Secondary malignant neoplasms after Wilms tumor: An international collaborative study (original) (raw)
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Annals of Oncology, 2012
Background: The randomised findings of the UKW3 trial were that preoperative chemotherapy was associated with a more advantageous stage distribution and reduction in therapy burden versus immediate nephrectomy without compromising outcome in localised Wilms' tumour (WT). We analysed outcome in all WT registered in UKW3. Patients and methods: Seven hundred and eighteen WT cases (7% anaplastic) were registered in UKW3. We assigned a treatment stage and conducted survival analysis. Results: Five-year event-free survival (EFS) and overall survival (OS) were 77.2% [95% confidence interval (CI) 73.9-80.2] and 87.5% (95% CI 84.8-89.7) after median follow-up of 9.5 years and 10.0 years, respectively. Five-year OS in localised non-anaplastic cases was 92.9% (95% CI 90.2-94.9). Anaplasia was associated with adverse outcome compared with non-anaplastic cases: 5-year EFS of 42.0% (95% CI 28.3-55.1) versus 79.8% (95% CI 76.5-82.7) and 5-year OS of 60% (95% CI 45.1-72.0) versus 89.6% (95% CI 87.0-91.7), respectively. Outcomes were similar for nonanaplastic stage I or II but significantly poorer in stage III cases than stage I. Five-year OS after relapse was 54.1% (95% CI 44.5-62.8). Forty-seven percent of non-anaplastic WT received anthracycline; 27% were treated with radiotherapy first line. Conclusion: These outcomes provide a baseline for future comparisons of WT treatment approach, burden and patient outcome.
Current status and future perspectives of wilms tumor treatment in Iran
Background: Wilms tumor (WT) is among the most common pediatric malignancies. In this study, the authors tried to evaluate the adherence to internationally-approved WT treatment protocols in our tertiary medical center in Iran. Methods: In this retrospective study, the medical records of 72 pathologically confirmed WT patients who underwent treatment from April 2014 to February 2020 were evaluated. Demographic characteristics, histologic features of the tumors and metastases, utilized treatments, and survival rates were subsequently investigated. Results: From the total of 72 patients, 31 (43.1%) and 41 (56.9%) were males and females, respectively. The median age at the time of diagnosis was 44.0 (interquartile range: 18.5, 72.0) months. Among the patients, favorable histology was observed in 68 (94.6%) patients, while 4 (5.4%) patients had unfavorable histology. Regarding chemotherapy, 34/56 (60.7%), 4/56 (7.1%), and 18/56 (32.2%) received adjuvant, neoadjuvant, and combined chemotherapy, respectively. The mean numbers of neoadjuvant and adjuvant chemotherapy sessions were 9.4 ± 5.6 and 14.5 ± 11.1, respectively. 32/72 (44.4%) of the patients received adjuvant radiotherapy with a mean number of 7.3 ± 3.6 sessions. Overall survival rates were 86% at 1-year, 74% at 3-year, and 62% at 5-year. Conclusion: Our results suggested that while the demographic characteristics of WT patients in Iran resemble those in other countries, abidance to internationally recommended protocols is relatively low. Moreover, survival rates were rather dismal in our study compared to those from other developing countries, further signifying the need for the development of a nation-specific treatment protocol for WT.
European Journal of Cancer, 2022
Background: Anaplasia is an unfavorable prognostic histologic feature in Wilms tumor (WT). Patients with stage I anaplastic WT (AWT) typically achieve good outcomes, albeit with more treatment than for stage I non-anaplastic WT. Since the SIOP-WT-2001 study, patients with focal AWT (FAWT) have been classified as intermediate-risk and received less intense treatment than patients with diffuse AWT (DAWT). The aim of the study was to analyze outcome in these patients. Patients and Methods: Retrospective analysis of clinico-pathologic features and outcomes of 59 patients with stage I AWT (19 FAWT, 40 DAWT) from the SIOP-WT-2001 GPOH and UK-CCLG groups. The patients with FAWT were treated as intermediate-risk WT, with eight weeks of vincristine and actinomycin D (four weeks pre-operatively, and four weeks post-operatively). For comparison, we also assessed outcomes in 818 patients with stage I intermediate-risk non-anaplastic WT (IR-non-AWT). The patients with DAWT were treated with vincristine, actinomycin D and doxorubicin for 31 weeks. No group received radiotherapy. Results: Median follow-up was 67.6 months. 4-year EFS and OS in the FAWT group were 87% (95% CI 72-100) and 100%, respectively, in the DAWT group 85% (95% CI 74-98) and 93% (95% CI 85-100), respectively, and in the IR-non-AWT group 91% (95% CI 89-93) and 98% (95% CI 97-99), respectively. Conclusions: Outcomes for patients with stage I FAWT were comparable with those of other, identically treated, patients with stage I IR-non-AWT. Patients with stage I DAWT 4 also showed good outcomes, albeit with more intensive chemotherapy than IR-non-AWT, but without radiotherapy.
Evaluation of Late Adverse Events in Long-Term Wilms' Tumor Survivors
International Journal of Radiation Oncology*Biology*Physics, 2010
Purpose: To evaluate the prevalence and severity of adverse events (AEs) and treatment-related risk factors in long-term Wilms' tumor (WT) survivors, with special attention to radiotherapy. Methods and Materials: The single-center study cohort consisted of 185 WT survivors treated between 1966 and 1996, who survived at least 5 years after diagnosis. All survivors were invited to a late-effects clinic for medical assessment of AEs. AEs were graded for severity in a standardized manner. Detailed radiotherapy data enabled us to calculate the equivalent dose in 2 Gy fractions (EQD 2) to compare radiation doses in a uniform way. Risk factors were evaluated with multivariate logistic regression analysis. Results: Medical follow-up was complete for 98% of survivors (median follow-up, 18.9 years; median attained age, 22.9 years); 123 survivors had 462 AEs, of which 392 had Grade 1 or 2 events. Radiotherapy to flank/abdomen increased the risk of any AE (OR, 1.08 Gy À1 [CI, 1.04-1.13]). Furthermore, radiotherapy to flank/abdomen was associated with orthopedic events (OR, 1.09 Gy À1 [CI, 1.05-1.13]) and second tumors (OR, 1.11 Gy À1 [CI, 1.03-1.19]). Chest irradiation increased the risk of pulmonary events (OR, 1.14 Gy À1 [CI, 1.06-1.21]). Both flank/abdominal and chest irradiation were associated with cardiovascular events (
The changing management of Wilms' tumor over a 30-year period 1949-1978
Cancer, 1985
Seventy-four patients with histologically confirmed diagnoses of Wilms' tumor were treated between 1949 and 1978 at the Mallinckrodt Institute of Radiology and St. Louis Children's Hospital. The results have been divided into two eras of therapy, Le., before and after 1965, when chemotherapy became a major modality for Wilms' tumor therapy. Analysis at 5 years by era of therapy has shown similar disease-free survival results for Stage I ("prechemotherapy" era 67% versus "chemotherapy" era 75%). whereas the Stage I1 ("prechemotherapy" era 33% versus "chemotherapy" era 100%) and
Results of novel strategies for treatment of Wilms' tumor
International braz j urol, 2007
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% and 76.7 ± 6.6%. Short duration therapy for stage I tumor showed a disease-free survival rate of 100% 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%. 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.
Response rates in relapsed wilms' tumor. A need for new effective agents
Cancer, 1991
Three hundred eighty-one children with Wilms' tumor were treated in the United Kingdom Children's Cancer Study Group WT1 Study (1982 to 1986). Seventy-one patients had relapses during or after treatment with surgery and chemotherapy, and radiation therapy, depending on stage and histologic characteristics. Forty-nine patients were evaluable for disease response to second-line chemotherapy alone, Evaluation of response to chemotherapy was impossible in the remaining patients because either surgery or radiation therapy was used at the time of relapse. With second-line combination chemotherapy (which included ifosfamide, etoposide/ VM26, cisplatin/carboplatin, bleomycin, melphalan, and Thiotepa [Lederle Laboratories, Pearl River, NY]), there were five complete responses and 12 partial responses. In patients with favorable histologic findings, six of nine with Stage I, five of ten with Stage 11, none of 11 with Stage 111, three of 16 with Stage IV, and one of five with Stage V disease survived. Two survivors were treated with chemotherapy alone; the others received combined treatment with chemotherapy, radiation therapy, and/or surgery. For those with unfavorable histologic findings of any stage, only two of 20 survived. The authors conclude that, even for patients with localized disease with favorable histologic findings, the "salvage" rate is little more than SO%, and for all other stages and histologic findings the likelihood of cure after relapse is remote. There is clearly a need for additional effective chemotherapeutic agents for these patients. Cancer 67:567-571,1991. ORE THAN 85% of patients with Wilms' tumors are M cured by conventional multimodal therapy, including surgery, radiation therapy, and chemotherapy. Most recurrences are seen within 2 years of diagnosis, although metastases have developed up to 11 years after From the United Kingdom Children's Cancer Study Group Supported by the Cancer Research Campaign. t Supported by the Koningin Wilhelmina Fonds, the Dutch Organi
Journal of the Egyptian National Cancer Institute
Background: Wilms' tumor (WT) affects one in 10,000 children and accounts for 5% of all childhood cancers. Although the overall relapse rate for children with WT has decreased to less than 15 %, the overall survival for patients with recurrent disease remains poor at approximately 50 %. The aim of the study to evaluate the outcome of relapsed Wilms' tumor pediatric patients treated at the National Cancer Institute (NCI), Egypt, between January 2008 and December 2015. Results: One hundred thirty (130) patients diagnosed with WT during the study period, thirty (23%) patients had relapsed. The median follow up period was 22.3 months (range 3.6-140 months). The Overall Survival (OS) was 30.9% while the event-free survival (EFS) was 29.8% at a 5-year follow up period. Median time from diagnosis to relapse was 14.4 months. A second complete remission was attained in 18/30 patients (60%). The outcome of the 30 patients; 11 are alive and 19 had died. Three factors in our univariate analysis were prognostically significant for survival after relapse. The first was radiotherapy given after relapse (p = 0.012). The 5-year EFS and OS for the group that received radiotherapy were 41.9% versus 16.7% and 11.1% respectively for those that did not. The second was the state of lymph nodes among patients with local stage III (p = 0.004). Lastly, when risk stratification has been applied retrospectively on our study group, it proved to be statistically significant (p = 0.029). Conclusion: Among relapsed pediatric WT, radiotherapy improved survival at the time of relapse and local stage III with positive lymph nodes had the worst survival among other stage III patients.