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.

Le néphroblastome au CHU de Brazzaville

Le néphroblastome est le deuxième cancer de l'enfant à Brazzaville. Nous avons mené cette étude afin d'en dégager les principales caractéristiques et les difficultés de leur prise en charge à Brazzaville au Congo.Il s'était agi d'une étude descriptive transversale sur une période de six ans (2008-2013). Nous avions ainsi colligé les patients atteints de néphroblastome dont nous avons décrits les aspects épidémiologiques, cliniques et thérapeutiques. Durant la période d'étude, 52 enfants, âgés de 0 à 14 ans avaient été colligés, dont 14 pour néphroblastome. Nous avions constaté une prédominance féminine et l'âge moyen au diagnostic était de 50,6 mois. La masse abdominale était la principale circonstance de découverte. Un cas de néphroblastome bilatéral avait été retrouvé. La survie globale à 6 mois, était de d'environ 65 %

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)

The Cost of Nephroblastoma Treatment in South Africa: A Very Cost-Effective Investment with Guidelines for the Rest of Africa

Background. Nephroblastoma is one of the most common childhood malignancies in Africa; but with a survival rate significantly lower than in developed countries. In African countries with a small gross domestic product (GDP) per capita; the cost of treating nephroblastoma may be prohibitive. Objectives. To determine the direct costs of treatment of nephroblastoma in South Africa (SA) and to propose a more cost-effective approach to investigations and treatment for the disease in Africa. Methods. Data from 2000 - 2010 from two SA paediatric oncology units were retrospectively analysed. The costs included investigations; chemotherapy and radiotherapy; comparing early-v. advanced-stage disease. In both units; the nephroblastoma International Society of Paediatric Oncology (SIOP) protocol was used. Results. Stage I disease was the most common; followed by stage IV. The total cost of diagnosis; staging and treatment of stage I disease was ZAR9 304.97 (EUR882.80 or USD1 093.40); compared with a five-times higher cost for stage IV (ZAR48 293.62 (EUR4 581.9 or USD5 674.9)). Treating one patient averted more than 32 disability adjusted life years. The investigation and treatment of early- and advanced-stage disease is very cost-effective when compared with the local GDP per capita. Conclusion. The cost of investigation and treatment of nephroblastoma remains a challenge everywhere; but especially in Africa. However; it is a very cost-effective disease to treat and children in Africa should not be denied treatment

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 abdominais malignos mais frequentes na infância: diagnóstico diferencial / Most common abdominal malignancies in childhood: differential diagnosis

Objetivo: Discutir as principais características clínicas do neuroblastoma, linfoma e tumor de Wilms, contribuindo para o diagnóstico precoce destas doenças. Fonte de dados: Medline PubMed. Síntese dos dados: Na prática clínica o achado de tumores abdominais na infância é frequente. A maioria das massas é causada por doenças benignas. Eventualmente, neoplasias malignas da infância também se manifestam como tumor abdominal. Os autores discutem neste artigo as principais diferenças clínicas e laboratoriais dos três tumores abdominais malignos mais frequentes na infância: linfoma não Hodgkin, neuroblastoma e tumor renal de Wilms. Conclusão: Através da análise dos dados clínicos e do exame físico do paciente com tumor maligno abdominal, o pediatra pode traçar hipóteses diagnósticas e encaminhar precocemente a criança para centros especializados.

Totally implantable catheter embolism: two related cases / Embolia de cateter totalmente implantável: relato de dois casos

CONTEXT AND OBJECTIVE: Long-term totally implantable catheters (e.g. Port-a-Cath®) are frequently used for long-term venous access in children with cancer. The use of this type of catheter is associated with complications such as infection, extrusion, extravasation and thrombosis. Embolism of catheter fragments is a rare complication, but has potential for morbidity. The aim here was to report on two cases in which embolism of fragments of a long-term totally implantable catheter occurred. DESIGN AND SETTING: Case series study at Hospital do Servidor Público Estadual, São Paulo. METHODS: Retrospective review of catheter embolism in oncological pediatric patients with long-term totally implantable catheters. RESULTS: The first patient was a 3-year-old girl diagnosed with stage IV Wilms' tumor. Treatment was started with the introduction of a totally implantable catheter through the subclavian vein. At the time of removal, it was realized that the catheter had fractured inside the heart. An endovascular procedure was necessary to remove the fragment. The second case was a boy diagnosed with stage II Wilms' tumor at the age of two years. At the time of removal, it was noticed that the catheter had disconnected from the reservoir and an endovascular procedure was also necessary to remove the embolized catheter. CONCLUSION: Embolism of fragments of totally implantable catheters is a rare complication that needs to be recognized even in asymptomatic patients.

CONTEXTO E OBJETIVO: Os cateteres de longa permanência totalmente implantáveis (por exemplo, Port-a-Cath®) são muito utilizados para acesso venoso de longa duração em crianças com câncer. O uso deste tipo de cateter está associado a algumas complicações como infecção, extrusão, extravasamento e trombose. A embolia de fragmento de cateter é complicação rara, mas com potencial morbidade. O objetivo do estudo foi relatar dois casos em que houve embolia de fragmento de cateter de longa permanência. TIPO DE ESTUDO E LOCAL: Série de casos do Hospital do Servidor Público estadual, São Paulo. MÉTODOS: Estudo retrospectivo das embolias de cateter de longa permanência em crianças. RESULTADOS: No primeiro caso, descreve-se uma menina com diagnóstico de tumor de Wilms estádio IV aos três anos de idade. Iniciou tratamento com implante de cateter de longa permanência em veia subclávia. Três anos após diagnóstico, foi programada a retirada do cateter por término de tratamento. No momento da retirada, houve fratura intracardíaca do cateter. Houve necessidade de retirada do fragmento intracardíaco com hemodinâmica. O segundo caso tratava-se de um menino com diagnóstico de tumor de Wilms estádio II aos dois anos de idade. Teve o cateter implantado no início do tratamento. No momento da retirada por fim de tratamento, o cateter já havia desconectado e embolizado. O fragmento intracardíaco foi retirado também por procedimento de hemodinâmica. CONCLUSÃO: A embolia de fragmentos de cateter em cateteres de longa permanência é uma complicação rara, mas que necessita ser reconhecida, mesmo em pacientes assintomáticos.

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

Tumor de Wilms: asociación de complicaciones quirúrgicas y hallazgos clínicos e imagenológicos / Wilms' tumor: surgical complications and clinical-imaging findings

Los pilares del tratamiento del Tumor de Wilms son la cirugía y la quimioterapia y, según la etapificación de la enfermedad, la radioterapia. En Chile, el protocolo terapéutico contempla definir si el tumor es resecable o irresecable al momento del diagnóstico de acuerdo a los hallazgos imagenológicos. Pensamos que debería establecerse elementos clínicos claros que permitan discernir objetivamente la resecabilidad tumoral en el nefroblastoma. Se realiza revisión de fichas clínicas y exámenes imagenológicos (ecografía y tomografía axial computarizada(Tac)) de pacientes tratados en el Hospital Roberto del Río entre julio 1995 y julio 2003 por uno de los autores. Una vez tabulados estos datos se entregaron a dos cirujanos oncólogos quienes por separado y en forma independiente y ciega según estos elementos clínicos debían considerarar si el tumor era resecable o irresecable. El grado de acuerdo entre los cirujanos se midió mediante el empleo del Indice Kappa. Las determinaciones de significación estadística se realizaron mediante prueba de Fisher_Irwing. Los índices Kappa de concordabilidad entre los 2 cirujanos observadores fue Clínica = 0.611; Ecografía = 0.87; Tac = 0.72. La diferencia estadística en la concurrencia de complicaciones quirúrgicas entre los 14 pacientes en que el tratamiento quirúrgico se realizó en la oportunidad de consenso y aquellos en los que no hubo acuerdo entre los cirujanos evaluadores A y B y la indicación del equipo fue significativa ( p= 0.0083 ). Proponemos los siguientes criterios predictivos de complicaciones en el tratamiento quirúrgico del Tumor de Wilms: Un tumor renal unilateral que aumente en 20 por ciento ó más el volumen renal para ese paciente comparándolo con el contralateral será considerado irresecable, si además se agregan al menos dos de los hallazgos clínicos que se mencionan a continuación: No movilidad del tumor en la palpación abdominal Circulación abdominal colateral marcada Tumor que traspasa la línea media.

Wilms' tumor: a 10 year retrospective study

Wilms' tumor [nephroblastoma] is the most common renal malignancy of childhood. The aim of the study was to evaluate the characteristics of Wilms' tumor and the results of combined modality treatment obtained in our center in Tehran. Fifty-five patients diagnosed as having Wilms' tumor were studied in the period between February 1992 and March 2002. Demographic features, mode of presentation, associated anomalies, the stage of tumor, histopathologic results, and the survival rates were evaluated. Of these 55 patients, 31 were males and 24 were females [M/F = 1.2]. The mean age at the time of diagnosis was 45.2 months. The distribution of 54 operated patients according to the surgical stage was: stage I 32.7%, stage II 16.36%, stage III 38.1%, stage IV 9%, and stage V 1.8%[one patient [1.8%] has not been operated]. Favorable histology was diagnosed in 54.5% and unfavorable histology in 43.6% of the patients. The patients were treated according to National Wilms' tumor Study protocols. The relapse-free and overall 4 years survival rates were 71% and 86%, respectively. As a developing country, with similar relapse free and overall survival rates to National Wilms' Tumor study, our institution showed an improvement in the treatment of patients with Wilms' tumor in recent 10 years, but with more adaptation to the National Wilms' Tumor Study treatment protocols better optimum results seem to be achievable

Tumor de Wilms del adulto / Wilms´ tumor of the adult

Se presenta y se comenta un caso de un paciente de 24 años con un tumor de Wilms en el adulto, asícomo el diagnóstico positivo y diferencial y la terapéutica empleada con mayor frecuencia en estaenfermedad. El tumor de Wilms es una dolencia de extraordinaria rareza en el adulto

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

Wilm's tumor in a solitary kidney complicated by chemotherapy induced obstructive uropathy.

A three-year-old male child with Wilm's tumor of left kidney and right sided unilateral renal agenesis is reported. The left renal vein was located posterior to the aorta. He was managed with medical measures alone. The initial phase of treatment was complicated by chemotherapy induced dislodgment of the tumor fragment and subsequent distal obstruction.

Wilms' tumour: presentation and treatment

To determine the clinical presentation and treatment results of patients of Wilms tumour treated with United Kingdom Children's Cancer Study Group [UKCCSG] protocol. Design: An interventional study. Place and Duration of Study: The study was conducted in the Paediatric Oncology Department of Combined Military Hospital, Rawalpindi [CMH RWP], from January 1999 to December 2000. Subject and All patients of Wilms' tumour managed by using UKCCSG treatment protocol were included in the study. The patients were thoroughly investigated before treatment. Surgery, chemotherapy and radiotherapy were performed as per protocol. Twenty-two patients including 13 boys and 09 girls with the mean age of 28 months, ranging from 4 months to 7 years were studied. The tumour involved right kidney in 13 and left in 9 patients. Two [09%] patients had stage I, 5 [23%] stage II, 11 [50%] stage III and 04 [18%] stage IV disease. Histology was favourable in 18 and unfavourable in 04 patients. Nineteen [86.4%] patients underwent primary surgery, one had delayed surgery while 02 [9%] patients had inoperable tumour and died despite chemotherapy. Three patients died during maintenance chemotherapy and one after completion of treatment. The overall mortality was 06 [27.3%]. Three patients were lost to follow-up. The treatment outcome is comparable to UKCCSG results. Challenges to be addressed in our set up include illiteracy, deficiency of resources, and regularity of treatment and follow-up. system and, therefore, its application is recommended to evaluate ovarian masses in clinical practice

Antígeno MIB-1 em nefroblastomas / MIB-1 antigen in nephroblastomas

Foram estudados 35 pacientes tratados com nefrectomia radical e quimioterapia, em 17 (48,5 por cento) dos quais se associou também a radioterapia. A idade média dos pacientes foi de 38 +/- 29 meses e o tempo de seguimento pós-operatório de 69 +/- 66 meses. Óbito pela neoplasia ocorreu em 5 pacientes, todos com histologia favorável. As peças conservadas em formol ou blocos de parafina para imunhistoquímica com anticorpo MIB-1, método da avidina-biotina-peroxidase. Em 13/35 (37,1 por cento) dos tumores a marcação para o MIB-1 foi positiva. As proporções respectivas de marcação para blastema, epitélio e estroma foram: 34,6 por cento, 18,7 por cento e 0 por cento. Não houve diferença estatística entre a marcação imunohistoquímica e com o tipo histológico, estádio e sobrevida dos pacientes.

Wilms' tumor: Riyadh armed forces hospital experience

The aim of this study is to analyse Wilms' tumor cases treated at Riyadh Armed Forces Hospital. twenty patients with Wilms' tumor were identified between January 1983 and November 1995. Mean age at presentation was 38.6 months, ranging from 5 months to 7 years. The prevalence of associated congenital abnormalities was as high as 20%. Females tended to present about 9 months younger and had less advanced disease compared to males. Disease at presentation tended to be advanced with one quarter of patients presenting with stage four disease. The five years disease-free survival probability was 93.5% with a median follow-up of 3 years and 6 months. females tended to present at a younger age, had less advanced disease and a more favourable outcome compared to males. There is an increased prevalence of associated abnormalities compared to international series. Long term survival proved to be similar to international studies