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Distribution of renal cancer subtypes in patients with suspicion of renal tumor. RCC: renal cell carcinoma, CCSK: clear cell sarcoma of the kidney, MN: mesoblastic nephroma, CN: cystic nephroma, MRTK: malignant rhabdoid tumor of the kidney. https://doi.org/10.1371/journal.pone.0261729.g001
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Around 6% of all childhood malignancies represent renal tumors, of which a majority includes Wilms tumor (WT). Although survival rates have improved over the last decades, specific patients are still at risk for adverse outcome. In the Netherlands, since 2015, pediatric oncology care for renal tumors has been centralized in the Princess Máxima Cent...
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Context 1
... median age at presentation of the remaining 163 patients was 35 months (range: 0-226 months) ( Table 1). The distribution of renal tumor subtypes (WT/CPDN, nephrogenic rests, CCSK, MRTK, CN, MN, RCC, angiomyolipoma and others) are listed in Table 1 and Fig 1. Sixty-four (39%) patients presented with a prominent abdominal mass and 48 (29%) with abdominal pain. Asymptomatic presentation was recorded in 29 (15%) patients. ...
Citations
... 10,11 However, due to the variety of cancers that occur and the diversity of patient needs in this generation, 10-12 the status of centralized cancer care could vary according to the type of cancer and age group. 13,14 Despite this, there is a lack of data showing type of treatment hospital, such as how many children and AYAs receive cancer treatment at the designated cancer hospitals (PCHs or DCCHs) across the country. ...
Cancer in children, adolescents, and young adults (AYAs) although rare, is the leading disease-specific cause of death in Japan. This study aims to investigate cancer incidence and type of treatment hospital among children and AYAs in Japan. Cancer incidence data (2016-2018) for those aged 0-39 years were obtained from the Japanese population-based National Cancer Registry. Cancer types were classified according to the 2017 update of the International Classification of Childhood Cancer (Third Edition), and AYA Site Recode 2020 Revision. Cases were also categorized into three groups: those treated at core hospitals for pediatric cancer treatment (pediatric cancer hospitals [PCHs]), those treated at designated cancer care hospitals, and those treated at nondesignated hospitals. The age-standardized incidence rate was 166.6 (per million-person years) for children (age 0-14 years) and 579.0 for AYAs (age 15-39 years) (including all cancers and benign or uncertain-behavior central nervous system [CNS] tumors). The type of cancer varied with age: hematological malignancies, blastomas, and CNS tumors were common in children under 10 years, malignant bone tumors and soft tissue sarcomas were relatively common in teenagers, and in young adults over 20 years, carcinomas in thyroid, testis, gastrointestinal, female cervix, and breast were common. The proportion of cases treated at PCHs ranged from 20% to 30% for children, 10% or less for AYAs, and differed according to age group and cancer type. Based on this information, the optimal system of cancer care should be discussed.
... This study was conducted on 105 patients from a national cohort of WT in the Netherlands [23]. All children that were diagnosed between 2014 and 2019, younger than 18 years, Center, and histopathology findings were based on conventional pathology according to SIOP classification [24]. ...
Simple Summary
Wilms tumor (WT) is the most frequent pediatric tumor in children and shows highly variable histology, leading to variation in classification. Artificial intelligence-based automatic recognition holds the promise that this may be done in a more consistent way than human observers can. We have therefore studied digital microscopic slides, stained with standard hematoxylin and eosin, of 72 WT patients and used a deep learning (DL) system for the recognition of 15 different normal and tumor components. We show that such DL system can do this task with high accuracy, as exemplified by a Dice score of 0.85 for the 15 components. This approach may allow future automated WT classification.
Abstract
(1) Background: Histopathological assessment of Wilms tumors (WT) is crucial for risk group classification to guide postoperative stratification in chemotherapy pre-treated WT cases. However, due to the heterogeneous nature of the tumor, significant interobserver variation between pathologists in WT diagnosis has been observed, potentially leading to misclassification and suboptimal treatment. We investigated whether artificial intelligence (AI) can contribute to accurate and reproducible histopathological assessment of WT through recognition of individual histopathological tumor components. (2) Methods: We assessed the performance of a deep learning-based AI system in quantifying WT components in hematoxylin and eosin-stained slides by calculating the Sørensen–Dice coefficient for fifteen predefined renal tissue components, including six tumor-related components. We trained the AI system using multiclass annotations from 72 whole-slide images of patients diagnosed with WT. (3) Results: The overall Dice coefficient for all fifteen tissue components was 0.85 and for the six tumor-related components was 0.79. Tumor segmentation worked best to reliably identify necrosis (Dice coefficient 0.98) and blastema (Dice coefficient 0.82). (4) Conclusions: Accurate histopathological classification of WT may be feasible using a digital pathology-based AI system in a national cohort of WT patients.
... Wilms tumor is the most prevalent malignant kidney cancer in children. 1,2 According to the Renal Tumour Study Group of the International Society of Paediatric Oncology (SIOP-RTSG), 2 most of these patients are indicated for chemotherapy before surgery, i.e., neoadjuvant chemotherapy. Volume measurements on magnetic resonance imaging (MRI) are used throughout the treatment, for example in the choice for nephron sparing surgery. 2 Clinically, this is done measuring the diameters of the tumor in three directions, which is an estimate of the true tumor volume. ...
... Wilms tumor is one of the most prevalent malignant solid tumors in pediatric patients. With a combination of neoadjuvant chemotherapy, surgery, and post-operative chemotherapy survival up to 90% can be achieved [1,2]. Throughout the treatment process, quantifying tumor volume on magnetic resonance imaging (MRI) or computed tomography (CT) is important for several reasons. ...
... First, we had a limited sample size. This is, however, the largest unselected national MRI cohort to date including all pediatric Wilms tumor patients in the Netherlands in the period of 2018-2020 [2,24]. Second, although we divided the tumors over three observers for segmentation, the population of tumors and the techniques are derived from a single-institution study. ...
Simple Summary
Volume measurements are important in tumor evaluations of children with a Wilms tumor. Current volume measurements might not be accurate. Our study had two aims. Our first aim was to assess whether manual segmentation of MRI can accurately quantify the volume of Wilms tumors. Our second aim was to show if manual segmentation can be automated using deep learning. We compared radiological-based and manual segmentation-based measurements. Next, we developed an automated segmentation method. Radiological measurements underestimate the actual tumor volume by about 10% irrespective of tumor size. Deep learning can potentially be used to replace manual segmentations in volume measurements.
Abstract
Wilms tumor is a common pediatric solid tumor. To evaluate tumor response to chemotherapy and decide whether nephron-sparing surgery is possible, tumor volume measurements based on magnetic resonance imaging (MRI) are important. Currently, radiological volume measurements are based on measuring tumor dimensions in three directions. Manual segmentation-based volume measurements might be more accurate, but this process is time-consuming and user-dependent. The aim of this study was to investigate whether manual segmentation-based volume measurements are more accurate and to explore whether these segmentations can be automated using deep learning. We included the MRI images of 45 Wilms tumor patients (age 0–18 years). First, we compared radiological tumor volumes with manual segmentation-based tumor volume measurements. Next, we created an automated segmentation method by training a nnU-Net in a five-fold cross-validation. Segmentation quality was validated by comparing the automated segmentation with the manually created ground truth segmentations, using Dice scores and the 95th percentile of the Hausdorff distances (HD95). On average, manual tumor segmentations result in larger tumor volumes. For automated segmentation, the median dice was 0.90. The median HD95 was 7.2 mm. We showed that radiological volume measurements underestimated tumor volume by about 10% when compared to manual segmentation-based volume measurements. Deep learning can potentially be used to replace manual segmentation to benefit from accurate volume measurements without time and observer constraints.
... Pediatric renal cell carcinoma (RCC) is a rare renal malignancy [1,2]. Although Wilms tumors (WTs) show the highest prevalence in young children, the incidence of RCC increases in the second decade of life [1,3,4]. Whereas in the Renal Tumor Study Group of the International Society of Pediatric Oncology (SIOP-RTSG), pre-operative chemotherapy is the standard of care for WTs, upfront surgery is recommended for localized RCC [5]. ...
Simple Summary
Whereas renal cell carcinoma (RCC) is the most common renal tumor in adults, pediatric RCC is a rare malignancy. The previous literature focusing on cross-sectional imaging of RCC concerns mainly computed tomography in adults, whereas in children, a different distribution of subtypes is seen, as well as a preference for magnetic resonance imaging (MRI). Therefore, the aim of this study was to identify MRI characteristics of pediatric and young-adult RCC through a case series and literature review focusing on translocation-type RCC (MiT-RCC) and the pediatric and young-adult population. In our review as well as in our case series T2-weighted hypo-intensity seems to be a potential discriminative characteristic. Moreover, an irregular growth pattern and limited diffusion restriction were often described. Nevertheless, we conclude the discrimination of RCC subtypes, and especially the differentiation of RCC from other pediatric renal tumors, remains difficult.
Abstract
Pediatric renal cell carcinoma (RCC) is a rare malignancy. Magnetic resonance imaging (MRI) is the preferred imaging modality for assessment of these tumors. The previous literature has suggested that cross-sectional-imaging findings differ between RCC and other pediatric renal tumors and between RCC subtypes. However, studies focusing on MRI characteristics are limited. Therefore, this study aims to identify MRI characteristics of pediatric and young-adult RCC, through a single-center case series and literature review. Six identified diagnostic MRI scans were retrospectively assessed, and an extensive literature review was conducted. The included patients had a median age of 12 years (63–193 months). Among other subtypes, 2/6 (33%) were translocation-type RCC (MiT-RCC) and 2/6 (33%) were clear-cell RCC. Median tumor volume was 393 cm³ (29–2191 cm³). Five tumors had a hypo-intense appearance on T2-weighted imaging, whereas 4/6 were iso-intense on T1-weighted imaging. Four/six tumors showed well-defined margins. The median apparent diffusion coefficient (ADC) values ranged from 0.70 to 1.20 × 10⁻³ mm²/s. In thirteen identified articles focusing on MRI characteristics of MiT-RCC, the majority of the patients also showed T2-weighted hypo-intensity. T1-weighted hyper-intensity, irregular growth pattern and limited diffusion–restriction were also often described. Discrimination of RCC subtypes and differentiation from other pediatric renal tumors based on MRI remains difficult. Nevertheless, T2-weighted hypo-intensity of the tumor seems a potential distinctive characteristic.
... CPDN has no specific clinical characteristics, and most patients show only a painless abdominal mass found unintentionally or a renal tumor found by abdominal ultrasound or CT (8). In the statistical literature, abdominal mass accounted for 87.1% (54/62) of cases. ...
Cases of patients with cystic partially differentiated nephroblastoma (CPDN) have been reported to date, which presented as polycystic renal tumor in all of them. It is a special pathological type of nephroblastoma. Here, we report the first case of a unicystic CPDN in a child. The patient was diagnosed with a simple renal cyst and underwent laparoscopic decortication. The naive nephron was found in the pathological section, and the diagnosis of CPDN was confirmed. The patient then underwent a radical nephrectomy and six cycles of postoperative chemotherapy. There was no recurrence or metastasis after 2 years of follow-up. Pediatric CPDN presenting as a unicystic renal tumor poses a new challenge to the diagnosis, differential diagnosis, and treatment of unicystic renal tumor.
... The second, a three-year-old girl, died during postoperative treatment for lung and bone metastasized WT, local stage III. She died from increased intracranial pressure due to a concomitant leptomeningeal rhabdomyosarcoma, which was identified only during treatment as a secondary malignancy [24]. ...
Simple Summary
Survival of children with Wilms tumor is excellent. However, treatment-related complications may occur, requiring treatment at the pediatric intensive care unit (PICU). The aim of our retrospective study was to assess the frequency, clinical characteristics, and outcome of 175 children with Wilms tumor requiring treatment at the PICU in the Netherlands. Thirty-three patients (almost 20%) required unplanned PICU admission during their disease course. Younger age at diagnosis, intensive chemotherapy regimens, and bilateral tumor surgery were risk factors for these unplanned PICU admissions. Three children required renal replacement therapy, two of which continued dialysis after PICU discharge. Two children died during their PICU stay. During follow up, hypertension and renal dysfunction were frequently observed, which justifies special attention for kidney function and blood pressure monitoring during and after treatment of these children.
Abstract
Survival rates are excellent for children with Wilms tumor (WT), yet tumor and treatment-related complications may require pediatric intensive care unit (PICU) admission. We assessed the frequency, clinical characteristics, and outcome of children with WT requiring PICU admissions in a multicenter, retrospective study in the Netherlands. Admission reasons of unplanned PICU admissions were described in relation to treatment phase. Unplanned PICU admissions were compared to a control group of no or planned PICU admissions, with regard to patient characteristics and short and long term outcomes. In a multicenter cohort of 175 children with an underlying WT, 50 unplanned PICU admissions were registered in 33 patients. Reasons for admission were diverse and varied per treatment phase. Younger age at diagnosis, intensive chemotherapy regimens, and bilateral tumor surgery were observed in children with unplanned PICU admission versus the other WT patients. Three children required renal replacement therapy, two of which continued dialysis after PICU discharge (both with bilateral disease). Two children died during their PICU stay. During follow-up, hypertension and chronic kidney disease (18.2 vs. 4.2% and 15.2 vs. 0.7%) were more frequently observed in unplanned PICU admitted patients compared to the other patients. No significant differences in cardiac morbidity, relapse, or progression were observed. Almost 20% of children with WT required unplanned PICU admission, with young age and treatment intensity as potential risk factors. Hypertension and renal impairment were frequently observed in these patients, warranting special attention at presentation and during treatment and follow-up.
... Interestingly, centralization does seemingly not only improve outcome but can also influence the epidemiological landscape: in the Netherlands, renal tumors have been centralized from 2015 and first results, published in 2022, showed that centralization of care for children with renal tumors led to referral of more than expected cases. Further and very promising, national centralization led to enhanced development of molecular diagnostics and other innovation-based treatments for the future [19]. In adults, studies addressing the effect of centralization are much more numerous and conclusive. ...
Background:
Pediatric liver surgery is complex, and complications are not uncommon. Centralization of highly specialized surgery has been shown to improve quality of care. In 2012, pediatric liver surgery was centralized in Switzerland in one national center. This study analyses results before and after centralization.
Methods:
Retrospective monocentric comparative study. Analysis of medical records of children (0-16 years) operated for any liver tumor between 1 January 2001 and 31 December 2020. Forty-one patients were included: 14 before centralization (before 1 January 2012) and 27 after centralization (after 1 January 2012). Epidemiological, pre-, intra-, and post-operative data were collected. Fischer's exact and t-test were used to compare groups.
Results:
The two cohorts were homogeneous. Operating time was reduced, although not significantly, from 366 to 277 min. Length of postoperative stay and mortality were not statistically different between groups. Yet, after centralization, overall postoperative complication rate decreased significantly from 57% to 15% (p = 0.01), Clavien > III complications decreased from 50% to 7% (p < 0.01), and hepatic recurrences were also significantly reduced (40% to 5%, p = 0.03).
Conclusion:
Centralization of the surgical management of liver tumors in Switzerland has improved quality of care in our center by significantly reducing postoperative complications and hepatic recurrence.
Background
Radiologic volumetric evaluation of Wilms’ tumor (WT) is an important indicator to guide treatment decisions. However, due to the heterogeneity of the tumors, radiologists have main-guard differences in diagnosis that can lead to misdiagnosis and poor treatment. The aim of this study was to explore whether CT-based outlining of WT foci can be automated using deep learning.
Methods
We included CT intravenous phase images of 105 patients with WT and double-blind outlining of lesions by two radiologists. Then, we trained an automatic segmentation model using nnUnet. The Dice similarity coefficient (DSC) and 95th percentile Hausdorff distance (HD95) were used to assess the performance. Next, we optimized the automatic segmentation results based on the ratio of the three-dimensional diameter of the lesion to improve the performance of volumetric assessment.
Results
The DSC and HD95 was 0.83 ± 0.22 and 10.50 ± 8.98 mm. The absolute difference and percentage difference in tumor size was 72.27 ± 134.84 cm³ and 21.08% ± 30.46%. After optimization according to our method, it decreased to 40.22 ± 96.06 cm³ and 10.16% ± 9.70%.
Conclusion
We introduce a novel method that enhances the accuracy of predicting WT volume by integrating AI automated outlining and 3D tumor diameters. This approach surpasses the accuracy of using AI outcomes alone and has the potential to enhance the clinical evaluation of pediatric patients with WT. By intertwining AI outcomes with clinical data, this method becomes more interpretive and offers promising applications beyond Wilms tumor, extending to other pediatric diseases.
About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions.
A retrospective study of pediatric Wilms tumors treated at three sites (January 1999–June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed.
Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd–Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5–20 years), overall survival was 89% and event-free survival was 78%.
Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.
