Last update 01 Nov 2024

Clear Cell Sarcoma of Kidney

Last update 01 Nov 2024

Basic Info

Synonyms

CCSK, CCSK - clear cell sarcoma of kidney, Childhood Clear Cell Sarcoma of the Kidney

+ [23]

Introduction

A rare pediatric sarcoma affecting the kidney. It is characterized by the presence of epithelioid or spindle cells forming cords or nests, separated by fibrovascular septa. It metastasizes to lung, bone, brain and soft tissue.

Drugs associated with Clear Cell Sarcoma of Kidney

Sunitinib Malate

Target

PDGFR x RTK x VEGFR

Mechanism

PDGFR antagonists [+2]

Active Org.

Pfizer Japan, Inc. [+4]

Originator Org.

Pfizer Inc.

Active Indication

Malignant Gastric Gastrointestinal Stromal Tumor [+12]

Inactive Indication

Adrenocortical Carcinoma [+76]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date26 Jan 2006

Mycophenolate Mofetil

Target

IMPDH

Mechanism

IMPDH inhibitors

Active Org.

Roche Registration GmbH [+9]

Originator Org.

Syntex Pharmaceuticals Ltd.

Active Indication

Hematopoietic stem cell transplantation [+24]

Inactive Indication

Acute Graft Versus Host Disease [+101]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date03 May 1995

Dalteparin Sodium

Target

factor Xa x thrombin

Mechanism

factor Xa inhibitors [+1]

Active Org.

Pfizer Inc. [+1]

Originator Org.

Pfizer Inc.

Active Indication

Angina, Unstable [+4]

Inactive Indication

Acute Lymphoblastic Leukemia [+15]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date21 Jan 1992

Clinical Trials associated with Clear Cell Sarcoma of Kidney

JPRN-UMIN000028428 / CompletedIIT

Phase II Clinical Study on Children and Adolescents/Young Adults with Clear Cell Sarcoma of the Kidney - Phase II Clinical Study on Children and Adolescents/Young Adults with Clear Cell Sarcoma of the Kidney

Start Date03 Jul 2017

Sponsor / Collaborator-

NCT03382158 / RecruitingNot ApplicableIIT

International Pleuropulmonary Blastoma/DICER1 Registry (for PPB, DICER1 and Associated Conditions)

Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm of the lung presenting in early childhood. Type I PPB is a purely cystic lesion, Type II is a partially cystic, partially solid tumor, Type III is a completely solid tumor. Treatment of children with PPB is at the discretion of the treating institution. This study builds off of the 2009 study and will also seek to enroll individuals with DICER1-associated conditions, some of whom may present only with the DICER1 gene mutation, which will help the Registry understand how these tumors and conditions develop, their clinical course and the most effective treatments.

Start Date06 Dec 2016

Sponsor / Collaborator

The University of Texas MD Anderson Cancer Center

[+15]

NCT01154816 / CompletedPhase 2IIT

A Phase II Study of MLN8237, a Selective Aurora A Kinase Inhibitor in Children With Recurrent/Refractory Solid Tumors and Leukemias

This phase II trial is studying the side effects of and how well alisertib works in treating young patients with relapsed or refractory solid tumors or leukemia. Alisertib may stop the growth of cancer cells by blocking some of the enzymes needed for cell growth.

Start Date01 Feb 2011

Sponsor / Collaborator

The Children's Oncology Group Foundation, Inc.

[+1]

100 Clinical Results associated with Clear Cell Sarcoma of Kidney

100 Translational Medicine associated with Clear Cell Sarcoma of Kidney

0 Patents (Medical) associated with Clear Cell Sarcoma of Kidney

594

Literatures (Medical) associated with Clear Cell Sarcoma of Kidney

01 Dec 2024·European Journal of Medical Genetics

Novel CTR9 germline pathogenic splice site variant in siblings with Wilms tumor from Tanzania

Article

Author: Kambainei, Lossim G ; Mremi, Alex ; Hamel, Ben C ; Majaliwa, Esther ; Mensenkamp, Arjen R ; Iman, Ali

Wilms tumor is the most common childhood renal malignancy. Though mostly non-genetic, it can be syndromic with the involvement of many Wilms tumor predisposing genes and non-syndromic with the involvement of four genes: WT1, REST, TRIM28, and CTR9. Familial and bilateral Wilms tumors do occur, but these are rare. So far, four Wilms tumor families with pathogenic variants in the CTR9 gene have been described, all (presumably) inherited from unaffected fathers, and three leading to deletion of exon 9. We are reporting female siblings, of whom one has a bilateral Wilms tumor, with a novel pathogenic splice site variant in the CTR9 gene, leading to deletion of exon 9, and inherited from their asymptomatic father. The loss of heterozygosity in the tumor was confirmed. In conclusion, CTR9 pathogenic variants are a very rare cause of Wilms tumors and typically result in familial Wilms tumors.

01 Oct 2024·Journal of Pediatric Urology

Stratification of Wilms tumor patients using physicochemical properties of the adaptive immune receptor polypeptides, IGL and TRG

Article

Author: Blanck, George ; Medikonda, Nikhila ; Gozlan, Etienne C. ; Adams, David P. ; Song, Joanna J ; Song, Joanna J. ; Sahoo, Arpan ; Gozlan, Etienne C ; Yeagley, Michelle ; Adams, David P

INTRODUCTIONWilms tumor (WT) is the most common pediatric renal malignancy. Current guidelines that stratify WT risk and determine treatment courses are inadequate, as over 60 % of WT survivors develop treatment-related complications. Recently, numerous advances in establishing patient sub-groups with different clinical features have been realized by evaluating the adaptive immune receptor (IR) complementarity determining region-3 (CDR3) amino acid (AA) sequences, a reasonable series of successes, given the prominent role of the CDR3 in antigen binding, including tumor antigen binding. However, the possibility that adaptive IR chemical variability correlates with distinct survival outcomes for WT has not yet been explored.OBJECTIVEThe goal of this study was to isolate the T-cell receptor and B-cell receptor recombination, sequencing reads from WT RNAseq files, representing the actual tumor tissue, translate the sequences to AAs, identify the adaptive IR CDR3 domains, and determine whether the physicochemical properties of those CDR3 AA sequences correlated with survival probability distinctions.STUDY DESIGNWT RNA-seq files were mined to obtain the CDR3 AAs for various adaptive IRs. The physicochemical properties of these CDR3s were examined for trends in how those properties correlated with survival probabilities for WT patients, using a Kaplan-Meier analyses, verified via several approaches.RESULTSThe above processes indicated the association of the (a) IGL CDR3s' instability index and the (b) TRG CDR3s' fraction disorder promoting features with better outcomes. Additionally, the IGL CDR3 data were assessed using the Predictor of Natural Disordered Regions web tool, which strengthened the evidence for the association with the IGL CDR3 instability index with a better outcome.DISCUSSIONThe approaches described here indicate that greater adaptive IR CDR3 instability and flexibility may serve as prognostic indicators; and may indicate the flexibility of CDR3 domains provides for greater opportunity to bind tumor antigens.CONCLUSIONFurther exploration and development of these approaches and findings may lead to new guidelines for more precise treatment regimens, or even watchful waiting periods, that could thereby decrease the lifetime occurrence of adverse events.

01 Aug 2024·Urology

Chromophobe Renal Cell Carcinoma in a Pediatric Patient With Neurofibromatosis Type 1: A Case Report and Review of the Literature

Article

Renal Cell Carcinoma is rare in the pediatric population, making up only 2%-6% of all pediatric renal tumors. Literature on pediatric Chromophobe Renal Cell Carcinoma (chRCC) is exceptionally limited. In this report, we describe the case of a 12-year-old patient with Neurofibromatosis Type 1 (NF1), incidentally found to have a kidney lesion with pathology revealing chRCC. Treatment included open partial nephrectomy with lymph node dissection and current follow-up is nearly 1 year. To our knowledge, this is the first case of chRCC in the setting of NF1.

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Clear Cell Sarcoma of Kidney

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