Progress Update in Pediatric Renal Tumors

Jain, Juhi; Sutton, Kathryn S.; Hong, Andrew L.

doi:10.1007/s11912-021-01016-y

Cited by 20 publications

(18 citation statements)

References 98 publications

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“…Survival rates of pediatric renal tumors have significantly improved [10][11][12]. The overall survival (OS) rate for children with localized WT is currently greater than 90%, whereas lower survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC, and relapsed WT [1].…”

Section: A C C E P T E D a R T I C L E Korean Cancer Associationmentioning

confidence: 99%

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data

Koh¹,

Han²,

Choi³

et al. 2023

Cancer Res Treat

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Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors.We investigated the epidemiology of pediatric renal tumors in Korea. Materials and MethodsFrom January 2001 to December 2015, data of pediatric patients (0-18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. ResultsAmong 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). ConclusionThe pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

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Section: A C C E P T E D a R T I C L E Korean Cancer Associationmentioning

confidence: 99%

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data

Koh¹,

Han²,

Choi³

et al. 2023

Cancer Res Treat

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“…Genetic aberrations in Wilms tumors discussed as potential targets for molecular interventions include WT1, CTNNB1, WTX, TP53, FBXW7, MYCN, SIX 1/2, DICER1, DROSHA, DGCR8, and IGF2, but preclinical data has become available only recently [ 431 ]. Promising target structures are antiangiogenic compounds, inhibitors to aurora-A-kinase, the mTOR pathway, c-Met, JAK2, cell cycle, telomerase, HER2, ATR, and in particular the WNT signaling pathway which is mutated in 35% of Wilms tumors as previously mentioned [ 432 ]. Recent clinical trials showed durable responses for the combination of TRK inhibitors and monoclonal antibodies directed against PD-1 (nivolumab, pembrolizumab) for advanced treatment-refractory renal cell carcinoma which might be implemented into therapeutic strategies for Wilms tumors [ 419 , 420 ].…”

Section: Childhood Cancer and Second Primary Malignanciesmentioning

confidence: 99%

Childhood Cancer: Occurrence, Treatment and Risk of Second Primary Malignancies

Zahnreich

Schmidberger

2021

Cancers

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Cancer represents the leading cause of disease-related death and treatment-associated morbidity in children with an increasing trend in recent decades worldwide. Nevertheless, the 5-year survival of childhood cancer patients has been raised impressively to more than 80% during the past decades, primarily attributed to improved diagnostic technologies and multiagent cytotoxic regimens. This strong benefit of more efficient tumor control and prolonged survival is compromised by an increased risk of adverse and fatal late sequelae. Long-term survivors of pediatric tumors are at the utmost risk for non-carcinogenic late effects such as cardiomyopathies, neurotoxicity, or pneumopathies, as well as the development of secondary primary malignancies as the most detrimental consequence of genotoxic chemo- and radiotherapy. Promising approaches to reducing the risk of adverse late effects in childhood cancer survivors include high precision irradiation techniques like proton radiotherapy or non-genotoxic targeted therapies and immune-based treatments. However, to date, these therapies are rarely used to treat pediatric cancer patients and survival rates, as well as incidences of late effects, have changed little over the past two decades in this population. Here we provide an overview of the epidemiology and etiology of childhood cancers, current developments for their treatment, and therapy-related adverse late health consequences with a special focus on second primary malignancies.

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“…In general, this group of cancers has a 5-year overall survival rate of approximately 70% [2,3]. Pediatric renal tumors include Wilms tumor (~80%) [3], renal cell carcinoma (RCC, 2-6%) [4], clearcell sarcoma of the kidney (CCSK, 2-4%) [5], congenital mesoblastic nephroma (4%) [6], malignant rhabdoid tumor (MRT, 1.5-4%) [7], and other less common cancers such as cystic nephroma and metanephric tumors (~2%) [8]. RCC has a global incidence of about 4% [9], with a 5-year overall survival rate of approximately 78.8% [10].…”

Section: Introductionmentioning

confidence: 99%

Recent Advances in Renal Medullary Carcinoma

Hong

2022

IJMS

Self Cite

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Renal medullary carcinoma (RMC) is a rare renal malignancy that has been associated with sickle hemoglobinopathies. RMC is aggressive, difficult to treat, and occurs primarily in adolescents and young adults of African ancestry. This cancer is driven by the loss of SMARCB1, a tumor suppressor seen in a number of primarily rare childhood cancers (e.g., rhabdoid tumor of the kidney and atypical teratoid rhabdoid tumor). Treatment options remain limited due in part to the limited knowledge of RMC biology. However, significant advances have been made in unraveling the biology of RMC, from genomics to therapeutic targets, over the past 5 years. In this review, we will present these advances and discuss what new questions exist in the field.

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Progress Update in Pediatric Renal Tumors

Cited by 20 publications

References 98 publications

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data

Childhood Cancer: Occurrence, Treatment and Risk of Second Primary Malignancies

Recent Advances in Renal Medullary Carcinoma

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