China
Africa
Explore chapters and articles related to this topic
Molecular Approaches Towards the Isolation of Pediatric Cancer Predisposition Genes
Published in John T. Kemshead, Pediatric Tumors: Immunological and Molecular Markers, 2020
Wilms’ tumor is a pediatric kidney tumor which affects about 1 in 10,000 children in the U.K. and accounts for 6% of all childhood cancers. Knudson and Strong66 estimated that 38% of all Wilms’ tumors are inherited in an autosomal dominant pattern with reduced penetrance, but familial and bilateral tumors are much less common than in Rb. For example, of the first 300 cases in the first U.K. Childhood Cancer Study Group trial, none were familial and only 4% were bilateral.67 It is clear that, if this tumor can be described as hereditary, the penetrance must be low. Analysis of chromosome deletion patients (see below) shows that there is only a 50% chance of tumor formation in the presence of a predisposing deletion68 which also indicates reduced penetrance of the gene. Several examples of familial Wilms’ tumor have been reported.69,70 In the U.K., the mean age of onset of the bilateral cases is 30 months compared with 42 months for the unilateral cases supporting a two-hit hypothesis.
Mulibrey Nanism
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Further, mulibrey nanism patients who develop Wilms tumor may show swelling in the abdomen, blood in the urine and abnormal urine color, fever, poor appetite, high blood pressure, abdominal or chest pain, nausea, constipation, large and distended veins across the abdomen, malaise or feeling unwell, vomiting, unexplained weight loss.
Paediatric cancer
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Wilms’ tumour is both radio- and chemosensitive and the prognosis for localized disease is therefore very good with 80%–90% of patients with stage 1 or 2 disease being cured. There are a number of important prognostic features including anaplasia, aneuploidy, lymph node or hepatic metastases and intravascular tumour thrombus, and loss of heterozygosity (LOH) at 1p and 16q; however, even those presenting with distant metastases (stage 4) have a cure rate of over 70%.
Pediatric Primary Yolk Sac Tumour of the Kidney: Recommendations for Pretreatment Diagnosis
Published in Fetal and Pediatric Pathology, 2023
Shilpi Thakur, Aanchal Kakkar, Manisha Jana, Prasenjit Das, Sandeep P. Agarwala, Venkateswaran K. Iyer
The primary objective of pre-chemotherapy tissue diagnosis of pediatric renal masses is to separate Wilms tumor from non-Wilms tumors, as their management protocols differ, and this holds true for GCTs as well. YSTs are exquisitely responsive to platinum-based chemotherapy i.e. bleomycin, etoposide and cisplatin, with survival rates following chemotherapy reaching 100% [14]. Surgical excision of residual masses may or may not be necessary depending on the response. Wilms tumor is treated with chemotherapy (vincristine, actinomycin D, doxorubicin) followed by surgical resection. This difference in management necessitates preoperative distinction of intrarenal YSTs from Wilms tumor. On radiology, YST lacks any distinguishing features that would help differentiate it from Wilms tumor [15]. Both can present as heterogeneous solid, enhancing mass lesions, and have a well-defined margin causing ‘displacement’ rather than invasion of adjacent organs. One important imaging marker described in YST is the presence of small enhancing tumoral nodules within the renal parenchyma with rim enhancement. Secondly, unlike Wilms tumor, YST can show invasion into the pelvicalyceal system [15]. In the present case, there were no atypical clinical or imaging features to suggest that this was not a Wilms tumor
Syrian and Turkish children with cancer: a comparison on survival and associated factors
Published in Pediatric Hematology and Oncology, 2020
Begül Yağcı-Küpeli, Ayşe Özkan
Overall survival was 55.7% in Syrian patients in our study and this rate is lower than our Turkish study population and survival rates in most of the developed countries. Metastatic or advanced-stage disease, poor compliance to treatment and progression or relapse were associated with lower survival rates in refugees in our study. Accessibility to the diagnostic and therapeutic interventions have crucial role in curing cancer. Despite increasing survival rates in developed countries, a substantial number of children who live in underdeveloped countries face difficulties in reaching cancer treatment.10 Ekenze et al. reported overall survival of 53.3% in patients with Wilms tumor which is one of the best cured cancer of childhood. They reported late presentation of patients with large and advanced stage tumors, inadequate diagnostic tools such as imaging techniques or biological studies for tumor tissue, lack of radiotherapy, poor compliance to treatment as reasons for inferior outcome.11
High-dose chemotherapy and autologous hematopoietic stem-cell rescue for treatment of relapsed and refractory Wilms tumor: Re-evaluating outcomes
Published in Pediatric Hematology and Oncology, 2018
Jenna Rossoff, William T. Tse, Reggie E. Duerst, Jennifer Schneiderman, Elaine Morgan, Morris Kletzel, Sonali Chaudhury
Survival for patients with relapsed and refractory Wilms tumor has improved; however, it remains far inferior to those responding to initial therapy.2 We reviewed our single institution experience with high-risk Wilms tumor patients and showed that HD-ASCR is well tolerated with low TRM and overall low long-term morbidity. Three- and five-year DFS and OS to be 46% (CI 25–67%) and 60% (CI 39–80%), and 40% (CI 19–61%) and 54% (CI 33–75%), respectively. This is worse than our institution’s previous report due to late relapse.4 Our results are similar to those reported from other centers, with DFS and OS ranging from 34% to 64% and 48% to 73%, respectively.4–10 Malogolowkin, et al. recently published one of the largest studies on 253 patients with relapsed Wilms tumor who were reported to the CIBMTR with five-year event-free survival and OS 36% and 45%, respectively.14 Not only did this study show similar DFS and OS to ours, but it validated our low rates of TRM with the majority of deaths resulting from relapse of primary disease.