Treatment of Anaplastic Histology Wilms' Tumor: Results From the Fifth National Wilms' Tumor Study

2006 ◽  
Vol 24 (15) ◽  
pp. 2352-2358 ◽  
Author(s):  
Jeffrey S. Dome ◽  
Cecilia A. Cotton ◽  
Elizabeth J. Perlman ◽  
Norman E. Breslow ◽  
John A. Kalapurakal ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Treatment Strategies ◽  
Stage I ◽  
Event Free Survival ◽  
Free Survival ◽  
Treatment Regimens ◽  
Favorable Histology ◽  
Tumor Study ◽  
Anaplastic Histology ◽  
Novel Treatment Strategies

Purpose An objective of the fifth National Wilms' Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for anaplastic histology Wilms' tumor (AH). Patients and Methods Prospective single-arm studies were conducted. Patients with stage I AH were treated with vincristine and dactinomycin for 18 weeks. Patients with stages II to IV diffuse AH were treated with vincristine, doxorubicin, cyclophosphamide, and etoposide for 24 weeks plus flank/abdominal radiation. Results A total of 2,596 patients with Wilms' tumor were enrolled onto NWTS-5, of whom 281 (10.8%) had AH. Four-year event-free survival (EFS) and overall survival (OS) estimates for assessable patients with stage I AH (n = 29) were 69.5% (95% CI, 46.9 to 84.0) and 82.6% (95% CI, 63.1 to 92.4). In comparison, 4-year EFS and OS estimates for patients with stage I favorable histology (FH; n = 473) were 92.4% (95% CI, 89.5 to 94.5) and 98.3% (95% CI, 96.4 to 99.2). Four-year EFS estimates for patients who underwent immediate nephrectomy with stages II (n = 23), III (n = 43), and IV (n = 15) diffuse AH were 82.6% (95% CI, 60.1 to 93.1), 64.7% (95% CI, 48.3 to 77.7), and 33.3% (95% CI, 12.2 to 56.4), respectively. OS was similar to EFS for these groups. There were no local recurrences among patients with stage II AH. Four-year EFS and OS estimates for patients with bilateral AH (n = 29) were 43.8% (95% CI, 24.2 to 61.8) and 55.2% (95% CI, 34.8 to 71.7), respectively. Conclusion The prognosis for patients with stage I AH is worse than that for patients with stage I FH. Novel treatment strategies are needed to improve outcomes for patients with AH, especially those with stage III to V disease.

Treatment outcomes in patients less than 2 years of age with small, stage I, favorable-histology Wilms' tumors: a report from the National Wilms' Tumor Study.

1993 ◽  
Vol 11 (1) ◽  
pp. 91-95 ◽  
Author(s):  
D M Green ◽  
N E Breslow ◽  
J B Beckwith ◽  
J Takashima ◽  
P Kelalis ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Stage I ◽  
P Value ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Treatment Regimens ◽  
Survival Percentage ◽  
Favorable Histology ◽  
Specimen Weight ◽  
Tumor Studies

PURPOSE Retrospective analyses were performed to determine the effect of tumor weight and therapy modifications on outcome in patients less than 2 years of age with stage I favorable-histology Wilms' tumors. PATIENTS AND METHODS The 4-year relapse-free and overall survival percentages for patients randomized to different treatment regimens in National Wilms' Tumor Studies (NWTS)-1, -2, and -3 were calculated and compared. RESULTS The 4-year relapse-free survival percentages of patients whose specimen weight was less than 550 g were found to be 89.1% on NWTS-1, 96.0% on NWTS-2, and 93.2% on NWTS-3. There was no evidence that the relapse-free survival of these patients had improved over time (P value for trend = .99). The 4-year relapse-free survival percentage for similar age and stage patients whose specimen weight was 550 g or greater was significantly poorer than that of patients with smaller tumors (P = .02). CONCLUSION Changes in the NWTS treatment regimens over a period of more than 20 years have not improved on the excellent prognosis of patients who are less than 2 years of age at diagnosis and who have a stage I, favorable-histology Wilms' tumor with specimen weight less than 550 g. These data could be used as the basis for a future trial in which a subgroup of such patients is treated with nephrectomy only.

scholarly journals Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children’s Oncology Group AREN0533 Study

2018 ◽  
Vol 36 (16) ◽  
pp. 1564-1570 ◽  
Author(s):  
David B. Dix ◽  
Nita L. Seibel ◽  
Yueh-Yun Chi ◽  
Geetika Khanna ◽  
Eric Gratias ◽  
...  
Keyword(s):  
Treatment Strategy ◽  
Wilms Tumor ◽  
Lung Metastases ◽  
Lung Nodule ◽  
Stage Iv ◽  
Event Free Survival ◽  
Free Survival ◽  
Favorable Histology ◽  
Isolated Lung ◽  
Event Rates

Purpose The National Wilms Tumor Study (NWTS) treatment of favorable histology Wilms tumor with lung metastases was vincristine/dactinomycin/doxorubicin (DD4A) and lung radiation therapy (RT). The AREN0533 study applied a new risk stratification and treatment strategy to improve event-free survival (EFS) while reducing exposure to lung RT. Methods Patients with favorable histology Wilms tumor and isolated lung metastases showing complete lung nodule response (CR) after 6 weeks of DD4A continued receiving chemotherapy without lung RT. Patients with incomplete response (IR) or loss of heterozygosity at chromosomes 1p/16q received lung RT and four cycles of cyclophosphamide/etoposide in addition to DD4A drugs (Regimen M). AREN0533 was designed to preserve a 4-year EFS of 85% for lung nodule CR and improve 4-year EFS from 75% to 85% for lung nodule IR. Results Among 292 assessable patients, 133 had CR and 159 had IR. For patients with CR, 4-year EFS and overall survival (OS) estimates were 79.5% (95% CI, 71.2% to 87.8%) and 96.1% (95% CI, 92.1% to 100%), respectively. Expected versus observed event rates were 15% and 20.2% ( P = .052), respectively. For patients with IR, 4-year EFS and OS estimates were 88.5% (95% CI, 81.8% to 95.3%) and 95.4% (95% CI, 90.9% to 99.8%), respectively. Expected versus observed event rates were 25% and 12.2% ( P < .001), respectively. Overall, 4-year EFS and OS were 85.4% (95% CI, 80.5% to 90.2%) and 95.6% (95% CI, 92.8% to 98.4%) compared with 72.5% (95% CI, 66.9% to 78.1%; P < .001) and 84.0% (95% CI, 79.4% to 88.6%; P < .001), respectively, in the predecessor NWTS-5 study. Conclusion Excellent OS was achieved after omission of primary lung RT in patients with lung nodule CR, although there were more events than expected. EFS was significantly improved, with excellent OS, in patients with lung nodule IR using four cycles of cyclophosphamide/etoposide in addition to DD4A drugs. The overall AREN0533 treatment strategy yielded EFS and OS estimates that were superior to previous studies.

Decision analysis to compare treatment strategies for Stage I/favorable histology Wilms tumor

2010 ◽  
Vol 54 (7) ◽  
pp. 879-884 ◽  
Author(s):  
A. Lindsay Frazier ◽  
Robert C. Shamberger ◽  
Tara O. Henderson ◽  
Lisa Diller
Keyword(s):  
Decision Analysis ◽  
Wilms Tumor ◽  
Treatment Strategies ◽  
Stage I ◽  
Favorable Histology

Prognostic factors for children with recurrent Wilms' tumor: results from the Second and Third National Wilms' Tumor Study.

1989 ◽  
Vol 7 (5) ◽  
pp. 638-647 ◽  
Author(s):  
P Grundy ◽  
N Breslow ◽  
D M Green ◽  
K Sharples ◽  
A Evans ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Wilms Tumor ◽  
Survival Rates ◽  
Initial Therapy ◽  
Chemotherapeutic Agents ◽  
Stage I ◽  
Prognostic Features ◽  
Favorable Histology ◽  
Tumor Study ◽  
Unilateral Disease

The characteristics of 367 stage I-IV National Wilms' Tumor Study (NWTS) children who relapsed after initial treatment for unilateral disease in the second and third NWTS trials (NWTS-2 and -3) were analyzed to identify features predictive of survival. Although modifications in initial therapy resulted in a lower rate of first relapse in these two studies compared with NWTS-1, all previously identified prognostic factors after relapse remained statistically significant predictors of survival. Tumor histology, length of initial remission, initial therapy with two v three drugs, and site of relapse each were independently predictive of postrelapse survival. The 3-year postrelapse survival for all 367 patients was 30% +/- 3%; however, subgroups classified by these prognostic factors were identified that had 3-year postrelapse survival rates of greater than 40%. These subgroups included patients who had tumors of favorable histology (FH) that recurred (1) only in the lungs, (2) in the abdomen when radiotherapy (RT) was not initially given, or (3) that were originally stage I, (4) that were originally treated with only two drugs, or (5) that recurred 12 months or more after diagnosis. These results were achieved with the use of standard treatments, ie, surgery, RT, and chemotherapy using dactinomycin (AMD), vincristine (VCR), and Adriamycin [( ADR] doxorubicin; Adria Laboratories, Columbus, OH). It is suggested that patients in these groups might be managed with aggressive use of conventional therapies until newer chemotherapeutic agents and drug combinations are shown to be more effective. Patients with adverse prognostic features at relapse have a poor survival expectancy with standard measures. Salvage attempts for these patients are better based on experimental approaches.

Effect of duration of treatment on treatment outcome and cost of treatment for Wilms' tumor: a report from the National Wilms' Tumor Study Group.

1998 ◽  
Vol 16 (12) ◽  
pp. 3744-3751 ◽  
Author(s):  
D M Green ◽  
N E Breslow ◽  
J B Beckwith ◽  
J Z Finklestein ◽  
P Grundy ◽  
...  
Keyword(s):  
Single Dose ◽  
Divided Dose ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Duration Of Treatment ◽  
Total Treatment Cost ◽  
Administration Schedule ◽  
Treatment Regimens ◽  
Favorable Histology ◽  
Tumor Study

PURPOSE National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of the administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS Between August 6, 1986 and September 1, 1994, 905 previously untreated children aged younger than 16 years with stage II favorable histology (FH) WT (low-risk [LR]), stages III to IV FH WT, or stages I to IV clear-cell sarcoma of the kidney (high-risk[HR]) were randomized after the completion of 6 months of chemotherapy to discontinue (short) or continue for 9 additional months (long) treatment with chemotherapy regimens that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either divided-dose (STD) courses (3 days) or single-dose (PI) treatment with doxorubicin. RESULTS The 4-year relapse-free survival (RFS) rates after the second randomization for LR patients were 83.7% for the 190 patients treated with short and 88.2% for the 187 patients treated with long chemotherapy (P = .11). The 4-year RFS rates after the second randomization for HR FH patients were 89.7% for the 256 patients treated with short and 88.8% for the 246 patients treated with long chemotherapy (P = .87). The charge for treatment with the short PI treatment regimens for all children with stages I through IV FH WT was approximately one half of that with the long STD treatment regimens. CONCLUSION The short administration schedule for the treatment of children with WT is no less effective than the long administration schedule and can be administered at a substantially lower total treatment cost.

scholarly journals Wilms Tumor Treatment Outcomes: Perspectives From a Low-Income Setting

2017 ◽  
Vol 3 (5) ◽  
pp. 555-562 ◽  
Author(s):  
Festus Njuguna ◽  
Hugo A. Martijn ◽  
Robert Tenge Kuremu ◽  
Peter Saula ◽  
Patel Kirtika ◽  
...  
Keyword(s):  
Treatment Failure ◽  
Treatment Outcomes ◽  
Low Income ◽  
Wilms Tumor ◽  
High Income ◽  
Stage I ◽  
Event Free Survival ◽  
Free Survival ◽  
Stage Of Disease ◽  
Relapsed Disease

Purpose Wilms tumor is the commonest renal malignancy in childhood. Survival in high-income countries is approximately 90%, whereas in low-income countries, it is less than 50%. This study assessed treatment outcomes of patients with Wilms tumor at a Kenyan academic hospital. Patients and Methods We conducted a retrospective medical record review of all children diagnosed with Wilms tumor between 2010 and 2012. Data on treatment outcomes and various sociodemographic and clinical characteristics were collected. Results Of the 39 patients with Wilms tumor, 41% had event-free survival, 31% abandoned treatment, 23% died, and 5% had progressive or relapsed disease. Most patients presented at an advanced stage: stage I (0%), II (7%), III (43%), IV (40%), or V (10%). The most likely treatment outcome in patients with low-stage (I to III) disease was event-free survival (67%), whereas in those with high-stage (IV to V) disease, it was death (40%). No deaths or instances of progressive or relapsed disease were recorded among patients with low-stage disease; their only reason for treatment failure was abandonment of treatment. Stage of disease significantly affected treatment outcomes ( P = .014) and event-free survival estimates ( P < .001). Age at diagnosis, sex, duration of symptoms, distance to hospital, and health insurance status did not statistically significantly influence treatment outcomes or event-free survival estimates. Conclusion Survival of patients with Wilms tumor in Kenya is lower compared with that in high-income countries. Treatment abandonment is the most common cause of treatment failure. Stage of disease at diagnosis statistically significantly affects treatment outcomes and survival.

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.

Sign in / Sign up

Export Citation Format

Share Document