scholarly journals Renal tumors: Age spectrum and histological distribution across childhood

2020 ◽  
Author(s):  
Tingting Liu ◽  
Lika’a Fasih Y. Al-Kzayer ◽  
Yozo Nakazawa ◽  
lei chen
Keyword(s):  
Early Childhood ◽  
Age Of Onset ◽  
Wilms Tumor ◽  
Malignant Tumors ◽  
Age Distribution ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Benign Tumors ◽  
Congenital Mesoblastic Nephroma ◽  
The Relationship

Abstract Background: The objective of this study was to identify the relationship between the histopathological distribution of renal tumors and the age parameter across childhood period and to compare that with the available literature. Methods: A total of 193 pediatric patients with renal tumors were classified into 5 groups as embryo, infant, early childhood, childhood, and adolescent. Age distribution, pathological types and clinical characteristics were summarized. Results: Among the 193 patients, 95.8% presented with malignant tumors, and 4.2% showed benign tumors. The prevalence of Wilms’ tumor (WT( and non-WT, were 63.7% and 36.3%, respectively. Non-WT patients included clear cell sarcoma of the kidney (CCSK), followed by rhabdoid tumor of the kidney (RTK), congenital mesoblastic nephroma (CMN), cystic partially differentiated nephroblastoma (CPDN), and renal cell carcinoma (RCC). Early childhood (89/193, 46.2%) was the most common period of onset, followed by infant (73/193, 37.9%), childhood (19/193, 9.8%), embryo (8/193, 4.1%) and adolescent (4/193, 2.0%). WT predominantly occurred in infant and early childhood and decreased among older age group. Non-WT occurred various period of onset and rather common during embryo. 10.9% of patients were asymptomatic at the time of diagnosis and that was significantly observed in non-WT (P=0.04). Conclusions: Age of onset is a highly significant factor for pediatric renal tumors. Relationship between age spectrum and pathological characteristics influences distinguishing the histopathological distribution of WT and non-WT.

scholarly journals Renal tumors: Age spectrum and histological distribution across childhood

2020 ◽  
Author(s):  
Tingting Liu ◽  
Lika’a Fasih Y. Al-Kzayer ◽  
Yozo Nakazawa ◽  
lei chen
Keyword(s):  
Early Childhood ◽  
Age Of Onset ◽  
Wilms Tumor ◽  
Malignant Tumors ◽  
Age Distribution ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Benign Tumors ◽  
Congenital Mesoblastic Nephroma ◽  
The Relationship

Abstract BackgroundThe objective of this study was to identify the relationship between the histopathological distribution of renal tumors and the age parameter across childhood period and to compare that with the available literature.MethodsA total of 193 pediatric patients with renal tumors were classified into 5 groups as embryo, infant, early childhood, childhood, and adolescent. Age distribution, pathological types and clinical characteristics were summarized. ResultsAmong the 193 patients, 95.8% presented with malignant tumors, and 4.2% showed benign tumors. The prevalence of Wilms’ tumor (WT( and non-WT, were 63.7% and 36.3%, respectively. Non-WT patients included clear cell sarcoma of the kidney (CCSK), followed by rhabdoid tumor of the kidney (RTK), congenital mesoblastic nephroma (CMN), cystic partially differentiated nephroblastoma (CPDN), and renal cell carcinoma (RCC). Early childhood (89/193, 46.2%) was the most common period of onset, followed by infant (73/193, 37.9%), childhood (19/193, 9.8%), embryo (8/193, 4.1%) and adolescent (4/193, 2.0%). WT predominantly occurred in infant and early childhood and decreased among older age group. Non-WT occurred various period of onset and rather common during embryo. 10.9% of patients were asymptomatic at the time of diagnosis and that was significantly observed in non-WT (P=0.04). ConclusionsAge of onset is a highly significant factor for pediatric renal tumors. Relationship between age spectrum and pathological characteristics influences distinguishing the histopathological distribution of WT and non-WT.

Functional and Gene Expression Analysis of the p53 Signaling Pathway in Clear Cell Sarcoma of the Kidney and Congenital Mesoblastic Nephroma

2002 ◽  
Vol 5 (3) ◽  
pp. 257-268 ◽  
Author(s):  
Noel A. Brownlee ◽  
Debra J. Hazen-Martin ◽  
A. Julian Garvin ◽  
Gian G. Re
Keyword(s):  
Tumor Tissue ◽  
Clear Cell ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Mesoblastic Nephroma ◽  
Primary Tumors ◽  
Cell Sarcoma ◽  
Congenital Mesoblastic Nephroma ◽  
Mdr 1

Mutation of p53 has been implicated in progression of classical Wilms tumor (WT) into the anaplastic variant (AWT), drug resistance, and poor prognosis. Because of prognostic similarities, clear cell sarcoma of the kidney (CCSK) has been classified with AWT and other aggressive pediatric renal tumors, apart from congenital mesoblastic nephroma (CMN), which is instead a relatively benign tumor of neonates. Initially, CCSK and CMN were assumed to be ontologically related, but the role of p53 in the pathogenesis of either disease has not been sufficiently evaluated as in AWT. We examined the status of p53 in CMN and CCSK in comparison to AWT by immunohistochemistry and mRNA analysis of p53, the downstream effector p21 WAF-1/CIP-1 ( p21), the multidrug resistance gene MDR-1, a putative target of p53, and the p53-antagonist Mdm-2. Surprisingly, strong p53 nuclear immunoreactivity was found in cultures from two CMN specimens, but not in frozen or fixed tumor tissue from five other CMN specimens, nor in cell lines or tumor tissue from CCSK. Sequence analysis excluded p53 mutations. The size of the p53 mRNA in CMN and CCSK primary tumors excluded gross deletions or rearrangements. Low levels of Mdm-2 mRNA in CCSK and CMN primary tumors and cultures did not support a role for Mdm-2. Absence of MDR-1 mRNA excluded MDR-1 in the drug-resistant phenotype of CCSK. Cisplatin-induced p21 transactivation assays and G1 cell cycle arrest analyses showed that p21 transactivation and G1 arrest occurred in both CCSK and CMN cultures, demonstrating integrity of the p53 signal transduction pathway. Absence of p53 functional abnormalities excluded relationships between CCSK and CMN as in AWT, supporting the association of cellular CMN with congenital fibrosarcomas as more recently proposed.

Tubular Entrapment May Be Inconspicuous in Clear Cell Sarcoma of the Kidney in Early Infancy, Compared to Childhood: An In-Depth Case Comparison

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S59-S60
Author(s):  
Krutika Patel ◽  
Sara Avalos Hernandez ◽  
S Shawn Liu ◽  
J Elliot Carter ◽  
Elizabeth Manci
Keyword(s):  
Growth Pattern ◽  
Clear Cell ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Study Groups ◽  
Growth Patterns ◽  
Molecular Techniques ◽  
Early Infancy ◽  
Renal Tumors ◽  
Cell Sarcoma

Abstract Introduction Clear cell sarcoma of kidney (CCSK) is a rare malignancy accounting for <0.5% of all primary renal tumors, commonly diagnosed between 2 and 4 years of age and rarely occurring in early infancy. The challenging differentiation between CCSK and blastemal Wilms tumor is important because of the distinct clinical pattern of CCSK to recur and metastasize to bone and brain. The aim of this study is to discern subtle features that could assist pathologists in diagnosing CCSK in infancy. Method In-depth comparison of clinical, histological, and immunohistochemical findings in a case of CCSK diagnosed at 5 months of age with two cases of CCSK diagnosed at 2 and 3 years of age. Results Both groups were male, and each presented with an abdominal mass. Grossly, a single, firm, well-demarcated tumor, morphologically comprising monotonous small primitive round-to-polygonal/spindle cells, was seen in both groups. The major differences between the study groups were growth patterns and stromal reactions. In infancy, the growth pattern was diffusely uniform sheets of malignant cells with no entrapment of tubules and inconspicuous stromal changes. However, in childhood cases, the growth pattern included well-defined tubular entrapment, as well as focal microcyst formation, myxomatous stroma, palisading bodies, and anaplastic and/or rhabdoid histology. In both study groups, the immunohistochemistry showed strong immunoreactivity with cyclin D1 and nonspecific positivity for vimentin, CD99, and BAF47. Conclusion CCSK has notoriously diverse histological heterogeneity and mimics other pediatric renal tumors, making diagnosis treacherous, and commonly erroneous as Wilms tumor with unfavorable histology. Despite the advent of immunohistochemical and molecular techniques, a thorough morphologic analysis remains key in accurately diagnosing CCSK at any age, especially in early infancy. This small in-depth comparison of CCSK by age groups suggests that tubular entrapment and stromal changes may be less conspicuous in CCSK in early infancy than at older ages.

Incidence of benign versus malignant renal tumors in selected studies.

2012 ◽  
Vol 30 (5_suppl) ◽  
pp. 357-357 ◽  
Author(s):  
Paul Russo ◽  
Robert G. Uzzo ◽  
William Thomas Lowrance ◽  
Aviva Asnis-Alibozek ◽  
Norman David LaFrance ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Malignant Tumors ◽  
Early Stage ◽  
Renal Tumors ◽  
Benign Tumors ◽  
Stage Migration ◽  
Renal Masses ◽  
Definitive Method

357 Background: Use of cross-sectional imaging has increased the detection rate for small renal tumors; more patients now present with early-stage renal cell carcinoma (RCC) or benign or indolent renal masses. Histopathology after surgical resection is the definitive method for characterizing renal tumors. Stage migration of renal masses creates uncertainty about the percentage of resected masses that will be benign vs malignant. We sought to better define these proportions through a targeted review of the literature. Methods: PubMed/select congresses were searched to identify the histologic classification of renal masses in a representative sample from the contemporary literature: [search] incidence AND (renal cell carcinoma AND benign); incidence AND (renal tumor AND benign); percentage AND (renal cell carcinoma AND benign); limit: 2003–2011. Results: Most representative studies included procedures conducted in the mid-1990s to the mid-to-late 2000s. Studies origin was US (n=8), Korea (n=3), China, Japan, Germany, Austria, Australia, and multisite (Israel/France/US; all n=1). Only 8 studies had n≥500 (range, 70–10,404). The proportion of benign masses are shown (see Table); half of the studies reported values between 16% and 17%. The majority found that benign tumors were more likely to be smaller in size (<4 or <7 cm) than malignant tumors. 11 studies reported the RCC subtype (% clear cell range, 46%–83%). Conclusions: Benign renal tumors occur ~15% of the time and are more prevalent among small masses. Nearly 25% of resected lesions are benign or indolent and may not require surgery. Preoperative discrimination of more aggressive renal masses would be an important clinical advance that could improve clinicians’ diagnostic confidence and guide patient management. Funding: Wilex AG/IBA Molecular. [Table: see text]

scholarly journals HISTOPATHOLOGICAL SPECTRUM OF NEOPLASTIC LESIONS OF KIDNEY- A THREE YEARS STUDY

2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Saket Sarswat ◽  
Vimlesh . ◽  
D.P. Soni
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Wilms Tumor ◽  
Malignant Tumors ◽  
Pathological Process ◽  
Benign Tumors ◽  
Older Individuals ◽  
Neoplastic Lesions ◽  
Paediatric Age

Background: Kidney can be involved in various pathological process. Both benign & malignant tumors can occur in the kidney. They arise from different components of renal parenchyma, notably tubular epithelium.1 99 percent of renal neoplasms are malignant, with renal cell carcinoma and wilm’s tumor being the most common2. Men have higher incidence than women (approximately 1.6:1) and vast majority are diagnosed after 65 years of age. Material and methods: Prospective and retrospective study from January 2017 to December 2019 in the Department of Pathology. Results: The study comprised of 67 cases of neoplastic conditions, out of which 63 cases are malignant and only 4 cases were recorded as benign. Conclusion: Malignant tumors far more common than benign lesions. In adults and older individuals, renal cell carcinoma was most common while among paediatric age group, wilms tumor was most common. Benign tumors were uncommon. Keywords: Kidney, renal cell carcinoma, wilms tumor

Diagnostic Utility of BCOR Antibody in Clear Cell Sarcomas of Kidney

2019 ◽  
Vol 28 (5) ◽  
pp. 477-481 ◽  
Author(s):  
Muhammad Zeeshan Khan ◽  
Noreen Akhtar ◽  
Usman Hassan ◽  
Sajid Mushtaq
Keyword(s):  
Clear Cell ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Nuclear Staining ◽  
Mesoblastic Nephroma ◽  
The Past ◽  
Weak Staining ◽  
Congenital Mesoblastic Nephroma ◽  
Strong Staining ◽  
Strong Nuclear Staining

Purpose. Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant renal tumor. It is the second most common renal pediatric renal malignancy after Wilms tumor. It exhibits a heterogeneous morphology, with overlapping features with its close differentials, which results in diagnostic challenges. There was no specific immunohistochemical marker in the past, to help in this regard. BCOR antibody has recently been suggested to be helpful in the differential diagnosis. We aim to study the utility of the BCOR antibody in the diagnosis of CCSK. Methods. We selected a total of 27 cases of CCSK (n = 12), Wilms tumor (n = 12), and congenital mesoblastic nephroma (n = 3). All cases were evaluated for the extent and intensity of nuclear labeling for BCOR antibody by immunohistochemistry (IHC). Results. We found that BCOR IHC was positive in 11 out of 12 cases with diffuse and strong staining in 8 of the cases. None of the cases of Wilms tumor and congenital mesoblastic nephroma were positive. Only 2 cases of Wilms tumor showed minimal and weak staining in <5% of cells. Conclusion. Diffuse and strong nuclear staining for the BCOR antibody is highly specific for CCSK among common pediatric renal malignancies. Our study confirms that BCOR IHC is a good IHC marker for the diagnosis of CCSK.

A Canine Model of Familial Mammary Gland Neoplasia

1998 ◽  
Vol 35 (3) ◽  
pp. 168-177 ◽  
Author(s):  
K. A. Schafer ◽  
G. Kelly ◽  
R. Schrader ◽  
W. C. Griffith ◽  
B. A. Muggenburg ◽  
...  
Keyword(s):  
Proportional Hazards ◽  
Age Of Onset ◽  
Malignant Tumors ◽  
Tumor Incidence ◽  
Benign Tumors ◽  
Tumor Type ◽  
Familial Predisposition ◽  
Kaplan Meier ◽  
Resistant Family ◽  
Familial Differences

Intact female Beagles from life-span studies in the Lovelace Respiratory Research Institute colony were examined for mammary tumor incidence. The breeding colony, founded in 1963, produced five generations from 28 founder females. After proportional hazards analysis, two maternal families were shown to have markedly different phenotypes, one susceptible and one resistant to mammary neoplasia, as compared with the entire colony. When tumors were subdivided into benign and malignant based on local invasiveness, familial differences in tumor incidence were preserved for each tumor type. Fifty-seven females in the susceptible family developed 149 benign and 39 malignant tumors, and 95 females in the resistant family developed 70 benign and 20 malignant tumors. The ratio of benign to malignant tumors of about 4:1 for both families was higher than expected. Using Kaplan–Meier and log-rank analyses, the susceptible family had a 50% malignant tumor incidence by age 13.6 years, whereas the resistant family did not have a 50% incidence until 17.0 years ( P = 0.0065). Because of marked censoring, Kaplan–Meier analyses could not provide an estimate of the 50% benign tumor incidence; mean incidence age was calculated instead. These estimates for benign tumors for susceptible and resistant families were 10.8 and 13.8 years ( P = 0.0001), respectively. Using χ2 tests, families had no differences in the occurrence of the types of benign ( P = 0.098) or malignant ( P = 0.194) tumors or in the ratio of benign to malignant tumors ( P = 0.778). Immunohistochemical analysis of malignant tumors from both families did not demonstrate differences in p53 mutation rate or p185 erbB-2 expression. These results suggest that 1) genetic factors produce familial differences in the age of onset of both benign and malignant mammary tumors; histologic types do not segregate by family; 2) the ratio of benign to malignant tumors is greater than formerly reported; and 3) neither p53 nor p185 erbB-2 alterations are the basis for the familial predisposition.

scholarly journals Non-Wilms' Renal Tumors In Children:A 139 Cases Series

2021 ◽  
Author(s):  
Yi Wei Fang ◽  
Hong Cheng Song ◽  
Ning Sun ◽  
Wei Ping Zhang
Keyword(s):  
Cell Cancer ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Comprehensive Treatment ◽  
Malignant Rhabdoid Tumor ◽  
Cystic Nephroma ◽  
Single Center ◽  
Congenital Mesoblastic Nephroma ◽  
Lost To Follow Up

Abstract Background:Pediatric non-Wilms renal tumors (NWRTs) which comprise a small proportion of renal tumors,are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment.This study aimed to determine the clinical characteristics, management and prognosis of children with non-Wilms' renal tumors(NWRTs). Methods:Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01–2019.10) at a single center were reviewed retrospectively.Results:The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney(MRTK)(n: 30, 21.6%), renal cell cancer(RCC)(n: 26,18.7%), clear cell sarcoma of the kidney(CCSK)(n: 24,17.3%), congenital mesoblastic nephroma(CMN)(n: 21,15.1%), cystic nephroma(CN)(n: 16,11.5%),metanephric tumours(n: 12, 8.6%), renal angiomyoliporma(RAML)(n: 3, 2.2%), renal primitive neuroectodermal tumor(rPNET)(n: 2, 1.4%), renal hemangioma(n: 2, 1.4%), inflammatory myofibroblastic tumor(IMT)(n: 2, 1.4%), ossifying renal tumor of infancy(ORTI)(n: 1, 0.7%). 123 children were followed up with an average of 42 months. 16 children were lost to follow-up. Tumor-free survival was observed in 94 children. 28 children(22.8%) were died.Conclusions:Pediatric NWRTs comprises 19.1% of all renal tumors in our single center. Accurate diagnoses along with appropriate management are important factors in improving patients outcome. The mainstay treatment of malignant NWRTs including MRTK,CCSK,RCC and PNET is comprehensive treatment. The mainstay treatment of benign NWRTs including RAML,CN, ORTI, CMN,metanephric tumours, and renal hemangioma is surgical resection alone.

scholarly journals Childhood Clear Cell Sarcoma of Kidney: Incidence and Survival

2021 ◽  
Vol 9 ◽  
Author(s):  
Hui Gao ◽  
Qi-Yuan Cheng ◽  
Qian Zhao ◽  
Long-Xiang Tao ◽  
Cheng Zhang
Keyword(s):  
Cox Regression ◽  
Clear Cell ◽  
Wilms Tumor ◽  
Tumor Development ◽  
Clear Cell Sarcoma ◽  
Cancer Surveillance ◽  
Renal Tumors ◽  
Rank Test ◽  
Cell Sarcoma ◽  
Adjusted Incidence

This study is to describe current incidence of childhood clear cell sarcoma of kidney (CCSK) and to investigate the present survival of this cancer. Surveillance, Epidemiology, and End Result (SEER) data was used to identify children with CCSK and Wilms tumor (WT) aged 0–19 years in the US. Age-adjusted incidences were estimated over the decades. Age- and sex-specific epidemiology was also presented. Propensity score matching was used to balance features of CCSK and WT cases. Log rank test was used to compare survivals and Cox regression was used to evaluate independent effects of factors. The present age-adjusted incidence of childhood CCSK was 0.205 per million, which remained stable for years and ranked third in all pediatric renal tumors. The incidence rate ratios for boy and age under 4 were 3 and 21, respectively. The current 5-year overall survival (OS) rate for CCSK was 87%, which is not evidently inferior to that for WT (90%); however the outcome of CCSK was significantly poorer if both groups were well-balanced (OS rate was 86 vs. 95%). Early year of diagnosis and distant metastasis were independent survival factors. In conclusion, occurrence of CCSK remains stable over the years, with an age-adjusted incidence of 0.205 per million. Boy and age under 4 are risk factors for tumor development. CCSK currently has a favorable outcome but its nature may be more aggressive than common kidney tumor, which in turn proves efficacy of modern treatment.

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