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.

Dental management of a child undergoing treatment for Wilms' tumor: a case report

Introduction: Wilms' tumor is a malignant renal neoplasm that frequently occurs in children during the first decade of life. clinically, it is a rapidly growing abdominal mass that causes low back pain and hematuria. computerized axial tomography or nuclear magnetic resonance are fundamental for its diagnosis, and chemotherapy and surgery have become first-choice treatments. after diagnosis, the majority of treatment plans involve the administration of antineoplastic drugs, whose side effects may include mucositis, candidiasis, xerostomia, caries, and worsen other previously diagnosed lesions, regardless of the organ affected by the tumor. treatment is more effective if provided by a multidisciplinary team in which the dentist plays a significant role in the implementation of an integral oral care protocol. in the present study, the management of a pediatric patient under antineoplastic treatment for Wilms' tumor is reported. case report: a four-year-old female patient diagnosed with Wilms' tumor, who required antineoplastic treatment. she had temporary dentition with early childhood caries, irreversible pulpal lesions and agenesis of teeth 72, 82, and the germ of tooth 42. the patient received modeling based behavior management therapy, prophylactic oral hygiene, and restoration of teeth affected by caries. to present this case, the "CARE" guidelines were used. conclusion: poor oral health status prior to cancer therapy directly affects the quality of life and the treatment of a patient, increasing the risks of local or systemic infections. as such evaluation and dental treatment before antineoplastic therapy is important to prevent oral complications and lesions.

Tumor de Wilms en niños de Costa Rica / (Wilms tumor in Costa Rican children)

Resumen Objetivo: describir las características clínicas del grupo de niños diagnosticados con tumor de Wilms en Costa Rica y cuál es la evolución clínica de acuerdo con los diversos tratamientos que reciben en el Servicio de Oncohematología del Hospital Nacional de Niños. Métodos: este es un estudio descriptivo retrospectivo que analiza un periodo de 20 años de pacientes con diagnóstico de tumor de Wilms confirmado por biopsia. Se revisó 69 expedientes de pacientes con edades entre 0 y 13 años, en los cuales se analizaron los datos clínicos, terapéuticos (quimioterapia, radioterapia) y pronósticos (sobrevida global y libre de enfermedad) de cada uno. Los resultados obtenidos se compararon con los de otros estudios latinoamericanos y europeos. Se realizaron modificaciones en el manejo de los pacientes costarricenses, con base en los resultados obtenidos. Resultados: se analizó una muestra total de 69 casos. Entre los resultados se encontró que los pacientes presentaban una edad promedio diagnóstica de 41,3 meses. El seguimiento promedio fue de 7,4 años. La masa abdominal fue el hallazgo clínico predominante (55%). Además, el estadio III fue el más común (31,8%). La nefrectomía total sin ruptura tumoral fue el procedimiento quirúrgico en la mayoría de los casos. El 80% de los pacientes presentó histología favorable en el análisis histopatológico. En el 51% de los casos se utilizó quimioterapia prequirúrgica. Un 17% de los pacientes presentó metástasis pulmonar. La sobrevida global fue del 73,3% y la sobrevida libre de enfermedad, del 69%. Conclusiones: los pacientes costarricenses con tumor de Wilms localizados tuvieron una sobrevida inferior a la de los países desarrollados, y similar en casos metastásicos.

Abstract Objective: to describe the clinical characteristics of the group of children diagnosed with Wilms' tumor in Costa Rica and what was the clinical evolution according to the different treatments received in the Oncohematology Division of the National Children's Hospital. Methods: this is a retrospective descriptive study that analyzed the patients with diagnosis of Wilms tumor confirmed by biopsy in period of 20 years. We reviewed 69 records of patients aged between 0 and 13 years, in which the clinical, therapeutic (chemotherapy, radiotherapy) and prognostic data (global and disease-free survival) of each one were analyzed. Results: a total sample of 69 cases was analyzed. Among the results, it was found that the patients presented an average age of 41.3 months at the moment of diagnosis. The average follow-up was 7.4 years. The presence of an abdominal mass was the predominant clinical finding (55%). In addition, stage III was the most common (31.8%). Total nephrectomy without tumor rupture was the surgical procedure in the majority of cases. 80% of the patients presented favorable histology in the histopathological analysis. In 51% of cases, pre-surgical chemotherapy was used. 17% of the patients presented pulmonary metastasis. Overall survival was 73.3% and disease free survival was 69%. Conclusions: Costa Rican patients with localized Wilms' tumor had poorer results than that of patients in developed countries, but the survival was similar in metastatic cases.

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)

Resultado del tratamiento del tumor de Wilms en población pediátrica / Treatment results of Wilms'tumor in pediatric population

Introducción: El tumor de Wilms (TW) es el tumor renal maligno más frecuente en la infancia, su tratamiento incluye, según su estadio y forma de presentación, cirugía precoz, radioterapia (RT) y quimioterapia (QT) según el protocolo PINDA. El objetivo es conocer los resultados de la serie histórica del Instituto Nacional del Cáncer (INC). Pacientes y Método: Revisión retrospectiva de todos los pacientes tratados con el diagnóstico de TW en el INC. Se describe la población de pacientes, tratamiento de RT recibidos, resultados de sobrevida global y factores pronósticos. Resultados: Desde septiembre de 1993 hasta diciembre de 2010 se trataron con RT 110 niños. La mediana de edad al diagnóstico fue de 3,6 años. La mediana de seguimiento fue de 128 meses desde el fin de RT. A junio de 2012, del total de 107 pacientes con seguimiento, 22 habían fallecido, 19 por progresión de enfermedad. Todos los pacientes que fallecieron lo hicieron antes de tres años desde el tratamiento. La sobrevida global a 2, 4, 12 años fue de 82 por ciento, 78 por ciento, 78 por ciento respectivamente. En análisis multivariado, cada día después de la cirugía sin comenzar la RT disminuye las probabilidades de sobrevida (p = 0,04). Conclusiones: El tratamiento del TW tiene un excelente pronóstico. La sobrevida, luego de los 3 años se estabiliza sin presentar eventos, indistintamente del grupo al que pertenezca el paciente. Dentro de los factores pronósticos, en pacientes con indicación de RT, esta se debe iniciar precozmente, al parecer lo más cercano a la cirugía.

Introduction: Wilms tumor (WT) is the most common form of malignant kidney tumor in childhood. According to PINDA protocols, its treatment includes, depending on stage and presentation, early surgery, radiotherapy (RT) and chemotherapy (CT). The objective of this work is to review the results of all patients of the National Cancer Institute (NCI) with this condition. Patients and Method: A retrospective review of all patients diagnosed with WT at the NCI was conducted. Patient population, RT treatment received and overall survival results were described and prognostic factors were searched. Results: From September 1993 to December 2010, 110 children were treated with RT. The median age at diagnosis was 3.6 years old. Median follow-up was 128 months after RT. In June 2012, out of a total of 107 patients with follow up, 22 had died, 19 due to disease progression. All deaths occurred within three years of treatment. Overall survival at 2, 4 and 12 years old was 82 percent, 78 percent, 78 percent respectively. A multivariate analysis showed that each day after surgery and without starting RT, the chances of survival decreased (p = 0.04). Conclusions: WT treatment has an excellent prognosis. Survival after 3 years stabilizes without presenting complications, regardless of the group to which the patient belongs. Among the prognostic factors for patients with RT prescription, this radiation should be started early, as close to the surgery as possible.

Neoplasias sólidas de células redondas en edad pediátrica: correlación entre diagnóstico morfológico e inmunohistoquímica / Solid neoplasm of circle celis in the pediatric age: correlation between morphological and immunohistochemistry diagnóstic

Precisar la importancia de realizar el estudio de inmunohistoquímica y su correlación con el diagnóstico anatomopatológico de rutina para el diagnóstico de los tumores sólidos malignos de células redondas en pacientes pediátricos. Se realizó un estudio transversal, descriptivo, retrospectivo no experimental. Se estudiaron 147 pacientes con diagnóstico de neoplasia de células redondas, de los cuales 96 cumplieron con criterios de inclusión. La mayor frecuencia de casos la obtuvo el linfoma No Hodgkin (20,8%), seguido del linfoma de Hodgkin (15,6%) y tumor de Wilms (17,7%). El grupo etario mayormente afectado fue el escolar (37,5%). El género predominante fue el masculino (60,4%). Las manifestaciones clínicas más frecuentes fueron masa palpable (76,04%), fiebre(44,79%), anemia (41,67%), dolor (41,30%) y pérdida de peso (33,33%). Se evidenció la mayor correlación diagnóstica en el tumor de Wilms (100%), seguido del linfoma No Hodgkin (80%) y el linfoma de Hodgkin (75%); la menor correlación la presentaron el sarcoma de Ewingy el neuroblastoma (41,7%, ambos). La correlación total resultó “considerable” según la fuerza de concordancia del coeficiente de Kappa (k: 0,601. P=0,000). Se requiere la integración del examen histopatológico de rutina como método diagnóstico de pesquisa y del estudio de inmunohistoquímica como método auxiliar diagnóstico, para determinar la histogénesis de las neoplasias, descartar diagnósticos diferenciales, realizar un diseño terapéutico apropiado y precisar el pronóstico.

To precise the state and the importance o fthe study of immunohistochemistry and its correlation with the routine pathological diagnosis to use in diagnosis of malignant solid tumors of round cells in pediatric patients. We was performed a transversal, descriptive and retrospective non-experimental study. We studied 147 patients diagnosed with round cell tumor, 96 of them weremet in inclusion criteria. The highest frequency of the cases was Non-Hodgkin lymphoma (20.8%), followed by the Hodgkin lymphoma (15.6%) and the Wilms tumor(17.7%). The age group most affected was the school(37.5%). The male gender was the predominant (60.4%).The most frequent clinical manifestations were a palpablemass (76.04%), the fever (44.79%), the anemia (41.67%),the pain (41.30%) and the weight loss (33.33%). Evidencedthe highest correlation in the diagnosis of Wilms tumor(100%), followed by Non-Hodgkin lymphoma (80%) and Hodgkin lymphoma (75%); showed the lowest correlation between the Ewing sarcoma and neuroblastoma (41.7%, both). The total correlation is “considerable” accordingto strength of agreement the Kappa coefficient (k: 0.601.P=0.000). It requires the integration of routine histopathological examination as diagnostic method of research and study of immunohistochemistry as an auxiliary diagnostic method to determine the histogenesis of neoplasm and exclude the differential diagnoses, make anappropriate therapeutic design and the determine prognosis of this group of patients.

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.

Pattern, clinical presentation and management of wilms' tumor in Moshi, Tanzania

The aim of this study was to audit the treatment outcome of children with Wilms' tumor in East Africa, at a Urology referral center with limited access to imaging modalities and chemotherapeutic drugs, and no radiation facility. This is a retrospective analysis of the hospital records of children with a diagnosis of Wilm's tumor treated from June 1996 to May 2005 at the Association of Surgeons of East Africa [ASEA] Institute of Urology, Kilimanjaro Christian Medical Centre [KCMC], Moshi, Tanzania. In total, 50 patients were diagnosed with Wilms' tumor in the 9 years study period, but only 39 files contained sufficient information for analysis. The average age at presentation was 44.7 months [median 36, range 8 to 120 months]. 25.6% of the children were >60 months old. The male:female ratio was 1.4:1. All of the children presented with an abdominal mass detected by the parents. The hemoglobin was < 100gm/L in 21 [53.8%] and the platelet count was >450,000/ml in 8 [20.5%] cases. Ultrasound imaging was obtained in all cases, but CT imaging was not taken. Intravenous urography was done in 38 children and showed non-visualization of the involved kidney in 16 [42.1%]. Fine needle aspiration cytology [FNAC] was performed in 25 cases and was diagnostic in 23 [92%]. Preoperative vincristine and dactinomycin was given to 23 patients [59%] with marked tumor shrinkage in 20 [87%], while 3 [13%] showed no response. Radical nephrectomy was performed in all patients, and 3 patients [7.7%] died within 24 hours of surgery. Pathologic analysis showed favorable histology in 35 [89.7%] and unfavorable histology in 4 [10.3%] patients. Based on the pre- and intra-operative findings. NWTS stage 1,2,3,4 and 5 was present in 25.6%, 17.9%, 15.4%, 38.5% and 2.6%, respectively. Postoperative dactinomycin and vincristine was given monthly for one year in all patients, while doxorubicin and cyclophospamide were reserved for those with no response to the first-line drugs, or recurrent tumor. Radiotherapy was not available. At 12 months' follow-up the overall disease-free survival was 35.9%, recurrence had occurred in 38.6%, death in 15.4%, and 10.3% were lost to follow-up. Children with Wilms' tumor in East Africa still have a dismal prognosis, with treatment outcomes at levels where it was before the advent of chemotherapy and radiation therapy in more advanced centers

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 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.

Lesiones cutáneas causadas por Pseudomonas aeruginosa en pacientes inmunocomprometidos / Cutaneous lesions caused by Pseudomonas aeruginosa in an immunodeficient patient

Introducción. Las lesiones cutáneas asociadas a Pseudomonas aeruginosa en pacientes son diversas. En pacientes inmunocompetentes las lesiones son benignas y autolimitadas. En pacientes inmunocomprometidos se presentan lesiones dérmicas con o sin bacteriemia e incluyen: ectimas grangrenoso, bulas hemorrágicas, celulitis, placas, pápulas, petequias, equimosis, nódulos subcutáneos eritematosos o violáceos, lesiones erisipeloides y conjuntivitis. Caso clínico. Lactante mayor masculino de 1 año 4 meses de edad con tumor de Wilms que había recibido quimioterapia 18 días previos a su ingreso, motivado por inflamación, aumento de temperatura y eritema escrotal, fisuras perianales y fiebre; con granulocitopenia grave por quimioterapia y con datos de choque, ingresó al servicio de Terapia Intensiva manejándose con ceftazidima, clindamicina, ampicilina y aminas; las lesiones escrotales evolucionaron a necrosis y ulceración. Presentó conjuntivitis purulenta, nódulos violáceos en muslos abdomen, los cultivos reportaron P. aeruginosa. Por mala evolución (progresión de las lesiones, fiebre e inestabilidad hemodinámica) se cambió el manejo a imipenem-amikacina por 14 días de acuerdo a la sensibilidad (método de Kiry-Bauer), apreciándose posteriormente evolución satisfactoria. Conclusión. Es importante reconocer lesiones cutáneas por P. aeruginosa para sospechar infección por este germen y dar un tratamiento oportuno

Bilateral Wilms tumor: an eleven year experience.

Over the last 11 years, 101 patients of Wilms tumor were treated at our hospital. Of these, 7 had bilateral Wilms tumors (BWT). The first two patients were referred in terminal stages and died. Of the latter five, three have survived while one is on treatment. One patient succumbed to liver metastasis, he had stage III disease on one side and was irregular in his chemotherapy protocol. Remarkably, one of the survivors was cured even after developing vertebral metastasis, retroperitoneal recurrence and bilateral lung metastasis. Part of the success was due to "salvage chemotherapy regimens" using Cis-plantinum, ifosphamide and VP-16. Flow cytometric analysis of DNA ploidy performed on only three of the five patients showed that two tumors were aneuploid and one was diploid. Spectacular cure rates can be achieved in BWT by renal conservation surgeries and effective chemotherapy. The use of "salvage chemotherapeutic regimes" and the study of DNA ploidy is being highlighted.

Controvérsias sobre tratamento pré-operatório em tumor de Wilms / Controversies regarding preoperative chemotherapy in Wilm's tumor

O tratamento pré-operatório em tumor de Wilms continua controverso. Ambas as condutas apresentam vantagens e desvantagens. Näo há dúvida de que às vezes é indispensável, porém persiste a dúvida se deva ser indicado sempre como é feito no estudo da Sociedade Internacional de Oncologia Pediátrica (SIOP). Nesta revisäo discutimos as indicaçöes a as contra-indicaçöes e citamos a experiência brasileira.

Management of metastatic Wilms tumor: an eleven year experience.

During the last 11 years, 101 patients of Wilms tumor were seen. Of these, 13 patients had metastatic disease at onset while 24 patients presented with relapse at a latter date. Seven patients have been cured, all had favourable histology. The risk factors associated with relapse were found to be unfavourable histology, lymph node involvement, age more than 6 years, diffuse spill, capsular and vascular invasion, and aneuploidy. Of the 18 patients who had relapsed, 14 were found to be aneuploid. Judicious use of various therapeutic options like radiotherapy to metastatic sites, second look surgery, resection of pulmonary metastasis and use of cis-platinum, VP-16 and Ifosfamide as "alternative chemotherapy protocols" were used to achieve cure in these advance cases.