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.

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.

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.

Improved Survival in Patients with Recurrent Wilms Tumor: the Experience of the Seoul National University Children's Hospital

The survival in cases with relapsed Wilms tumor is dismal. Recently, however the introduction of new therapeutic agents and experimental strategies has improved the survival. We analysed the survival of patients with relapsed Wilms tumor according to the treatment period. During the early period 1983-1993, patients who had received two drugs were treated with doxorubicin and the others were treated with cisplatin and etoposide, whereas during the late period 1994-2004, patients were treated with combinations of cyclophosphamide/etoposide and carboplatin/etoposide. During the early period, 8 of 57 experienced relapse, and 8 of 41 relapsed during the late period. Only 2 patients treated during the early period survived in complete response (CR), whereas during the late period, 5 patients remained alive in CR, and 3 of those received high-dose chemotherapy (HDC) with autologous peripheral stem cell rescue (SCR). The estimated 5 yr event-free survival rate was 37.5% in the entire study group, 50% for patients in the late period, and 25% for patients in the early period (p=0.38). The survival in patients with relapsed Wilms tumor dramatically improved during the late period and HDC with SCR was one of the effective salvage strategies.

Clinicopathological profile of Wilms' tumor.

The profile of renal tumors in children less than 15 years of age during the period 1991-1997 is presented. Among the 37 children with kidney tumors, 29 (78.4%) had Wilms' tumor. There was also a 20-year-old female with Wilms' tumor. The median age at presentation was 2.6 years (range 2.5 months to 20 years). 66.7% of the cases diagnosed were < or = 3 years and 90% were < or = 6 years. Five cases were under one year of age. The male to female ratio was 2:1. Twenty-two cases (73.3%) were triphasic and 7 (23.3%) were biphasic. Only one case was monophasic with blastemal component. Five cases (16.7%) showed nephrogenic rests in the uninvolved renal parenchyma and one case had nephroblastomatosis. The tumor was favorable in 26 cases (86.7%) and unfavorable in 4. Fourteen cases were in-patients while 16 were outside referrals. The pathological (10 cases whose specimens were sent from other centers) and clinicopathological (13 hospitalized patients) staging showed 10 cases (43.5%) with stage 1, 4 cases (17.4%) with stage 2, and 7 cases (30.4%) with stage 3. In two cases (8.7%), there was stage 4 disease. The length of the follow-up period in the 13 hospitalized patients ranged from 7 days to 5 years 5 months (median 14 months). There was one recurrence and one death after 2 years of diagnosis.

Outcome of solid renal tumours in children at the University Hospital of the West Indies

Of the twenty-nine children with solid tumours treated at the University Hospital of the West Indies (UHWI) between January, 1972 and December, 1991, there were twenty-eight cases of nephroblastoma and one of mesoblastic nephroma. Peak incidence was between the ages of two and four years. Twenty-five children had radical nephrectomy while one had bilateral partial nephrectomy. In thirteen cases, pre-operative chemotherapy and radiotherapy was used. Post-operative chemotherapy and radiotherapy were used in 24 and 13 cases, respectively. Stage of the tumour was the most decisive factor influencing the outcome. Whereas there was 100 per cent cure rate in Stages I and II, Stage III had only a 55.5 per cent survival rate and non of the Stage IV survived. Bilateral (Stage V) tumours are curable if individual tumours are localised, as in one of the two cases. The benign mesoblastic nephroma, in a one-month-old infant, was cured by nephrectomy alone. While the present therapy of radical nephrectomy along with combination chemotherapy is satisfactory for early stages, more aggressive adjuvant therapy is needed for improving the results in Stages III and IV