Wilms Tumor

2018 ◽  
Author(s):  
Graciela Argote-Romero
Keyword(s):  
Wilms Tumor ◽  
Incidental Finding ◽  
Abdominal Mass ◽  
Disease Free Survival ◽  
Von Willebrand Disease ◽  
Renal Tumors ◽  
Intravenous Contrast ◽  
Favorable Histology ◽  
Disease Free Survival Rate ◽  
Von Willebrand

Wilms tumor, known as well as nephroblastoma, is the most common primary malignant renal tumor in children. Over 95% of all renal tumors in patients under the age of 15 are Wilms tumors. The mean age at the time of diagnosis is 3.5 years. Wilms tumors are usually an incidental finding, a large abdominal mass discovered by a family member or pediatrician. Hematuria and hypertension can be present at the time of initial diagnosis. Up to 8% of the patients will have von Willebrand disease; therefore, all patients should have baseline coagulation studies. All patients should have either computed tomography of the abdomen and pelvis with oral and intravenous contrast or magnetic resonance imaging of the abdomen and pelvis with gadolinium. Treatment includes radical nephrectomy, chemotherapy, and, in some cases, radiotherapy. Emergency surgery is rarely. The disease-free survival rate is 86% for favorable-histology tumors and 64% for anaplastic tumors.

Nuclear Morphometry and Prognosis in Favorable Histology Wilms' Tumor: A Prospective Reevaluation

1999 ◽  
Vol 17 (7) ◽  
pp. 2123-2123 ◽  
Author(s):  
Norman E. Breslow ◽  
Alan W. Partin ◽  
Benjamin R. Lee ◽  
Katherine A. Guthrie ◽  
J. Bruce Beckwith ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Discriminant Function ◽  
Wilms Tumor ◽  
Disease Free Survival ◽  
Data Set ◽  
Free Survival ◽  
Favorable Histology ◽  
Relative Risks ◽  
Nuclear Morphometry ◽  
Disease Free

PURPOSE: This study was designed to evaluate the ability of a previously published nuclear morphometry discriminant function to predict disease-free survival in patients with Wilms' tumor. PATIENTS AND METHODS: We identified 218 patients with stage I-IV Wilms' tumor of favorable histology who were entered onto the National Wilms' Tumor Study (NWTS) between January 1, 1990 and April 15, 1994. The nuclear morphometry score was calculated for each patient as follows: MVf = (0.02 × AGE) + (1.17 × SNRF) + (90.6 × LEFD) − 94, with AGE denoting age at diagnosis in months, SNRF the skewness of the nuclear roundness factor, and LEFD the lowest value of nuclear ellipticity as measured by the feret diameter method. Relative risks of relapse were estimated for the total score and for each of its components. Sensitivity and specificity were determined for the criterion of “MVf is greater than −0.35” as a predictor of relapse. RESULTS: By contrast with previously published results, neither the SNRF nor the LEFD made any contribution to the prediction of disease-free survival. Sensitivity and specificity of the criterion of “MVf is greater than −0.35” were 71% and 56%, respectively. CONCLUSION: Re-evaluation of a published nuclear morphometry score showed that it did not predict disease-free survival in patients with Wilms' tumor. The earlier study very likely overestimated the predictive power of nuclear morphometry by using the same data set both to develop the score and to evaluate its properties. Because of the huge number of combinations of nuclear morphometry measurements that may enter into the multivariate discriminant function, use of appropriate statistical methods is essential to estimate accurately the sensitivity and specificity.

Acquired von Willebrand disease in a patient with Wilms tumor

1979 ◽  
Vol 95 (6) ◽  
pp. 997-999 ◽  
Author(s):  
Peter A. Noronha ◽  
Marilyn A. Hruby ◽  
Helen S. Maurer
Keyword(s):  
Wilms Tumor ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand

scholarly journals WILMS TUMOR IN ADULTS: CASE REPORT AND REVIEW OF THE LITERATURE

2017 ◽  
Vol 5 (3) ◽  
pp. 110-115
Author(s):  
Bruno Rafael Kunz Bereza ◽  
Tariane Friedrich Foiato Maneti ◽  
Vitor Arce Ferreira Cathcart ◽  
Raimundo Romilton Leal do Rosário ◽  
Paula Nunes ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Clinical Symptoms ◽  
Wilms Tumor ◽  
Abdominal Mass ◽  
Renal Tumors ◽  
Recommended Treatment ◽  
Solid Renal Mass ◽  
Adults And Children ◽  
Pathological Report

Wilms Tumor (WT) is an extremely rare neoplasia in adults. It represents 95% and 1% of all renal tumors, in adults and children respectively. Clinical symptoms are also distinct between adults and children. In adults, symptoms are usually abdominal pain and hematuria, on the other hand, pediatric patients frequently present with an asymptomatic abdominal mass. The recommended treatment for WT in adults follows protocols established for pediatric patients. Currently, the 5-year overall survival rate for adults with WT is of approximately 90%. The adoption of multimodal therapy systematized, and proposed by current protocols, allowed the results to become much more robust, with a significant improvement from less than 30% to 90%. However, recent reviews suggest a worse outcome for adults when compared to children, even when submitted to the same treatment regimen, and comparable according to histology and tumor stage.We present a case of a 26 years-old male patient, who underwent surgery to treat a solid renal mass. Pathological report demonstrated a Wilms tumor. The patiente also received adjuvant chemotherapy with dactinomycin and vincristine. After 12 months follow-up, patient has no evidence of disease.

Acquired von Willebrand disease in children with a Wilms' tumor

1996 ◽  
Vol 26 (4) ◽  
pp. 238-243 ◽  
Author(s):  
Anne B. Jonge Poerink-Stockschläder ◽  
Ina Dekker ◽  
Inge M. Risseeuw-Appel ◽  
Karel Haählen
Keyword(s):  
Wilms Tumor ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand

scholarly journals Renal Primitive Neuroectodermal Tumors

2012 ◽  
Vol 136 (6) ◽  
pp. 686-690 ◽  
Author(s):  
Tanner Bartholow ◽  
Anil Parwani
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Correct Diagnosis ◽  
Age Distribution ◽  
Disease Free Survival ◽  
Neuroectodermal Tumor ◽  
Renal Tumors ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Genetic Abnormalities ◽  
Disease Free Survival Rate

Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

Acquired von Willebrand disease in Wilms' tumor patients.

1992 ◽  
Vol 10 (3) ◽  
pp. 422-427 ◽  
Author(s):  
M J Coppes ◽  
S W Zandvoort ◽  
C R Sparling ◽  
A O Poon ◽  
S Weitzman ◽  
...  
Keyword(s):  
Platelet Count ◽  
Wilms Tumor ◽  
Prospective Trial ◽  
Von Willebrand Disease ◽  
Laboratory Evaluation ◽  
Type I ◽  
Newly Diagnosed ◽  
Type Iii ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand

PURPOSE A prospective study was performed to determine the incidence of acquired von Willebrand disease (vWD) in children with newly diagnosed Wilms' tumor. PATIENTS AND METHODS Fifty consecutive children with newly diagnosed Wilms' tumor were evaluated. Detailed family and bleeding histories were obtained in all cases. Laboratory evaluation included measurement of the circulating platelet count, bleeding time (BT), factor VIII (FVIII) and von Willebrand factor (vWF) levels, and ristocetin cofactor (RCoF) activity. A vWF multimer analysis was obtained in all cases in which vWD was suspected. RESULTS Four of 50 (8%) consecutive children with a diagnosis of Wilms' tumor were found to have acquired vWD. Laboratory findings indicated type III vWD in two patients and type I vWD in the other two. CONCLUSIONS The incidence of acquired vWD in association with Wilms' tumor merits further study through a large prospective trial. Such a trial should include careful family and clinical bleeding histories plus measurement of a platelet count, BT, coagulant FVIII and vWF levels, RCoF activity, and vWF multimer analysis. The response to 1-desamino-8-D-arginine vasopressin (DDAVP) should be tested in all patients with Wilms' tumor and acquired vWD, including patients with a type III profile, before an invasive procedure is performed. Successful use of DDAVP may avoid exposure of affected patients to blood products.

Treatment With Nephrectomy Only for Small, Stage I/Favorable Histology Wilms’ Tumor: A Report From the National Wilms’ Tumor Study Group

2001 ◽  
Vol 19 (17) ◽  
pp. 3719-3724 ◽  
Author(s):  
Daniel M. Green ◽  
Norman E. Breslow ◽  
J. Bruce Beckwith ◽  
Michael L. Ritchey ◽  
Robert C. Shamberger ◽  
...  
Keyword(s):  
Survival Rate ◽  
Wilms Tumor ◽  
Cumulative Risk ◽  
Disease Free Survival ◽  
Stage I ◽  
Stopping Rules ◽  
Surgical Specimen ◽  
Free Survival ◽  
Favorable Histology ◽  
Disease Free

PURPOSE: Children younger than 24 months with small (< 550 g), favorable histology (FH) Wilms tumors (WTs) were shown in a pilot study to have an excellent prognosis when treated with nephrectomy only. PATIENTS AND METHODS: A study of nephrectomy only for the tratment of selected children with FH WT was undertaken. Stringent stopping rules were designed to insure closure of the study if the true 2-year relapse-free survival rate was 90% or lower. RESULTS: Seventy-five previously untreated children younger than 24 months with stage I/FH WTs for which the surgical specimen weighed less than 550 g were treated with nephrectomy only. Three patients developed metachronous, contralateral WT 1.1, 1.4, and 2.3 years after nephrectomy, and eight patients relapsed 0.3 to 1.05 years after diagnosis (median, 0.4 years; mean, 0.51 years). The sites of relapse were lung (n = 5) and operative bed (n = 3). The 2-year disease-free (relapse and metachronous contralateral WT) survival rate was 86.5%. The 2-year survival rate is 100% with a median follow-up of 2.84 years. The 2-year disease-free survival rate (excluding metachronous contralateral WT) was 89.2%, and the 2-year cumulative risk of metachronous contralateral WT was 3.1%. CONCLUSION: Children younger than 24 months treated with nephrectomy only for a stage I/FH WT that weighed less than 550 g had a risk of relapse, including the development of metachronous contralateral WT, of 13.5% 2 years after diagnosis. All patients who experienced relapse on this trial are alive at this time. This approach will be re-evaluated in a clinical trial using a less conservative stopping rule.

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