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Arch Pathol Lab Med 2019 Mar 27. Epub 2019 Mar 27.

From the Division of Anatomical Pathology, University of Cape Town, Cape Town, and the National Health Laboratory Service, Western Cape, South Africa (Drs Aldera and Pillay); the Department of Anatomical Pathology, Groote Schuur Hospital, Western Cape, South Africa (Dr Aldera); and the Department of Anatomical Pathology, Red Cross War Memorial Children's Hospital, Cape Town, South Africa (Dr Pillay).

Clear cell sarcoma of the kidney is an uncommon malignant pediatric renal neoplasm that typically presents in the 2- to 3-year age group and has a propensity for aggressive behavior and late relapses. Histologically, this tumor exhibits a great diversity of morphologic patterns which can mimic most other pediatric renal neoplasms, often leading to confusion and misdiagnosis. Until recently, adjunct immunohistochemical and molecular genetic tests to support the diagnosis were lacking. Read More

Magn Reson Imaging Clin N Am 2019 May;27(2):279-290

Department of Radiology, Seattle Children's, University of Washington, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA.

Pediatric renal tumors may be malignant or benign. Wilms tumor, the most common malignant pediatric renal tumor, arises sporadically or with various syndromes. Renal cell carcinoma typically presents in older children. Read More

Am J Surg Pathol 2019 Mar 8. Epub 2019 Mar 8.

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY.

BCOR immunoreactivity is a sensitive and highly specific marker for clear cell sarcoma of the kidney (CCSK). However, a subset of adult renal sarcomas which overexpress BCOR are negative for BCOR genetic alterations, including BCOR gene fusions or BCOR-internal tandem duplication, and thus remain unclassified. We report 5 such undifferentiated renal/perirenal sarcomas which raised the differential diagnosis of CCSK due to their morphologic appearance and strong BCOR immunoreactivity, but which on RNA sequencing proved to be malignant solitary fibrous tumors (SFTs). Read More

Pediatr Blood Cancer 2019 Jun 13;66(6):e27627. Epub 2019 Feb 13.

Department of Cellular Pathology, University Hospital of Wales, Cardiff, UK.

Introduction: The International Society of Paediatric Oncology (SIOP) protocols recommend preoperative chemotherapy appropriate for Wilms tumors (WTs) in children with renal tumors aged ≥6 months, reserving biopsy for "atypical" cases. The Children's Cancer and Leukaemia Group (CCLG) joined the SIOP-WT-2001 study but continued the national practice of biopsy at presentation.

Method: Retrospective study of concordance between locally reported renal tumor biopsies and central pathology review nephrectomy diagnoses of children enrolled by CCLG centers in the SIOP-WT-2001 study. Read More

Pediatr Blood Cancer 2019 Jun 12;66(6):e27641. Epub 2019 Feb 12.

Imaging Department, Institut Curie, Paris, France.

Objectives: According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image-guided needle biopsy.

Methods: Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0-17. Read More

Diagn Pathol 2019 Feb 8;14(1):13. Epub 2019 Feb 8.

Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.

Background: Clear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric renal neoplasm with a heterogeneous histological appearance which often results in misdiagnosis. There are no specific immunohistochemical markers which can help in differentiating CCSK from other pediatric renal neoplasms. Recently Cyclin D1 has been investigated as a possible marker in this regard. Read More

Arch Pathol Lab Med 2019 Jan 10. Epub 2019 Jan 10.

From the Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Republic of Singapore.

Clear cell sarcoma of the kidney is the second most common primary renal malignancy in childhood. It is histologically diverse, making accurate diagnosis challenging in some cases. Recent molecular studies have uncovered BCOR exon 15 internal tandem duplications in most cases, and YWHAE-NUTM2 fusion in a few cases, with the remaining cases having other genetic mutations, including BCOR-CCNB3 fusion and EGFR mutations. Read More

BMJ Case Rep 2018 Dec 9;11(1). Epub 2018 Dec 9.

Department of Urology, Tallaght Hospital, Dublin, Ireland.

We report the first case of a synchronous ipsilateral primitive neuroectodermal tumour (PNET) and clear cell renal cell carcinoma of the kidney. A 37-year-old man presented to the emergency department with a 24-hour history of colicky abdominal pain and visible haematuria. He had no relevant surgical or medical history. Read More

Case Rep Pathol 2018 16;2018:3462096. Epub 2018 Oct 16.

Department of Pathology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA.

Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood. Histologically, CCSK can mimic myxoid variant of synovial sarcoma (SS); however, the double positivity for CD99 and TLE1 in SS helps in excluding CCSK. Herein, we report a rare case of CCSK arising in the left kidney of a 3-year-old girl. Read More

J Pediatr Surg 2019 Mar 2;54(3):550-556. Epub 2018 Oct 2.

Department of Radiology, The Children's Hospital, Zhejiang University School of Medicine, No. 3333 Binsheng Road, 310052 Hangzhou, China. Electronic address:

Background: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive malignant renal tumor. We describe our experience with neoadjuvant transcatheter arterial chemoembolization (TACE) and systematic chemotherapy for the treatment of advanced CCSK in children.

Methods: Between January 2010 and December 2016, seven patients (3 boys and 4 girls; median 2. Read More

Pediatr Blood Cancer 2019 Jan 25;66(1):e27450. Epub 2018 Sep 25.

Department of Epidemiology and Cancer Control, St Jude Children's Research Hospital, Memphis, Tennessee.

Purpose: To improve the event-free survival (EFS) and overall survival (OS) for patients with clear cell sarcoma of the kidney (CCSK) by incorporating cyclophosphamide and etoposide into treatment on National Wilms Tumor Study (NWTS)-5.

Patients And Methods: Patients less than 16 years of age with a centrally confirmed pathological diagnosis of CCSK were eligible for treatment on this prospective single-arm study conducted between August 1995 and June 2002. Staging consisted of CT scans of chest, abdomen, pelvis, bone scan, skeletal survey, and CT or MRI of the head. Read More

J Pediatr Urol 2018 Dec 1;14(6):571.e1-571.e6. Epub 2018 Aug 1.

Department of Radiology, The First Affiliated Hospital, Sun Yat-sen University, 58th, The Second Zhongshan Road, Guanzhou, China. Electronic address:

Introduction: Congenital mesoblastic nephroma (CMN) is a common solid renal tumor in the neonate. Congenital mesoblastic nephroma can be divided into classic, cellular, and mixed types. The prognosis of CMN is very optimistic. Read More

Genes Chromosomes Cancer 2018 10 20;57(10):525-529. Epub 2018 Aug 20.

Department of Pediatric Hematology and Oncology Research, National Research Institute for Child Health and Development, Tokyo, Japan.

Clear cell sarcoma of the kidney (CCSK) is the second most common renal malignancy in children. The prognosis is poorer in CCSK than in Wilms' tumor, and multimodal treatment including surgery, intensive chemotherapy, and radiation is required to improve the outcome for children with CCSK. Histological evaluation is required for the diagnosis. Read More

Urology 2018 Oct 1;120:222-230. Epub 2018 Aug 1.

University of Colorado, Department of Surgery, Division of Urology; Children's Hospital Colorado, Aurora, CO. Electronic address:

Objective: To identify factors impacting overall survival (OS) in children, adolescents, and young adults with high-risk renal tumors (HRRTs).

Methods: The National Cancer Database was queried for patients ≤30 years old diagnosed with anaplastic Wilms tumors (AWT), clear cell sarcoma of the kidney (CCSK), or rhabdoid tumor of the kidney (RTK) between 2004 and 2013. Demographic, clinical, and OS data were abstracted. Read More

Nat Commun 2018 06 18;9(1):2378. Epub 2018 Jun 18.

Wellcome Trust Sanger Institute, Hinxton, CB10 1SA, UK.

Soft tissue tumors of infancy encompass an overlapping spectrum of diseases that pose unique diagnostic and clinical challenges. We studied genomes and transcriptomes of cryptogenic congenital mesoblastic nephroma (CMN), and extended our findings to five anatomically or histologically related soft tissue tumors: infantile fibrosarcoma (IFS), nephroblastomatosis, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma of the kidney. A key finding is recurrent mutation of EGFR in CMN by internal tandem duplication of the kinase domain, thus delineating CMN from other childhood renal tumors. Read More

Am J Surg Pathol 2018 Aug;42(8):1128-1131

Department of Pathology, Memorial Sloan Kettering Cancer Center New York NY.

Pediatr Blood Cancer 2018 Jul 6;65(7):e27056. Epub 2018 Apr 6.

Osaka Women's and Children's Hospital, Izumi, Japan.

Background: Japan Wilms Tumor Study-2 (JWiTS-2) mandated central pathology review for all case registrations. The study aimed to compare the outcomes of patients with unilateral Wilms tumor enrolled on the JWiTS-1 and JWiTS-2 trials.

Procedure: The JWiTS-2 trial (2006-2014), a prospective, single-arm study, required compulsory submission of histologic slides to central pathology, while in the JWiTS-1 trial, such submission was not compulsory. Read More

North Clin Istanb 2018 18;5(1):60-63. Epub 2018 Jan 18.

Department of Pediatric Hematology and Oncology, Eskisehir Osmangazi Universitiy Faculty of Medicine, Eskisehir, Turkey.

Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It represents between 2% and 9% of all pediatric renal tumors, and generally arises before the age of 5 years. It often mimics other pediatric renal tumors. Read More

Pediatr Blood Cancer 2018 05 7;65(5):e26975. Epub 2018 Feb 7.

Fetal Pediatr Pathol 2018 Apr 6;37(2):126-133. Epub 2018 Mar 6.

a Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish Hospital/St. Louis Children's Hospital , Washington University Medical Center , St. Louis , Missouri , USA.

Introduction: Clear cell sarcoma of the kidney (CCSK) is childhood neoplasm with its own distinctive pattern of metastasis and may appear after a disease free interval of 5 years or more.

Materials And Methods: Histopathology and immunohistochemistry were available from the radical nephrectomy and the later partial cystectomy, which was performed after a seven disease-free interval.

Results: The pathologic features of the primary tumor were those of a classic CCSK with a monotypic pattern of uniform rounded to ovoid tumor cells with a background network of delicate blood vessels. Read More

Nat Rev Urol 2018 May 27;15(5):309-319. Epub 2018 Feb 27.

Department of Paediatric Oncology, Princess Máxima Center for Paediatric Oncology, Utrecht, Netherlands.

The International Society of Paediatric Oncology-Renal Tumour Study Group (SIOP-RTSG) has developed a new protocol for the diagnosis, treatment, and follow-up monitoring of childhood renal tumours - the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol). This protocol has been designed to continue international collaboration in the treatment of childhood renal tumours and will be implemented in over 50 different countries. Clear cell sarcoma of the kidney, which is a rare paediatric renal tumour that most commonly occurs in children between 2 and 4 years of age, is specifically addressed in the UMBRELLA protocol. Read More

J Pathol 2018 Mar 23;244(3):334-345. Epub 2018 Jan 23.

School of Medicine, University of Dublin, Trinity College, Dublin, Ireland.

The oncogenic mechanisms and tumour biology underpinning clear cell sarcoma of the kidney (CCSK), the second commonest paediatric renal malignancy, are poorly understood and currently, therapy depends heavily on doxorubicin with cardiotoxic side-effects. Previously, we characterized the balanced t(10;17)(q22;p13) chromosomal translocation, identified at that time as the only recurrent genetic aberration in CCSK. This translocation results in an in-frame fusion of the genes YWHAE (encoding 14-3-3ϵ) and NUTM2, with a somatic incidence of 12%. Read More

Brain Pathol 2018 09 28;28(5):710-720. Epub 2017 Dec 28.

Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Central nervous system high-grade neuroepithelial tumors with BCOR alteration (CNS HGNET-BCOR) are a recently reported rare entity, identified as a small fraction of tumors previously institutionally diagnosed as so-called CNS primitive neuroectodermal tumors. Their genetic characteristic is a somatic internal tandem duplication in the 3' end of BCOR (BCOR ITD), which has also been found in clear cell sarcomas of the kidney (CCSK) and soft tissue undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of infancy (URCS/PMMTI), and these BCOR ITD-positive tumors have been reported to share similar pathological features. In this study, we performed a clinicopathological and molecular analysis of six cases of CNS HGNET-BCOR, and compared them with their counterparts in the kidney and soft tissue. Read More

Indian J Med Paediatr Oncol 2017 Jul-Sep;38(3):357-359

Department of Paediatric Surgery, LTMG Hospital, Mumbai, Maharashtra, India.

Metanephric stromal tumor (MST) of kidney is an under-reported benign stromal specific renal neoplasm with good prognosis. This tumor is to be differentiated from congenital mesoblastic nephroma and clear-cell sarcoma of the kidney. In this case report, we describe the imaging, gross, and microscopic features of MST with ischemic left kidney in a 1-month-old child and discuss treatment with relevant literature. Read More

Am J Surg Pathol 2018 03;42(3):335-341

Department of Pathology, Brigham and Women's Hospital, Harvard Medical School.

Endometrial stromal sarcomas (ESSs) are mesenchymal uterine tumors characterized by recurrent genetic events, most commonly chromosomal rearrangements, that create oncogenic gene fusions. High-grade endometrial stromal sarcomas (HG-ESSs), as defined in the 2014 World Health Organization Classification, typically contain oncogenic YWHAE-NUTM2 fusions; however, although not well characterized, there are tumors morphologically overlapping with HG-ESS that do not contain the YWHAE-NUTM2 fusions. These fusions are also found in certain pediatric primitive sarcomas, including clear cell sarcoma of the kidney and soft tissue undifferentiated round cell sarcoma of infancy. Read More

Mol Oncol 2018 02 14;12(2):166-179. Epub 2017 Dec 14.

Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.

We recently identified hypermethylation at the gene promoter of transcription factor 21 (TCF21) in clear cell sarcoma of the kidney (CCSK), a rare pediatric renal tumor. TCF21 is a transcription factor involved in tubular epithelial development of the kidney and is a candidate tumor suppressor. As there are no in vitro models of CCSK, we employed a well-established clear cell renal cell carcinoma (ccRCC) cell line, 786-O, which also manifests high methylation at the TCF21 promoter, with consequent low TCF21 expression. Read More

Histopathology 2018 Jan 30;72(2):320-329. Epub 2017 Oct 30.

VIVA-KKH Paediatric Brain and Solid Tumour Laboratory, KK Women's and Children's Hospital, Singapore.

Aims: Clear cell sarcoma of the kidney (CCSK) is a rare paediatric renal malignant tumour. The majority of CCSKs have internal tandem duplications (ITDs) of the BCOR gene, whereas a minority have the YWHAE-NUTM2 gene fusion. A third 'double-negative' (DN) category comprises CCSKs with neither BCOR ITDs nor YWHAE-NUTM2 fusion. Read More

Am J Surg Pathol 2017 Dec;41(12):1702-1712

*Departments of Pathology and Oncology, Johns Hopkins University School of Medicine, Baltimore, MD †Department of Pathology, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan ‡Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY §Lab Bacchi, São Paulo, Brasil ∥University of Pittsburgh School of Medicine, Pittsburgh, PA.

We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Read More

Am J Surg Pathol 2017 Sep;41(9):1254-1260

Departments of *Pathology and Neuropathology †Radiotherapy ¶Hematology & Pediatric Oncology **Pediatric Neurosurgery, Timone Hospital, France #INSERM, CRO2 UMR_S 911, Aix-Marseille Université (AMU) ‡Clinical Cooperation Unit Neuropathology §Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) ∥Cancer Consortium (DKTK), Core Center Heidelberg, Heidelberg ††Department of Neuropathology, Medical Center Sigmund Freud, University of Bonn, Bonn, Germany.

The central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR) is a recently described molecular entity. We report 3 new CNS HGNET-BCOR cases sharing common clinical presentation and pathologic features. The 3 cases concerned children aged 3 to 7 years who presented with a voluminous mass of the cerebellum. Read More

Int J Surg Pathol 2018 Feb 11;26(1):56-63. Epub 2017 Jul 11.

1 Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

The majority of mixed epithelial and stromal tumors (MEST) of the kidney are benign entities found in female patients. Malignant MEST of the kidney is an extremely rare entity that often behaves clinically similar to an undifferentiated sarcoma. We report a case of a malignant MEST with synchronous papillary and clear cell renal cell carcinomas (RCCs) in a 61-year-old Caucasian man who presented with an incidental finding of a left renal mass on workup for back pain. Read More

J Natl Compr Canc Netw 2017 07;15(7):868-871

Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare tumor with <20 cases reported to date. Recently PMMTI tumors have been found to harbor internal tandem duplication (ITD), the same genetic alteration detected in clear cell sarcoma of the kidney (CCSK). Complete surgical resection of PMMTI is often curative, but no standard of care has been established for unresectable tumors. Read More

Hum Pathol 2017 09 10;67:226-228. Epub 2017 May 10.

Department of Medicine-DIMED, Pathology Unit, University of Padova, 35128 Padova, Italy.

Hum Pathol 2017 09 10;67:225-226. Epub 2017 May 10.

Department of Pathology, Ghent University and Ghent University Hospital, 9000 Ghent, Belgium.

Pediatr Blood Cancer 2017 Nov 25;64(11). Epub 2017 Apr 25.

Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Clear cell sarcoma of kidney (CCSK) is a rare renal malignancy, previously unreported in horseshoe kidney (HSK). B-cell lymphoma 6 corepressor (BCOR) gene internal tandem duplication (ITD) was identified as a recurrent somatic alteration in approximately 85% of CCSKs. This and the YWHAE-NUTM2B/E fusion, the second most common recurrent molecular alteration in CCSK (10%), are considered to be mutually exclusive. Read More

Urology 2017 Aug 21;106:193-195. Epub 2017 Apr 21.

Department of Surgery, Division of Urology, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO. Electronic address:

Clear cell sarcoma of the kidney (CCSK) is the second most common pediatric renal malignancy after Wilms tumor. CCSK has the potential to metastasize to distant sites and was historically known as the bone metastasizing renal tumor. We report an exceedingly rare case of a bladder recurrence of CCSK. Read More

Appl Immunohistochem Mol Morphol 2018 Nov/Dec;26(10):721-726

Department of Pathology and Laboratory Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.

Pediatric renal tumors (PRT) with small round blue or spindle cell morphology can be diagnostically challenging and only a limited number of immunohistochemical markers have been documented to help in the diagnosis: paired box (Pax) 2 and nerve growth factor receptor (NGFR) positivity have been demonstrated in Wilms tumor (WT) and clear cell sarcoma of the kidney (CCSK), respectively. However, the immunohistochemical expression of these markers in other PRT remains unknown. This study investigated Pax8, Pax2, and NGFR immunophenotype in a large series of PRT. Read More

Eur J Cancer 2016 Oct 28;68:179-195. Epub 2016 Oct 28.

Cancer Section, University College London, Institute of Child Health, UK.

In Europe, almost 1000 children are diagnosed with a malignant renal tumour each year. The vast majority of cases are nephroblastoma, also known as Wilms' tumour (WT). Most children are treated according to Société Internationale d'Oncologie Pédiatrique Renal Tumour Study Group (SIOP-RTSG) protocols with pre-operative chemotherapy, surgery, and post-operative treatment dependent on stage and histology. Read More

Int J Surg Case Rep 2016 3;27:198-201. Epub 2016 Sep 3.

HPB Unit, Department of Surgery, University Hospital of Ioannina and Medical School, University of Ioannina, Stavros Niarchou Avenue, 45110 Greece.

Introduction: Pancreatic metastases are uncommon and only found in a minority of patients with widespread metastatic disease at autopsy. The most common primary cancer site resulting in pancreatic metastases is the kidney, followed by colorectal cancer, melanoma, breast cancer, lung carcinoma and sarcoma.

Presentation Of Case: Herein, we report a 63-year-old male patient who presented -3. Read More

Thromb Res 2016 Apr 8;140 Suppl 1:S198-9. Epub 2016 Apr 8.

Haematology/Oncology Division, The Hospital for Sick Children, University of Toronto.

Introduction: Venous thrombotic events (VTE) are becoming more and more common in children, particularly in the hospital setting. To date, 1 in 200 children admitted to tertiary pediatric hospitals are now being recognized to develop VTE. Amongst those patients with an identified thrombotic occlusion, pediatric patients diagnosed with renal tumors have long been recognized, but their ideal management in the instances of vascular invasion remains controversial. Read More

Medicine (Baltimore) 2016 Apr;95(15):e3377

From the Department of Radiology (WL, ZL), the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province; and Department of Function Examination (WH), Tangshan TCM Hospital, Tangshan, Hebei Province, China.

Follicular dendritic cell (FDC) sarcoma is a type of malignant tumor that originates from immune system-related FDCs. Pancreatic FDC sarcoma is a rare disease, and the specificity of the clinical presentation and laboratory results is unknown. We report the clinical process and imaging features of one case of pancreatic FDC sarcoma. Read More

Radiographics 2016 Mar-Apr;36(2):499-522

From the Department of Radiology and Radiological Sciences (E.M.C.), F. Edward Hébert School of Medicine, Uniformed Services University of the Health Sciences (A.R.G.), 4301 Jones Bridge Rd, Bethesda, MD 20814; Pediatric Radiology Section, American Institute for Radiologic Pathology, Silver Spring, Md (E.M.C.); and Department of Pathology, Eastern Virginia Medical School, Norfolk, Va (R.M.C.).

Wilms tumor is the second most common pediatric solid tumor and by far the most common renal tumor of infants and young children. As most tumors are large at presentation and are treated with nephrectomy, the role of imaging is primarily in preoperative planning and evaluation for metastatic disease. However, with treatment protocols increasingly involving use of preoperative (neoadjuvant) chemotherapy (the standard in Europe) and consideration of nephron-sparing surgery, the role of imaging is evolving to include providing initial disease staging information and a presumptive diagnosis to guide therapy. Read More

Bull Cancer 2016 Apr 28;103(4):402-11. Epub 2016 Feb 28.

Centre Léon-Bérard, institut d'hématologie et d'oncologie pédiatrique, 69008 Lyon, France.

Clear cell sarcoma of the kidney (CCSK) is a rare tumor that is diagnosed most often in children between 2- and 4-years-old of age. Usually, patients with CCSK are treated in international study for intrarenal tumors, preferentially Wilms tumor, according to bad histopronostic group. The purpose of this paper is to review the most important features in 2015 about epidemiology, radiology, anatomopathology and genetic of CCSK, and above all a synthesis about successive treatment strategies with their results. Read More

Oncol Lett 2016 Feb 4;11(2):953-958. Epub 2015 Dec 4.

Department of Nephrology, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.

Despite clear cell sarcoma of the kidney (CCSK) being the second most common renal tumor in children, its mechanism has not yet been fully investigated. The aim of the present study was to investigate the potential role of vascular endothelial growth factor A (VEGFA) in CCSK development. Following preprocessing of the original GSE2712 data, the differentially-expressed genes (DEGs) between 14 CCSK and 3 fetal kidney samples were identified through Significance Analysis of Microarrays, using the R package. Read More

J Cancer Res Ther 2015 Oct-Dec;11(4):1030

Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.

Renal cell carcinoma (RCC) is the most common malignancy of the kidney and has multiple subtypes. The sarcomatoid variety is considered a form of dedifferentiated carcinoma. It is more commonly associated with clear cell variant but very few are associated with chromophobe RCC. Read More

Genes Chromosomes Cancer 2016 06 22;55(6):549-50. Epub 2016 Mar 22.

Department of Pathology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University's Feinberg School of Medicine and Robert H. Lurie Cancer Center, Chicago, IL.

Oncotarget 2016 Mar;7(10):11127-36

Department of Clinical Genetics, Lund University, University and Regional Laboratories, Lund, Sweden.

The global methylation profile and the mutational status of 633 specific epigenetic regulators were analyzed in the pediatric tumor clear cell sarcoma of the kidney (CCSK). Methylation array analyses of 30 CCSKs revealed CCSK tumor DNA to be globally hypermethylated compared to Wilms tumor, normal fetal kidney, and adult kidney. The aberrant methylation pattern of CCSKs was associated with activation of genes involved in embryonic processes and with silencing of genes linked to normal kidney function. Read More

Nat Commun 2015 Nov 17;6:8891. Epub 2015 Nov 17.

Texas Children's Cancer Center, Houston, Texas 77030, USA.

The X-linked BCL-6 co-repressor (BCOR) gene encodes a key constituent of a variant polycomb repressive complex (PRC) that is mutated or translocated in human cancers. Here we report on the identification of somatic internal tandem duplications (ITDs) clustering in the C terminus of BCOR in 23 of 27 (85%) pediatric clear cell sarcomas of the kidney (CCSK) from two independent cohorts. We profile CCSK tumours using a combination of whole-exome, transcriptome and targeted sequencing. Read More