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ASSESSING THE OCCURRENCE OF CANCER IN HUMAN POPULATIONS
Published in Richard G. Cornell, Statistical Methods for Cancer Studies, 2020
The most specific survival data in the sense of controlling for therapy have been provided in recent years through the publications emanating from the various cooperative oncology groups in the United States. Many of these investigations are clinical trials of specific therapies for particular tumors. A good entree into the work of these groups can be obtained by perusing the journal Cancer Treatment Reports for recent years. The progress in fighting Wilms’ tumor in children accomplished by the Children’s Cancer Study Group as detailed in the publications of this group is one of remarkable improvement in survival. Somewhat similar progress has been accomplished in other groups for particular tumors (non-Hodgkins lymphoma and cancer of the testis), but for the major cancer sites survival prospects have improved, at best, very little in the past 25 years. These facts bear on the appropriateness of mortality or incidence as the endpoint to be used in particular studies.
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.
Paediatric Urology
Published in Manit Arya, Taimur T. Shah, Jas S. Kalsi, Herman S. Fernando, Iqbal S. Shergill, Asif Muneer, Hashim U. Ahmed, MCQs for the FRCS(Urol) and Postgraduate Urology Examinations, 2020
Jemma Hale, Arash K. Taghizadeh
Beckwith–Wiedemann syndrome is characterised by macroglossia, macrosomia, visceromegaly and midline abdominal wall defect such as omphalocele or exomphalos. It is associated with a significantly increased risk of developing Wilms’ tumour.
Renal Yolk Sac Tumor Clinically Misdiagnosed as Nephroblastoma: A Case Report
Published in Fetal and Pediatric Pathology, 2023
Meng Zhu, Chengmao Xia, Jie Yang, Zhe Liu, Xiaowen Zhao, Yaling Li, Bin Liu, Yanli Yang, Yali She
About 30 cases of intra-renal germ cell tumor (GCT) have been reported worldwide, mostly teratoma or mixed GCT [8]. So far, only 5 cases of primary yolk sac tumor in the kidney have been reported. The case characteristics of five cases of renal primary YST were summarized in Table 1, and this case was the sixth case. These were all male patients aged 1-43 years, including 5 children and adolescents, and 1 adult. Among them was a 2-year-old boy who was admitted to hospital with a gross hematuria after trauma, which had begun 7 days earlier. Color Doppler ultrasound and enhanced CT showed a complex mass with increased vascularity in the upper pole of the left kidney. The imaging features of the mass were highly suggestive of Wilms tumor. Laboratory tests revealed AFP was significantly increased, and aspiration cytology revealed YST, so the preoperative diagnosis was correct. The other 4 cases were diagnosed as nephroblastoma clinically and radiologically, and the preoperative diagnosis of 1 case was unclear. It could be seen from these cases that there was no relatively distinctive feature in the clinical manifestations of primary renal YST. In the absence of tissue diagnosis, it may be clinically misdiagnosed as nephroblastoma on preoperative imaging, and serum AFP detection is recommended.
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