Incidence of childhood renal tumours: An international population-based study.

IntroductionRenal cancers constitute about 6%–7% of all childhood tumours. Wilms’ tumour is the most common renal tumour in childhood. Clear cell sarcoma, malignant rhabdoid tumour, congenital mesoblastic nephroma, renal cell...

Renal tumors are not uncommon in children. In this study, we aimed to evaluate the clinical and pathological features of renal tumors in children. Between January 2008 and December 2017, the records of children with renal tumors in our institution were retrospectively analyzed. Data collected were composed of demographic and clinical characteristics including gender, age at time of diagnosis, symptoms, laterality of the tumor and pathological evaluation. A total of 48 children with renal tumor (28 males and 20 females) were included in the study. They were diagnosed at mean age of 53.26±46.64 months (range: 1-192) and the mean follow-up period was 73.45±48.92 months (range: 6-120). The most common symptom was a lump or mass in the area of the kidneys (45.8%), abdominal pain and hematuria (14.6%). Four patients (8.3%) were diagnosed at antenatal period. 68.8% of the children had Wilms tumor and the major histological groups of non-Wilms renal tumors were renal cell carcinoma (12.5%), congenital mesoblastic nephroma (10.4%) and angiomyolipoma (4.2%). 10.4% of the children had bilateral tumors and one patients had Wilms tumor with horseshoe kidney. 87.5% of the children were treated with surgery and of those 7 (14.5%) underwent nephron-sparing surgery. The patients had chemotherapy and radiotherapy (83.3% and 41.7%, respectively). Seven patients (14.6%) died during follow-up. Wilms tumor is the most common pediatric renal neoplasm. On the other hand, we showed that considerable number of children with renal tumors had non-Wilms tumors including renal cell carcinoma, congenital mesoblastic nephroma and angiomyolipoma.

Wilms tumor is the most common renal tumor of childhood. However, other epithelial, mesenchymal, and neuroectodermal neoplasms may also arise in the kidney during childhood, several of which show specific age distributions; in the 1st year of life, mesoblastic nephroma and rhabdoid tumor are more common, whereas renal cell carcinoma, primitive neuroectodermal tumor, and anaplastic Wilms tumors are relatively more frequent in older children and adolescents. The aim of this study is to describe the spectrum of renal tumors in children aged 10-16 years using data from 1492 patients registered in the UK Wilms Tumour 3 Trial (1991-2001) and International Society of Paediatric Oncology Wilms Tumour Trial 2001 (2002-2008) clinical trials of renal tumors in childhood. There were 67 (4.6%) tumors in children aged 10-16 years: 50 Wilms tumors (74.6%), 10 (14.9%) renal cell carcinomas, 3 (4.5%) renal medullary carcinomas, 2 (3%) primitive neuroectodermal tumors, 1 clear cell sarcoma of kidney, and 1 desmoplastic small round cell tumor. Fourteen percent of the Wilms tumors in this age group had diffuse anaplasia. Among the 10 renal cell carcinomas, 4 were associated with t(Xp-11.2), 3 were of papillary type II, 1 was papillary type I, 1 was clear cell type, and 1 was unclassified. Five-year overall survival for Wilms tumor was 63% (43% for anaplastic tumors), significantly lower than reported overall survival for all pediatric Wilms tumors. Only 40% of patients with renal cell carcinoma survived, and all patients with other tumors died.

Aim: Renal tumors in children are rare. Wilms’ tumor (WT) is the most common renal tumor in childhood. The aim of this study is to investigate the data on the demographic factors, treatment, and follow-up results of pediatric patients who were operated on for renal tumors and determine the factors affecting mortality. Material and Method: Patients who were operated for renal tumor in our clinic in 2005-2020 were evaluated retrospectively. Age at diagnosis, gender, complaint, localization of the involved kidney, additional anomalies, tumor size, tru-cut biopsy performed, tumor stage, distant metastasis, applied treatments, pathology, treatment complications, and follow-up period were examined. Results: Of the 47 patients, 25 were female and 22 were male. The mean age was 45.46 (2–204) months. The tumor was located in the right in 21 cases, left in 23 cases and bilateral in three cases. The mean length of the tumor was 11.73 cm. At the time of diagnosis, there was metastasis in 15 cases (31.9%). While metastasis were the most common seen in lung (24.3%), bone-cell metastases were seen in clear cell carcinoma (33.3%) and brain metastases were seen in rhabdoid tumor (50%). Tru-cut biopsy was performed in 15 (31.9%) cases and biopsy was diagnostic in ten cases. Histopathologically, 41 cases had WT (87.23%), three cases were clear cell carcinoma (6.3%), two cases had anaplasia rhabdoid tumor (4.2%), and one case had metanephric adenoma. Relaps occured in nine cases (19.14%) during the follow-up period. Histopathologically, one case was a clear cell carcinoma, one case was a rhabdoid tumor, and seven cases were unilateral WT. The survival rate of our series was 89,4%. Conclusion: The most common renal tumor in childhood was WT. Surgery have no effect on survival; the most important survival factors were detecting the existence of rhabdoid tumors and anaplasia histopathologically. Therefore, during the follow-up and treatment of renal tumors in childhood, pathological examination should be of primary importance and followed up with the pediatric oncologists.

Renal masses are most common in children between ages 1 to 3 years, with less known about renal tumors in older children and young adults. The aim of this study was to review the presentation, demographics, histology, and outcomes in patients over 5 years of age with renal tumors compared with younger children. 111 renal tumors were diagnosed in patients 5 years of age and older (median, 7 y; range, 5 to 31 y) between 1950 and 2017 at a single institution. Wilms tumor (WT) was the most common histology in 84 patients (75%), followed by renal cell carcinoma in 12 patients (10.7%). Abdominal pain was the most common presenting symptom (46%) followed by hematuria (28.8%), and a palpable abdominal mass (24.3%). For WT, older children more commonly presented with advanced-stage disease (stages 3 and 4) than younger children (57.7% vs. 11.5%; P<0.001). Event-free survival (EFS) and overall survival (OS) for favorable histology WT were not different between younger and older children (OS, P=0.43; EFS, P=0.46). In this cohort, older children more frequently present with variable signs and symptoms, less common histopathologies although WT was still most frequent, and more advanced-stage disease compared with younger cohorts, but without differences in EFS or OS.

This chapter discusses renal tumours in children and adolescents. Nephroblastoma or Wilms tumour (WT) is the most common childhood renal tumour, with an incidence of seven per one million children below 15 years of age. Diagnosis and treatment is done within prospective multicentre clinical trials conducted by large international study groups (International Society of Paediatric Oncology, SIOP; Children’s Oncology Group, COG). The outcome of WT is excellent, with around 90% survival rates, depending on age, stage histological subtyping, response to treatment, and molecular markers. Other renal tumours in childhood are clear-cell sarcoma of the kidney (CCSK), rhabdoid tumour of the kidney (RTK), renal-cell carcinoma (RCC), and congenital mesoblastic nephroma (CMN). Their treatment and outcome is different to WT.

Imaging accuracy and incidence of Wilms' and non-Wilms' renal tumors in children

Wilms tumor is the most common primary renal tumor in childhood. Children with Wilms tumor typically presentwith an asymptomatic abdominal mass, usually detected on a routine medical checkup or discovered coincidentallyby parents. The initial differential diagnosis is with extrarenal abdominal masses; once a tumor of renal origin isestablished, distinguishing between Wilms tumor and other primary renal neoplasms such as congenital mesoblasticnephroma, clear cell sarcoma, malignant rhabdoid tumor and renal cell carcinoma may not be easy.However, in many cases imaging findings in conjunction with the patient’s clinical and epidemiological data, allowthe diagnosis of Wilms tumor. Wilms tumor care offers one of the most striking examples of success of pediatric oncology. Over the last decades the European SIOP studies have been the key to developing standardized diagnosticprocedures, improved risk stratification, and adjusted treatment recommendations for children with Wilms tumor andthis has resulted rate of overall survival is currently greater than 90%. As in previous SIOP trials and studies, the newprotocol for the diagnosis and treatment of childhood renal tumors, the UMBRELLA SIOP–RTSG 2016, mandatespreoperative chemotherapy without preceding mandatory histological assessment.Therefore, imaging studies are essential to obtain a presumptive diagnosis of WT, to provide disease staging information and to measure the tumor volume after neoadjuvant chemotherapy for the purposes of postoperative treatmentstratification. This review describes role of imaging in the management of children with Wilms tumor, according tothe current recommendations of the UMBRELLA protocol.

Wilms tumor is the most common primary renal tumor in childhood. Children with Wilms tumor typically present with an asymptomatic abdominal mass, usually detected on a routine medical checkup or discovered coincidentally by parents. The initial differential diagnosis is with extrarenal abdominal masses; once a tumor of renal origin is established, distinguishing between Wilms tumor and other primary renal neoplasms such as congenital mesoblastic nephroma, clear cell sarcoma, malignant rhabdoid tumor and renal cell carcinoma may not be easy. However, in many cases imaging findings in conjunction with the patient's clinical and epidemiological data, allow the diagnosis of Wilms tumor. Wilms tumor care offers one of the most striking examples of success of pediatric oncology. Over the last decades the European SIOP studies have been the key to developing standardized diagnostic procedures, improved risk stratification, and adjusted treatment recommendations for children with Wilms tumor and this has resulted rate of overall survival is currently greater than 90%. As in previous SIOP trials and studies, the new protocol for the diagnosis and treatment of childhood renal tumors, the UMBRELLA SIOP–RTSG 2016, mandates preoperative chemotherapy without preceding mandatory histological assessment. Therefore, imaging studies are essential to obtain a presumptive diagnosis of WT, to provide disease staging information and to measure the tumor volume after neoadjuvant chemotherapy for the purposes of postoperative treatment stratification. This review describes role of imaging in the management of children with Wilms tumor, according to the current recommendations of the UMBRELLA protocol.

ObjectiveThis study evaluates the long-term trends in Multiple Myeloma (MM) incidence, mortality, and Age-Period-Cohort (APC) effects in the BRICS nations (Brazil, Russia, India, China, and South Africa).MethodsData on age-standardized incidence rate (ASIR), age-standardized mortality rate (ASMR), and 95% uncertainty intervals (UIs), were obtained from the Global Burden of Disease Study 2021. Joinpoint regression model was used to estimate the average annual percentage change (AAPC) and annual percentage change (APC) trends from 1992 to 2021, and the Age-Period-Cohort model evaluated nonlinear impacts of age, period, and cohort effects. Projections to 2046 were calculated using Bayesian APC modeling.ResultsFrom 1992 to 2021, MM incidence and death cases in the BRICS nations increased nearly four to fivefold, with ASIR and ASMR nearly doubling. China and India had lower ASIR and ASMR than other BRICS countries despite accounting for over half of cases and deaths. South Africa consistently had the highest ASIR and ASMR in both 1992 and 2021. China experienced a significant increase in ASIR (AAPC 4.92%, p < 0.001) and ASMR (AAPC 4.07%, p < 0.001) over the past three decades. MM incidence and mortality increased with aging, and the age effect on MM was more pronounced among individuals aged greater than 40 years. Birth cohorts’ impact on MM varied greatly between BRICS, with China suffering the largest risk increase among those born after the 1970s. Projections indicate ASIR and ASMR will reach 2.44 and 1.82 per 100,000 by 2046, continuing to rise across the BRICS nations.ConclusionsMM burden is rapidly increasing in the BRICS, closely tied to population aging. Targeted strategies addressing each country’s unique challenges are essential as the burden continues to grow.

Laparoscopic radical nephrectomy for the management of chromophobe renal cell carcinoma in a 13-year-old girl

After some decades with an increasing incidence of borderline ovarian tumors, more recent studies have observed that the incidence rate seems to be leveling off or declining. In this study, we describe the incidence of borderline ovarian tumors in Denmark 1997-2018 by histology, age at diagnosis and educational level. All borderline ovarian tumors registered in the Danish Pathology Registry during 1997-2018 were identified and individual-level educational information was retrieved from nationwide registers. Age-standardized incidence rates were estimated according to histology, age at diagnosis and educational level. To investigate incidence trends over time, the average annual percentage change and corresponding 95% confidence intervals (CIs) were estimated using Poisson regression. We identified 3927 women with borderline ovarian tumors during the study period, of which 1997 (50.9%) were serous and 1743 (44.4%) were mucinous. The age-standardized incidence rate of serous borderline ovarian tumors did not change significantly over calendar time (average annual percentage change=-0.13, 95% confidence interval [CI] -1.13 to 0.88). For mucinous tumors, the age-standardized incidence rate was also relatively stable during the first half of the study period, followed by a decrease from 2.56 to 1.25 per 100000 person-years between 2007-2011 and 2017-2018. Over the entire study period, the incidence rate of mucinous borderline tumors declined on average by 2.91% (95% CI -4.24 to -1.51) per year. The incidence of both types of borderline ovarian tumors seemed to be highest among women with a low educational level. Over calendar time, the incidence of mucinous tumors decreased in all educational groups, whereas the incidence of serous tumors decreased exclusively in women with a high educational level. Time trends did not differ markedly by age at diagnosis. In Denmark, the incidence of serous borderline ovarian tumors was stable during 1997-2018, whereas the incidence of mucinous borderline ovarian tumors decreased. The incidence rates of both types of borderline ovarian tumors tended to be highest among women with a low educational level throughout the study period.

Objectives Ovarian cancer (OC) is the deadliest gynecological cancer in the world. Deeper knowledge over time is the basis for global studies to design and implement effective measures to reduce inequalities; this study was conducted to investigate the trend of OC incidence and management in the world from 1990 to 2019. Methods We obtained crude numbers and age-standardized rate (ASRs) of OC annually from the 2019 Global Burden of Disease (GBD) study to examine OC’s morbidity, mortality rates, and disability-adjusted life years (DALYs) based on age group, sociodemographic index (SDI), WHO regions, continents, World Bank regions, and GBD regions from 1990 to 2019 in 204 countries and territories. The relative change (%) and average Annual Percent Change (AAPC) were used to display the epidemiological trend. Results Globally, the number of OC incidents increased from 141,706 in 1990 to 294,422 in 2019. Despite the age-standardized incidence rate (ASIR) in regions with high SDI having a downward trend, these areas recorded the highest incidence cases and ASIR in 2019. Although the World Bank high-income level had the most frequent incidence cases and ASIR, the ASIR in these regions decreased from 1990 to 2019. Among the continents, Europe and America have the highest ASIR but experienced a decreasing trend from 1990 until 2019 in ASIR. The age-standardized mortality rate (ASMR) in the World Bank high-income level experienced a decreasing trend in 1990–2019. In contrast, in the middle, low-middle, and low SDI regions, the death number increased approximately 3.5–4.1 times, and the ASMR had a significant increase from 0.5 in the middle to 0.75 in the low-middle SDI. Globally, the DALY cases of OC rose from 2,732,666 in 1990 to 5,359,737 in 2019; almost doubling. A significant decrease in the DALYs ASR was observed in seven GBD regions. The most pronounced decrease was found in Australia. Conclusions The trend of OC incidence and burden and approximate mortality were stable from 1990 to 2019; especially in lower socioeconomic areas and low-income countries; while the incidence ASR of this cancer in the high SDI regions decreased from 1990 to 2019. The key to reducing OC remains in primary prevention. Approaches such as weight loss, a healthy lifestyle and diet, promoting childbearing and breastfeeding, and recommending the use of oral contraceptives in eligible individuals can have a protective effect against this silent killer.

Wilms tumour is the most common renal tumour in children, accounting for above 90% of cases and comprising 8% of paediatric solid tumours. In adults, it is extremely rare and represents only 0.5% of all renal tumours. We reviewed 9 patients older than 16 years: three were treated in the Department of Paediatric Surgery and three in the Department of Urology; additionally, 3 patients were referred to us for consultation with regard to further treatment after having been operated on previously in other centres. Clinical presentation, stage distribution, histology, treatment, and prognosis of all the patients are presented. The age of 9 patients ranged from 17 to 32 years: there were 8 men and 1 women. Tumour stages were defined according to SIOP classification: 4 were of local stage II, 3 of stage III, and 2 of stage IV with metastases to lungs and bones. Three of them were treated according to the paediatric protocol of the Society of Paediatric Oncology (SIOP 93-01 protocol)-all after a fine-needle biopsy which confirmed nephroblastoma, received preoperative chemotherapy of two drugs (VCR + ACT D) during 4 weeks. Histological studies revealed a low-risk cystic tumour in one case, intermediate-risk tumour in 2 patients, and high-risk tumour with blastemal predominance or with the presence of diffuse anaplasia in 5 patients. One tumour was defined as a mixed high-risk tumour: renal cell carcinoma with nephroblastoma of blastemal predominance. Complete remission was achieved in 3 patients. Three relapsed after complete remission of 6-30 months. A second-line therapy was undertaken in these patients as well as in those who had undergone the initial treatment elsewhere. But the second-line therapy proved ineffective. Adult patients with Wilms tumour have more aggressive histology and poorer prognosis than children.

We present the preliminary results of robotic-assisted laparoscopic (RAL) total and partial nephrectomy for renal malignant tumors in children. This is a prospective study of patients operated with RAL between December 2016 and September 2018. Patients with Wilms tumors were treated according to the SIOP-2001 protocol. Patient and tumor characteristics, type of surgery, surgical-related morbidity, and oncologic outcomes were recorded. Results were compared with a series of patients with similar age- and tumor-related characteristics operated during the same period by an open surgical approach. Ten children underwent RAL nephrectomy with a mean age of five years (3.2-14.1 years). Total nephrectomy was done in six cases for Wilms tumor and in one case for renal sarcoma; three cases were converted. Complete removal of tumor without rupture was achieved in all cases. Postoperative course was uneventful, and patients were discharged between days 2 and 7. Neither recurrence nor medium-term complications occurred. Nine patients are alive with a median follow-up of 16 months (6-27 months) and one female died from complications of central nervous system metastases one year after surgery. When compared with the open surgical approach group, median tumor volume was smaller (P=0.005), hospital stay was shorter (P=0.01), and operative time was similar (P=0.20). RAL total and partial nephrectomy procedure for renal tumor in children may be an option in carefully selected cases. Indication should be discussed at tumor boards and surgery performed while adhering strictly to oncological surgical rules.