Tumor de Wilms: estudio de centro único de Los Andes Ecuatorianos: serie de casos con seguimiento / Wilms' tumor: single center study from the Ecuadorian Andes: case series with follow-up

El Tumor de Wilms (TW) es el tumor renal más frecuente entre los 1 y 5 años de edad. La evidencia existente respecto de aspectos clínicos, terapéuticos y de supervivencia (SV) del TW es escasa. El objetivo de este estudio fue determinar diferencias en la SV actuarial global (SVAG) y SV libre de enfermedad (SVLE) a 5 años en pacientes con TW tratados con quimioterapia neoadyuvante (QTNA) y cirugía inicial (CI). Serie de casos. Se incluyeron pacientes con TW de 11 meses y 13 años de edad, tratados en el Instituto del Cáncer SOLCA, Cuenca (1994-2019). Las variables resultado fueron SVAG y SVLE a 5 años. Otras variables de interés fueron: localización, estadio, histología, seguimiento y remisión completa (RC). Una vez concluidos sus tratamientos, los pacientes fueron sometidos a un seguimiento clínico. Se utilizó estadística descriptiva (medidas de tendencia central y dispersión) y analítica (Chi2, exacto de Fisher y corrección por continuidad). Se realizaron análisis de SV con curvas de Kaplan Meier y log-rank. Se reclutaron 36 pacientes (52,8 % hombres), con una mediana de edad de 44 meses; 55,5 % de ellos tuvieron histología favorable. La localización y estadio más frecuente fue riñón izquierdo (55,5 %) y I (33,3 %) respectivamente. El 58,3 % fueron sometidos a CI y el 41,7 % QTNA. Luego de aplicados los tratamientos 21 pacientes (58,3 %), alcanzaron RC. La SVAG y SVLE general a 5 años fue 72,0 % y 69,0 % respectivamente. Al comparar los subgrupos con QTNA y CI; se verificaron SVAG y SVLE a 5 años de 60,0 % y 81,0 % (p=0,118); y de 66,7 % y 71,4 % (p=0,536) respectivamente. La SVAG y SVLE verificadas son similares a las reportadas en otros estudios. No se evidenciaron diferencias de éstas con los tratamientos QTNA y CI.

Wilms tumor (WT) is the most common pediatric kidney tumor between 1 and 5 years of age. The existing evidence regarding clinical, therapeutic and survival (SV) aspects of TW is scarce. The aim of this study was to determine differences in 5-year overall survival (OS) and 5-year disease-free survival (DFS), in patients treated by WT with neoadjuvant chemotherapy (NACT) and initial surgery (IS). Case series. Patients with TW between 11 months and 13 years of age, treated at SOLCA Cancer Institute, Cuenca, Ecuador (1994-2019) were included. The outcome variables were OS and DFS. Once their treatments were completed, patients were followed clinically. Descriptive (measures of central tendency and dispersion) and analytical (Chi2, Fisher's exact and continuity correction) statistics were applied. SV analysis with Kaplan Meier curves and log-rank were performed. 36 patients (52.8 % men), with a median age of 44 months; 55.5 % of which had favorable histology were recruited. The most frequent location and stage was left kidney (55.5 %) and I (33.3 %) respectively. 58.3 % underwent IC and 41.7 % QTNA. After treatments, 21 patients (58.3 %) achieved complete remission. General OS and DFS were 72.0 % and 69.0 % respectively. When comparing subgroups with QTNA and CI. When comparing the subgroups with QTNA and CI, OS and DFS of 60.0 % and 81.0 % were verified (p=0.118); and of 66.7 % and 71.4 % (p=0.536) respectively. General OS and DFS observed are similar to those reported in other studies. No differences were evidenced with QTNA and CI treatments.

Síndrome de Beckwith˗ Wiedemann / Beckwith-Wiedemann syndrome

El síndrome de Beckwith-Wiedemann es caracterizado por presentar onfalocele, macroglosia, visceromegalias e hipoglucemia neonatal además de una gran diversidad de anomalías clínicas y de laboratorio. Esta enfermedad también se conoce como síndrome de onfalocele, macroglosia y gigantismo. Los problemas más significativos relacionados con la anestesia son hipoglicemia y macroglosia. Es imperativo realizar una evaluación preanestésica que incluya el sistema cardiovascular, sistema urinario, así como la vía aérea. Los niños con este síndrome pueden requerir diferentes procedimientos quirúrgicos. Se debe pronosticar un abordaje difícil de la vía respiratoria debido al crecimiento de la lengua que puede causar dificultad durante la ventilación y/o intubación endotraqueal. S debe monitorizar la glicemia perioperatoria para evitar secuelas neurológicas secundarias a hipoglicemia no diagnosticada. Se reporta el tratamiento perianestesiológico de un niño de cuatro años de edad con síndrome de Beckwith-Wiedemann que requirió tratamiento quirúrgico de un tumor de Wilms. Después de una evaluación minuciosa, se realizó intubación orotraqueal con un tubo 5.0 el cual se introdujo con facilidad bajo inducción con ketamina-vecuronio. La anestesia se mantuvo sin incidentes con isoflurano y fentanilo(AU)

Beckwith-Wiedemann syndrome is characterized by omphalocele, macroglossia, visceromegaly and neonatal hypoglycaemia, as well as a great diversity of clinical and laboratory abnormalities. This disease is also known as omphalocele, macroglossia and gigantism syndrome. The most significant problems related to anesthesia are hypoglycemia and macroglossia. It is imperative to perform a pre-anesthetic evaluation that includes the cardiovascular system, the urinary system, as well as the airway. Children with this syndrome may require different surgical procedures. A difficult approach to the airway should be predicted due to the growth of the tongue which can cause difficulty during ventilation and/or endotracheal intubation. Perioperative glycemia should be monitored in order to avoid neurological sequelae secondary to undiagnosed hypoglycemia. We report the perianesthesiological treatment of a four-year-old boy with Beckwith-Wiedemann syndrome who required surgical treatment for Wilms' tumor. After a thorough evaluation, orotracheal intubation was performed with a 5.0 tube, which was easily introduced with ketamine-vecuronium induction. Anesthesia was maintained without incident with isoflurane and fentanyl(AU)

Compromiso renal de los pacientes con tumor de Wilms bilateral / Kidney involvement in patients with bilateral Wilms tumor

El tumor de Wilms (TW) es el tumor renal maligno más frecuente en la infancia. Entre el 5-7% de los pacientes se presentan con enfermedad TW bilateral, de forma sincrónica o metacrónica. El TW bilateral generalmente ocurre en pacientes pequeños y más frecuentemente en niñas. La cirugía es un componente crítico en el tratamiento para lograr una alta tasa de curación preservando la mayor cantidad de masa renal. La quimioterapia preoperatoria a menudo reduce significativamente el tamaño del tumor, facilitando la cirugía posterior. Los pacientes con TW bilateral deben ser tratados en centros con experiencia en esta patología. El manejo de estos pacientes es aún un desafío (AU)

Wilms tumor (WT) is the most common malignant kidney tumor in childhood. Between 5-7% of the patients present with synchronous or metachronous bilateral WT. Bilateral WT typically occurs in young patients and most frequently in girls. Surgery is a critical component of the treatment as it has a high rate of cure while preserving the largest kidney mass. Preoperative chemotherapy often significantly reduces the size of the tumor, facilitating subsequent surgery. Patients with bilateral WT should be managed at centers with experience treating this disease. The management of these patients is still a challenge (AU)

Estenosis actínica de colon 22 años después / Actinic colon stenosis after 22 years

La estenosis actínica del intestino se produce en ocasiones después del tratamiento con radioterapia de distintos tumores del abdomen o de zonas vecinas, afecta la mucosa del colon y es más frecuente la que aparece en algunos pacientes que han recibido radioterapia para el tratamiento de tumores de la región pélvica durante muchos meses, o incluso años después. Presentamos a un paciente masculino de 34 años de edad, que desarrolló una estenosis colónica 22 años después de tratamiento radiante y quimioterapia por un tumor de Wilms, que requirió resección quirúrgica(AU)

Actinic stenosis of the intestine sometimes occurs after the radiotherapy applied to different tumors in the abdominal area or surrounding areas, it affects the colon mucosa and is more frequent in some patients who have been treated with radiotherapy to manage tumors of the pelvic region after many months or even years. Here is a male patient aged 34 years, who developed colon stenosis after 22 years of having been treated with radiation and chemotherapy since he had a Wilms' tumor that required surgical excision(AU)

Tumor de Wilms Extrarenal: reporte de un caso / Extrarenal Wilm's tumor: a case report

Se presenta el caso de un lactante varón de 1 año y 9 meses de edad que consultó por dolor abdominal encontrándose al examen clínico una masa abdominal. Se le efectuaron estudios de laboratorio incluyendo marcadores tumorales que fueron de rango normal, radiología simple de abdomen así como ecografía abdominal y TAC abdominal que mostraron una tumoración mixta con componente básicamente quístico sin calcificaciones, de localización retroperitoneal en el espacio paracavo y adyacente al riñón derecho al que comprime y desplaza en sentido posterior y caudal causándole moderada hidronefrosis así como desplazamiento medial de la cabeza del páncreas y duodeno. Se resecó el tumor en su totalidad demostrando el estudio anatomopatológico se trataba de un Tumor de Wilms extrarenal. Recibió un ciclo de quimioterapia con Vincristina y Actinomicina D. A la fecha tiene más de seis años de seguimiento, los estudios por urotomografía demuestran normalidad de ambos riñones y total ausencia de presencia tumoral encontrándose totalmente libre de molestias. El tumor de Wilms es el tumor maligno urogenital más frecuente de la infancia sin embargo su localización extrarenal es extremadamente rara excluyendo por definición la presencia de un tumor renal primario. Pueden ser componentes de otros tumores generalmente teratomas o ser lesiones aisladas que se originan de elementos del mesodermo primitivo tales como restos de los cuerpos de Wolffian y del blastema mesonéfrico. Se localiza con mayor frecuencia en el retroperitoneo habiéndose publicado casos de presentación en región inguinal, útero, testiculos, región lumbosacra y sacrocoxígea recomendándose en su manejo seguir los mismos protocolos de los tumores de Wilms intrarenales sobretodo los de formas quísticas, de buen pronóstico.

Revisão sobre tumor de Wilms / Review of Wilms' tumor

Os autores fazem uma revisão bibliográfica sobre Tumor de Wilms: sua epidemiologia, classificação, diagnóstico, estadiamento e tratamento.

Nephron-sparing surgery for bilateral wilms' tumours: a single-centre experience with 23 cases

Introduction: The challenge of management with bilateral Wilms' tumours is the eradication of the neoplasm; while at the same time preserving renal function. Surgical management with a variety of nephron-sparing techniques; combined with chemotherapy and occasionally supplemented by transplantation has evolved over the last 30 years to achieve remarkable success. We document the experience of a single centre in a developing country. Material and Methods: Twenty-three bilateral Wilms' tumours were seen in our service between 1981 and 2007. Treatment was; in most cases; according to National Wilms' Tumour Study Group protocols; with initial bilateral biopsy; neoadjuvant chemotherapy; and tumourectomy. Technique of nephrectomy included full mobilization of the tumour-involved kidney; topical cooling with slush ice; vascular exclusion; tumour resection and reconstruction of the remnant kidney. Results: Twelve patients are alive and free of disease one to 15 years after treatment; all with well-preserved renal function (lowest glomerular filtration rate was 65 ml/min per (1.73 m 2 ). None of the survivors have hypertension. Eleven have died (two of unrelated disease) including six of the seven with spread outside the kidney. All three with unfavourable histology are alive. Four of the five metachronous presentations are alive; as are eight of 12 patients with synchronous bilateral tumours who presented since 2000. Conclusions: Appropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal function in nearly all cases. Unfavourable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis

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.

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.

Results of novel strategies for treatment of Wilms' tumor

OBJECTIVE: To evaluate treatment outcomes in Wilms' tumor (WT). MATERIALS AND METHODS: We studied 53 children with median age of 2 years with WT, stages I-19, II-14, III-12, IV-6 and V-2. Treatment consisted of surgical excision plus adjuvant (40 children) or neoadjuvant and adjuvant chemotherapy (unresectable tumor, n = 8, or caval tumor extension, n = 5). Chemotherapy and radiotherapy followed protocols of Brazilian Wilms' Tumor Study Group excepting 16 cases with stage I disease that received a short duration postoperative treatment with vincristine (VCR - 11 doses) and dactinomycin (AMD - 4 doses). Relapsed WT was treated with multiagent regimens including cisplatin/carboplatin, cyclophosphamide, ifosfamide and etoposide. One patient with resistant relapsed WT was treated by high-dose conditioning chemotherapy with stem cell rescue. RESULTS: Overall and disease-free survival rates at 5 years were respectively 88.2 ± 5.0 percent and 76.7 ± 6.6 percent. Short duration therapy for stage I tumor showed a disease-free survival rate of 100 percent in a median time of 101 months (range 14 to 248 months). Overall and disease-free survival of 10 patients with recurrent WT at 5 years was 42.8 percent. The child treated with high-dose chemotherapy plus stem cell transplant is alive without evidence of disease 84 months from relapse. CONCLUSION: The postoperative chemotherapy in stage I disease can be reduced without compromising the cure rate. The treatment of unfavorable stage III and IV disease or relapsed tumor remains a challenge.

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.

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.

Tumor de Wilms em adulto / Adult Wilms tumor

Contexto: o tumor de Wilms em adulto é uma doença rara. Apresenta prognóstico pior que o tumor de Wilms na infância. Os autores relatam um caso de tumor de Wilms em paciente adulto que foi tratada com quimioterapia e nefrectomia parcial. Relato de Caso: a paciente apresentava-se com estado geral comprometido e ascite volumosa há um mês da admissão. A tomografia computadorizada de abdome revelou a presença de lesão sólida situada entre o rim e o fígado e a biópsia percutânea foi compatível com neoplasia de pequenas células. A paciente recebeu seis ciclos de taxol e cisplatina. O estudo imunoistoquímico sugeriu tratar-se de tumor de Wilms metastático. A paciente evoluiu com desaparecimento completo da ascite e persistência da massa residual retroperitoneal, que foi ressecada após o termino da quimioterapia. A paciente apresentou recidiva retroperitoneal e pulmonar dez meses após o termino do tratamento. Atualmente, encontra-se em vigência de quimioterapia. Conclusão: o tumor de Wilms em adulto apresenta uma evolução geralmente desfavorável.

Proteína p53 em nefroblastomas / P53 protein in nephroblastomas

A imunoexpressão do antígeno p53 foi estudada em 35 pacientes com idade média de 38 +/- 29 meses. Todos foram tratados com nefrectomia e quimioterapia, em 17 se associou também a radioterapia. O tempo médio de seguimento foi de 69 +/- 66 meses. Em 5 deles a histologia era defavorável. Em 10 casos (28,5 por cento) a marcação foi positiva, com ordem crescente de positividade para epitélio, blastema e estroma. Cinco pacientes faleceram da doença, nenhum deles com histologia desfavorável. Não se encontrou relação significante entre o padrão imunohistoquímico e os parêmetros seguintes: sobrevida dos pacientes, estádio e grau do tumor.

Primary extrarenal wilms' tumor in the retroperitoneum

Extrarenal Wilms' tumor is extremely rare. Approximately 54 cases have been reported in the world literature through 1993. Only eight extrarenal Wilms' tumors were enrolled in the National Wilms' Tumor Study [NWTS] between 1980 and 1986. These cases constituted a fraction of 1% of all Wilms' tumors reported to the N.W.T.S during this period. The authors report a case of retroperitoneal Wilms' tumor [Stage III] presenting as a lower abdominal mass in a previously healthy 6-year-old girl who was operated and staged according to the current N.W.T.S III criteria

Avaliaçäo do papel do cirurgiäo no tratamento do tumor de Wilms: análise de um estudo cooperativo / The role of surgery in the treatment of Wilms tumor: analysis of cooperative study

Objetivo. Detectar os efeitos do tratamento e estadiamento cirúrgicos nos resultados obtidos e verificar a possível relação entre tais resultados e o cumprimento das normas do protocolo cirúrgico preestabelecido (segundo as normas do NWTS). Casuística e Método. A casuística é composta de 166 pacientes de 1986 e dezembro de 1988, com dados atualizados até fevereiro de 1992. O período mínimo de acompanhamento foi de 24 meses para 147 pacientes e de três a 18 meses para 19 pacientes, sendo o período médio de acompanhamento de 36 meses. A todas as instituições que se integraram ao estudo cooperativo do GCBTTW foram fornecidos os protocolos, visando à uniformização dos procedimentos clínicos e cirúrgicos. Resultados. Após submeter os dados obtidos à análise estatística, verificamos que: 1) Não existe interferência da ligadura prévia do pedículo nos resultados (recidivas e mortalidade); 2) A ruptura tumoral intra-operatória com contaminação da cavidade peritoneal interfere desfavoravelmente no índice de mortalidade. 3) Existe discordância significante entre a opinião do cirurgião e a análise histopatológica quanto à invasão da adrenal e da gordura peri-renal. 4) Os tumores com peso superior a 500 g têm pior prognóstico. 5) O desempenho dos cirurgiões foi considerado inadequado em relação ao inventário ganglionar (56,6 por cento dos pacientes não tiveram gânglios analisados), o que pode ter contribuído para: menor índice de recidivas nos pacientes com gânglios acometidos; maior incidência de metástases pulmonares nos pacientes sem gânglios analisados; pacientes no Estádio II com melhor prognóstico em relação ao Estádio I, e pacientes em estádios I e III com índices de recidivas semelhantes.