of cases) and the normal renal tissue where it was not expressed. Immunopositivity of WT1 and p53 were significantly correlated. Immunopositivity of ß-catenina was seen in 85 % at the membrane (85%), 94% cytoplasm and 24% nuclear. Nuclear immunoexpression was detected specially on blastema and stromal component. Immunohistochemical expression of E-cadherina was detected in 47% at the membrane, 44% cytoplasm and 12% nuclear. Nuclear expression was seen more often at the blastema and stromal component (21%, 25% respectively). Immunopositivity of APC occurs at 95% on nucleus, cytoplasm (9%) and none at membrane. Among the blastema and the epithelial component 100% was positive on nucleus. On the stromal component 89% was positive at the nucleus. Immunoexpression of WT1 was correlated with local stage. The expression status of WT1, E-cadherin, ßcatenina, APC in this cohort was not of prognostic value.

Intracaval and intracardiac extension of Wilms' tumor: the influence of preoperative chemotherapy on surgical morbidity

OBJECTIVES: The aim of this retrospective study is to compare surgical complications and long-term survival in children with Wilms' tumor (WT) and tumor thrombus receiving or not preoperative chemotherapy MATERIALS AND METHODS: Review of the charts of 155 children with WT treated between 1983 and 2005, and analysis of 16/155 (10.3 percent) children with WT who presented cavoatrial tumor extension, being 8/16 IVC and 8/16 atrial thrombus RESULTS: Median age was 54 months. 2/16 had cardiac failure as the first symptom. 11/16(7 IVC and 4 atrial extension) (67 percent) were submitted to preoperative chemotherapy with vincristine plus actinomycin D, and 5/16(1 IVC and 4 atrial) (33 percent) underwent initial nephrectomy and thrombus resection. So, 11 patients were submitted to preoperative VCR/ACTD and 2/11 (18.1 percent) had complete regression of the thrombus, 6/11(54.5 percent) partial regression and 3/11 (27 percent) had no response. Among the partial responders, nephrectomy with thrombus removal was performed in all, including one patient with previous intracardiac involvement, without extracorporeal circulation procedures. In two of the three non-responders, cardiopulmonary bypass was necessary for thrombus removal. There were no surgical related deaths. Long-term survival is 91 percent in the group submitted to preoperative chemotherapy and 100 percent in the group who had surgery as first approach CONCLUSION: Preoperative chemotherapy was able to reduce thrombus extension in 8/11 (73 percent) treated patients and cardiopulmonary bypass was avoided in 2 patients with atrial thrombus. Surgical resection of tumor and thrombus was successful in all cases, receiving or not preoperative chemotherapy and overall survival was similar in both groups.

Tumor de Wilms: características clínicas e cirúrgicas / Wilms'tumor: clinical and surgical features

O tumor de Wilms (TW) é o tumor renal maligno mais comum na infância. O conhecimento de certas características clínicas e a realização de procedimentos cirúrgicos adequados podem ter impacto no prognóstico desta doença. Revisados prontuários de pacientes com diagnóstico de TW entre 1989 e 2005. Coletados dados demogrãficos, caracteríisticas clínicas e avaliação de procedimentos cirúrgicos. Durante o ato operatório, 38 pacientes foram submetidos à avaliação do rim contralateral através da palpação e em 13 relatos de cirurgia não foram encontradas descrições. Ruptura tumoral em 1 paciente; em dez prontuários havia registro de ausência de ruptura tumoral e em 41 prontuários não havia qualquer menção quanto à presença ou ausência dessa complicação. A histopatologia confirmou 45 casos de histologia favorável e os demais de histologia desfavorável. Os resultados apresentados permitem concluir que os pacientes estudados apresentam características demográficas gerais semelhantes aos da literatura.Considerando-se que em um número expressivo de pacientes observou-se falta de aderência a certas etapas do procedimento cirúrgico, incluindo ausência de biópsia de linfonodos e atrasos na realização da ressecção tumoral, os autores recomendam que o cirurgião pediátrico tenha uma participação mais efetiva na equipe multidisciplinar e na elaboração das rotinas do protocolo cirúrgico para pacientes com TW

Wilms´tumor (WT) is the most common malignant renal tumor in childhood. The knowledge of the clinical characteristics and the accomplishment of standard surgical procedures may have an impact in the prognosis of this disease. Medical records of newly diagnosed WT patients treated from 1989 to 2005 were reviewed. We collected data on demographics, clinical characteristics and whether certain recommended surgical standard procedures were carried out.The surgeon in 38 patients performed palpation of the contralateral kidney and in 13 medical records there was no report whether this procedure was carried out. Tumor spillage was reported 1 patient, reported as absent in 10 patients; we were unable to find any mention about tumor rupture on the surgeon’s report for 41 patients. There were 45 cases of favorable histology and 7 of unfavorable histology. The OS was of 69%, 71%, 79%, 50% and 40% for the stages I, II, III, IV and V, respectively. Five years OS was 73% and 65,2% for patients submitted to surgery before and after the 6th week after diagnosis, respectively. The results of the present study indicate that patients in this study show demographic characters similar to the literature. Considering that surgeons did not performed standard recommended surgical procedure such as lymph nodes biopsy and carried out late surgical resection of the primary tumor in many patients, there is a need for a more effective participation of the surgeon in the multidisciplinary team and possiblyin the designing protocols for the surgical management of patients with TW

Trombo tumoral en aurícula derecha en tumor de Wilms / Tumoral thrombus in right auricle in Wilms tumor

En el tumor de Wilms (TW) el trombo cavo atrial (TCA) es < 5 por ciento, siendo rareza el compromiso auricular derecho. Revisamos los abordajes quirúrgicos del TCA en el TW. Materiales y métodos: Paciente de 3 años y medio con tumor renal derecho de 10 cm, un cava inferior ocupada por trombo tumoral hasta la aurícula derecha. Además tumor en la vena renal izquierda y cava infra renal. Se utiliza quimioterapia preoperatoria previa biopsia por punción. Resultados: seis semanas de quimioterapia y un TAC demostró reducción 10 por ciento del tumor y menor TCA. Se realizó nefrectomía radical derecha con trombectomía cava abdominal y renal izquierda combinada con trombectomía cava toráxica y auricular, con paro cardiopulmonar, circulación extracorpórea e hipotermia. Evolucionó sin complicaciones, la biopsia no demostró tumor en el riñón o TCA. Se catalogó TW etapa III sin anaplasia y entró en un protocolo DD4A del NWTSG. A 1 mes de la cirugía el TAC mostró ausencia de tumor. Conclusiones: El compromiso tumoral de la cava y aurícula derecha es excepcional en TW. Los mejores resultados y menor morbilidad están asociados a quimioterapia preoperatoria y buena planificación de la vía de abordaje.

Introduction: In the tumor of Wilms (TW) the thrombus cava atrial (TCA) it is < 5%, being exceptional the atrial right involvement. We check the surgical routes of access of the TCA in the TW. Materials and methods: 3-year-old patient with renal right tumor of 10 cm, a inferior vena cava occupied by tumor thrombus up to the right auricle. Also tumor in the renal left vein and vena cava under the kidney. We use preoperative chemotherapy previous biopsy for puncture. Results: 6 weeks of chemotherapy and a TAC demonstrated reduction 10% of the tumor and decrease TCA. We realized radical right nephrectomy with thrombectomy abdominal cava and renal left combined with thrombectomy thorax cava and atrial right, with cardiopulmonar unemployment, extracorporeal circulation and hypothermia. She evolved without complications, the biopsy did not demonstrate tumor in the kidney or TCA. TW catalogued stage the IIIrd without anaplasia and she entered a protocol DD4A of the NWTSG. To 1 month of the surgery the TAC showed absence of tumor. Conclusions: The tumor commitment of the vena cava and right auricle is exceptional in TW. The best results and minor morbidity are associated with preoperative chemotherapy and good planning of the routes of access.

Prognóstico de pacientes com tumor de Wilms unilateral no Rio de Janeiro, 1990-2000 / Prognosis for patients with unilateral Wilms' tumor in Rio de Janeiro, Brazil, 1990-2000

OBJETIVO: Analisar a sobrevida e os principais fatores prognósticos entre os pacientes com tumor de Wilms unilateral. MÉTODOS: A coorte de estudo incluiu 132 casos de tumor de Wilms unilateral em menores de 15 anos de idade matriculados em serviço de oncologia pediátrica, de janeiro de 1990 a dezembro de 2000. Curvas de sobrevida foram confeccionadas utilizando-se o método de Kaplan-Meier e fatores prognósticos foram analisados pelo modelo de riscos proporcionais de Cox. RESULTADOS: A estimativa de sobrevida global em cinco anos foi 84,6 por cento. As probabilidades de sobrevida para os estádios I, II, III e IV foram de 100 por cento; 94,2 por cento; 83,2 por cento e 31,3 por cento, respectivamente. A taxa de sobrevida para os pacientes com: histologia favorável foi de 89,4 por cento, para aqueles com anaplasia focal 66,7 por cento e com anaplasia difusa 40 por cento. Todos os pacientes com doença em estádio IV e anaplasia difusa foram a óbito (n=4). Todos os pacientes com doença em estádio I, independente da histologia, permaneceram vivos até o final do período de seguimento. CONCLUSÕES: Entre as variáveis escolhidas para o modelo final apenas o estadiamento e a histologia permaneceram associados ao elevado risco de óbito enquanto que os casos na faixa etária entre 24 e 47 meses apresentaram melhor prognóstico que os demais. Esses resultados mostram a importância do diagnóstico em fases iniciais da doença e que a histologia é fundamental para orientar a terapia adequada.

Wilms' tumour in adults: a report of three cases.

Wilms' tumour is the most common pediatric renal neoplasm. Its occurrence in adults is rare, less than 1% of all nephroblastomas have been reported after the age of 15 years of age. We report 3 cases of Wilms' tumour in adults, their clinico-pathological profile and review the literature including the cases reported from India. Patients were young adults, their age ranging from 25 to 32 years. The largest tumour dimension was between 11 cm to 17 cm. Two cases were blastema predominant while the third case had typical triphasic histology. All cases were stage II (National Wilms' Tumour Study), favourable histology. Although Wilms' tumour in adults is morphologically similar to its childhood counterpart, it often responds poorly to the combination chemotherapy. The criteria for its diagnosis are strict and should be adhered to for its correct identification.

Update on Wilms' tumor in children

Although rare, Wilms' tumor is the most common primary renal malignancy in children and is associated with a number of congenital anomalies and documented syndromes. Appropriate laboratory, radiologic and pathologic investigations are necessary components of the evaluation of children with suspected Wilms' tumor. This provides accurate diagnosis and subsequent staging; information which is essential to generate a multidisciplinary treatment plan utilizing surgery, chemotherapy and radiotherapy. Patients treated for Wilms' tumor as children must continue to be monitored for possible long-term sequelae as adults including secondary malignancies as well as treatment-related toxicity

Multicystic nephroma--report of two cases.

Multicystic Nephroma (MCN) is an uncommon renal pathology, characterized by the presence of usually unilateral circumscribed focal lesion consisting of multiple thin walled cysts. The etiology and pathogenesis of multicystic nephroma is not clear, and it is considered as a neoplastic lesion by many authors. To the best of our knowledge it has not yet been reported from India. We report first two pediatric cases of MCN from India. Computerized Tomography (CT) scan in both the cases revealed a unilateral cystic lesion in the lower pole of kidney. Keeping in mind the age, clinical presentation and radiological appearance, a possibility of Wilm 's tumour with cystic change could not be ruled out preoperatively and both children underwent nephrectomy. Since MCN has a benign behaviour it must be differentiated from focal cystic neoplastic lesions, including Cystic Partially Differentiated Nephroblastoma (CPDN), which has a low but distinct capability for local recurrence, and from Wilm's tumour with cystic change. Segmental form of unilateral renal dysplasia also needs to be considered in the clinical and radiological differential diagnoses.

Tumor de Wilms extra-renal: relato de caso / Extrarenal Wilms' tumor: a case report

Um caso de tumor de Wilms extra-renal de localização retroperitoneal em uma paciente do sexo feminino de dois anos de idade é apresentado, associado a revisão de literatura. Foram realizados exames de radiografia simples do abdome, urografia excretora, ultra-sonografia e tomografia computadorizada sem e com contraste, que evidenciaram a presença de massa retroperitoneal adjacente ao rim direito. A paciente foi submetida a intervenção cirúrgica, com ressecção de toda a massa, sendo o diagnóstico de tumor de Wilms confirmado com exame anatomopatológico. O tumor de Wilms extra-renal é uma entidade extremamente rara e maligna, descrito na literatura principalmente sob a forma de relato de caso. Pode ocorrer no retroperitônio, útero e ovários, canal inguinal, testículos, pele, e até mesmo no tórax. O mecanismo exato que poderia explicar a ocorrência deste tumor em tecido extra-renal não foi bem estabelecido ainda. O diagnóstico é feito através do estudo anatomopatológico da lesão, geralmente após intervenção cirúrgica.

The authors report a case of a two year-old girl with an extrarenal Wilms' tumor in the retroperitoneum. Abdominal plain films, intravenous urography, abdominal ultrasound and computed tomography examinations showed a retroperitoneal mass adjacent to the right kidney. The patient underwent surgery with complete resection of the mass. The diagnosis of Wilms' tumor was confirmed by histopathological study. Extrarenal Wilms' tumor is an extremely rare malignancy which is almost always presented in medical literature as a case report. The tumor may arise in the retroperitoneum, uterus and ovaries, inguinal groove, testes, skin, and even in the thorax. The exact mechanism of occurrence of this tumor in extrarenal tissues has not yet been established. The diagnosis relies on histopathological study, generally after surgical intervention.