Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution

Qureshi, Sajid; Bhagat, Monica; Verma, Kamlesh; Yadav, Subhash; Prasad, Maya; Vora, Tushar; Chinnaswamy, Girish; Amin, Nayana; Smriti, Vasundhara; Baheti, Akshay D.; Laskar, Siddharth; Khanna, Nehal; Ramadwar, Mukta; Shah, Sneha

doi:10.1016/j.jpurol.2020.05.168

Cited by 14 publications

(17 citation statements)

References 24 publications

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“…When we compare our registration data with the national cancer registry in The Netherlands (NCR database), it demonstrates that we indeed managed to attract all but 1 Dutch patients with renal tumors that are considered 'malignant' (WT/CPDN, CCSK, MRTK, RCC) until the age of 19 years from the very first moment. In total, 37 (22%) patients were not WT/CPDN/nephroblastomatosis/nephrogenic rest (non-WT), which is higher than non-WT percentages in reported series [45,46]. This illustrates that centralization of care leads to a more complete epidemiological picture of renal tumor subtypes.…”

Section: Discussionmentioning

confidence: 75%

Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

et al. 2022

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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 Center for Pediatric Oncology. Here, we describe experiences of the first 5 years of centralized care and explore whether this influences the epidemiological landscape by comparing data with the Netherlands Cancer Registry (NCR). We identified all patients <19 years with a renal mass diagnosed between 01-01-2015 and 31-12-2019 in the Princess Máxima Center. Epidemiology, characteristics and management were analyzed. We identified 164 patients (including 1 patient who refused consent for registration), in our center with a suspicion of a renal tumor. The remaining 163 cases included WT (n = 118)/cystic partially differentiated nephroblastoma (n = 2)/nephrogenic rests only (n = 6) and non-WT (n = 37). In this period, the NCR included 138 children, 1 17-year-old patient was not referred to the Princess Máxima Center. Central radiology review (before starting treatment) was performed in 121/163 patients, and central pathology review in 148/152 patients that underwent surgery. Treatment stratification, according to SIOP/EpSSG protocols was pursued based on multidisciplinary consensus. Preoperative chemotherapy was administered in 133 patients, whereas 19 patients underwent upfront surgery. Surgery was performed in 152 patients, and from 133 biomaterial was stored. Centralization of care for children with renal tumors led to referral of all but 1 new renal tumor cases in the Netherlands, and leads to referral of very rare subtypes not registered in the NCR, that benefit from high quality diagnostics and multidisciplinary decision making. National centralization of care led to enhanced development of molecular diagnostics and other innovation-based treatments for the future.

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Section: Discussionmentioning

confidence: 75%

Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

et al. 2022

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“…1 Data are limited from LMIC settings, but at one Indian center, the translocation subtype was responsible for 70% of RCC cases. 2 This has been associated with a worse prognosis. 52 Patients with localized, resectable disease had similar positive outcomes and those with metastatic disease had similarly poor outcomes to patients in HICs.…”

Section: Renal Cell Carcinomamentioning

confidence: 99%

Pediatric renal tumor epidemiology: Global perspectives, progress, and challenges

Libes

Hol

Neto

et al. 2022

Pediatric Blood & Cancer

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Pediatric renal tumors account for 3%-11% of childhood cancers, the most common of which is Wilms tumor or nephroblastoma. Epidemiology plays a key role in cancer prevention and control by describing the distribution of cancer and discovering risk factors for cancer. Large pediatric research consortium trials have led to a clearer understanding of pediatric renal tumors, identification of risk factors, and development of more risk-adapted therapies. These therapies have improved event-free and overall survival for children. However, several challenges remain and not all children have benefited from the improved outcomes. In this article, we review the global epidemiology of pediatric renal tumors, including key consortium and global studies. We identify current knowledge gaps and challenges facing both high and low middle-incomes countries.

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“…Malignant non-Wilms tumors (NWTs) in children include clear cell sarcoma of the kidney (CCSK), malignant rhabdomyoma tumor of the kidney (MRTK), renal cell carcinoma (RCC) and other extremely rare malignant tumors [ 1 – 3 ]. Preoperative identification of the tumor type relies primarily on imaging or diagnostic needle biopsy.…”

Section: Introductionmentioning

confidence: 99%

“…CT remains the imaging modality of choice, and low-dose scanning methods do not increase radiation doses to children. Because the pathology, treatment and prognosis of malignant NWTs are distinctly different [ 3 , 6 – 10 ], it is necessary to conduct CT imaging examinations before surgery to determine differential diagnoses. The purpose of this study was to review the clinical and imaging features of malignant NWTs and features of tumor metastasis.…”

Section: Introductionmentioning

confidence: 99%

Imaging features and differences among the three primary malignant non-Wilms tumors in children

Zhu

Huang

et al. 2021

BMC Med Imaging

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Background The pathology, treatment and prognosis of malignant non-Wilms tumors (NWTs) are different, so it is necessary to differentiate these types of tumors. The purpose of this study was to review the clinical and imaging features of malignant NWTs and features of tumor metastasis. Methods We retrospectively analyzed the CT images of 65 pediatric patients with NWTs from March 2008 to July 2020, mainly including clear cell sarcoma of the kidney (CCSK), malignant rhabdomyoma tumor of the kidney (MRTK) and renal cell carcinoma (RCC). Available pretreatment contrast-enhanced abdominal CT examinations were reviewed. The clinical features of the patients, imaging findings of the primary mass, and locoregional metastasis patterns were evaluated in correlation with pathological and surgical findings. Results The study included CCSK (22 cases), MRTK (27 cases) and RCC (16 cases). There were no significant differences observed among the sex ratios of CCSK, MRTK and RCC (all P > 0.05). Among the three tumors, the onset age of MRTK patients was the smallest, while that of RCC patients was the largest (all P < 0.05). The tumor diameter of CCSK was larger than that of MRTK and RCC (all P < 0.001). For hemorrhage and necrosis, the proportion of MRTK patients was larger than that of the other two tumors (P = 0.017). For calcification in tumors, the proportion of calcification in RCC was highest (P = 0.009). Only MRTK showed subcapsular fluid (P < 0.001). In the arterial phase, the proportion of slight enhancement in RCC was lower than that in the other two tumors (P = 0.007), and the proportion of marked enhancement was the highest (P = 0.002). In the venous phase, the proportion of slight enhancement in RCC was lower than that in the other two tumors (P < 0.001). Only CCSK had bone metastasis. There was no liver and lung metastasis in RCC. Conclusions NWTs have their own imaging and clinical manifestations. CCSK can cause vertebral metastasis, MRTK can cause subcapsular effusion, and RCC tumor density is usually high and calcification. These diagnostic points can play a role in clinical diagnosis.

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Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution

Cited by 14 publications

References 24 publications

Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

Pediatric renal tumor epidemiology: Global perspectives, progress, and challenges

Imaging features and differences among the three primary malignant non-Wilms tumors in children

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