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BMC Urol 2022 Jun 22;22(1):89. Epub 2022 Jun 22.

Department of Urology, National Children's Medical Center, Beijing Children's Hospital of Capital Medical University, No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China.

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. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs.

Methods: Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008. Read More

PeerJ 2022 10;10:e13516. Epub 2022 Jun 10.

Department of Biology, Brigham Young University, Provo, Utah, United States.

Fewer DNA mutations have been identified in pediatric tumors than in adult tumors, suggesting that alternative tumorigenic mechanisms, including aberrant DNA methylation, may play a prominent role. In one epigenetic process of regulating gene expression, methyl groups are attached at the 5-carbon of the cytosine ring, leading to 5-methylcytosine (5mC). In somatic cells, 5mC occurs mostly in CpG islands, which are often within promoter regions. Read More

J Exp Clin Cancer Res 2022 Jun 11;41(1):199. Epub 2022 Jun 11.

College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar.

Background: Large immunogenomic analyses have demonstrated the prognostic role of the functional orientation of the tumor microenvironment in adult solid tumors, this variable has been poorly explored in the pediatric counterpart.

Methods: We performed a systematic analysis of public RNAseq data (TARGET) for five pediatric tumor types (408 patients): Wilms tumor (WLM), neuroblastoma (NBL), osteosarcoma (OS), clear cell sarcoma of the kidney (CCSK) and rhabdoid tumor of the kidney (RT). We assessed the performance of the Immunologic Constant of Rejection (ICR), which captures an active Th1/cytotoxic response. Read More

Int J Surg Pathol 2022 Apr 28:10668969221097389. Epub 2022 Apr 28.

Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China.

Mixed epithelial and stromal tumors of the kidney (MESTK) are rare and recently defined entities that comprise both epithelial and stromal cells. MESTK is mostly benign; however, to date, 18 borderline or malignant cases have been reported. In this study, we report a case of carcinosarcoma exhibiting a large carcinoma and small sarcoma component, and review the relevant literature. Read More

Transl Cancer Res 2022 Jan;11(1):288-294

Department of Urology, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.

Clear cell sarcoma of the kidney (CCSK) in adults is extremely rare. In fact, only 16 adult CCSK cases have been reported from 1989 to 2020 in the English language literature. The pathologic diagnosis of the disease is difficult, and the optimal treatment is still unknown. Read More

J Pathol Clin Res 2022 05 17;8(3):217-232. Epub 2022 Feb 17.

SIREDO Oncology Center of Care, Innovation and Research for Children, Adolescent and Young Adults with Cancer, Institut Curie, Paris, France.

BCOR-ITD tumours form an emerging family of aggressive entities with an internal tandem duplication (ITD) in the last exon of the BCOR gene. The family includes cerebral tumours, termed central nervous system BCOR-ITD (CNS BCOR-ITD), and sarcomatous types described in the kidney as clear cell sarcoma of the kidney (CCSK), in the endometrium as high-grade endometrial stromal sarcoma, and in the bone and soft tissue as undifferentiated round cell sarcoma or primitive myxoid mesenchymal tumour of infancy. Based on a series of 33 retrospective cases, including 10 CNS BCOR-ITD and 23 BCOR-ITD sarcomas, we interrogated the homogeneity of the entity regarding clinical, radiological, and histopathological findings, and molecular signatures. Read More

Cancer Rep (Hoboken) 2022 02 29;5(2):e1458. Epub 2021 Dec 29.

Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Background: Clear cell sarcoma of the kidney (CCSK) is the second most common pediatric renal tumor.

Case: A 2-year-old boy was diagnosed with CCSK, which relapsed four times until he yielded to the disease at the age of 7 years. To characterize the longitudinal genetic alterations occurring in the present case, we performed targeted-capture sequencing by pediatric solid tumors panel (381 genes) for longitudinally sampled tumors, including autopsy samples of metastasis. Read More

Indian J Thorac Cardiovasc Surg 2022 Jan 29;38(1):92-95. Epub 2021 Sep 29.

Department of Pediatric Cardiac Sciences, Sir Gangaram Hospital, New Delhi, India.

Clear cell sarcoma of the kidney is a rare variety of renal tumor accounting for less than 5% of all pediatric renal tumors. Cardiopulmonary bypass along with hypothermic circulatory arrest is frequently used for management of tumor thrombus extending into supra-hepatic inferior vena cava and right atrium. In this paper, we present a strategy of avoiding circulatory arrest and hypothermia and thereby fast-tracking the recovery in managing a case of clear cell sarcoma of the kidney in a 3. Read More

Int J Surg Pathol 2022 Jun 13;30(4):437-442. Epub 2021 Dec 13.

117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China.

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma. We report one case of primary kidney SEF occurring in a 38-year-old man. Microscopically, epithelioid neoplastic cells are mainly arranged in cords and nests embedded in the dense sclerosing stroma. Read More

BMC Med Imaging 2021 12 1;21(1):181. Epub 2021 Dec 1.

Department of Radiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Xicheng District, Beijing, China, 100045.

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). Read More

Radiologe 2021 Jul 18;61(7):619-628. Epub 2021 Jun 18.

Klinik für Pädiatrische Onkologie und Hämatologie, Universitätskinderklinik, Universitätsklinikum des Saarlandes, Homburg, Deutschland.

Clinical/methodological Issue: Renal tumors in children are treated according to the guidelines of the Renal Tumor Study Group of the International Society of Pediatric Oncology (SIOP-RTSG). Nephroblastoma is the most frequent renal tumor in children.

Standard Radiological Methods: After sonography, magnetic resonance imaging (MRI) is the preferred imaging modality. Read More

Int J Surg Case Rep 2021 May 30;82:105943. Epub 2021 Apr 30.

Faculty of Medicine, Kilimanjaro Christian Medical University College, Box 2240, Moshi, Tanzania; Department of Urology, Kilimanjaro Christian Medical Centre, Box 3010, Moshi, Tanzania.

Introduction And Importance: Xanthogranulomatous pyelonephritis is an extremely rare but known form of chronic pyelonephritis resulting from prolonged suppuration of the kidney. Pre-operatively, it may mimic renal tuberculosis or neoplastic lesions including renal cell carcinoma due to its vague clinical presentation, equivocal laboratory and radiological investigations. Due to its rarity and academic interest, herein we report such a rare case we recently encountered in our clinical practice. Read More

Korean J Radiol 2021 07 1;22(7):1185-1193. Epub 2021 Apr 1.

Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.

Objective: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK.

Materials And Methods: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Read More

BMC Pediatr 2021 04 4;21(1):159. Epub 2021 Apr 4.

Department of Pathology, University of Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany.

Background: Lipoblastoma is a rare benign mesenchymal neoplasm of infancy that most commonly occurs on the extremities and trunk but can arise at variable sites of the body. Retroperitoneal lipoblastomas are particularly rare but can grow to enormous size, and preoperative diagnosis is difficult with diverse, mostly malignant differential diagnoses that would lead to aggressive therapy. Since lipoblastoma is a benign tumor that has an excellent prognosis after resection, correct diagnosis is crucial. Read More

Front Oncol 2021 25;11:645512. Epub 2021 Feb 25.

APHM, CHU Timone, Service d'Anatomie Pathologique et de Neuropathologie, Marseille, France.

is an epigenetic regulator altered by various mechanisms including -internal tandem duplication (-ITD) in a wide range of cancers. Six different -ITD in the 3'-part of the coding sequence of exon 15 have been reported ranging from 89 to 114 bp in length. -ITD is a common genetic alteration found in clear cell sarcoma of the kidney and primitive myxoid mesenchymal tumor of infancy (PMMTI) and it characterizes a new type of central nervous system tumor: "CNS tumor with -ITD". Read More

J Nippon Med Sch 2022 May 9;89(2):233-237. Epub 2021 Mar 9.

Department of Pediatrics, Nippon Medical School Hospital.

Clear-cell sarcoma of the kidney (CCSK) is a rare, aggressive pediatric renal tumor. Intratumoral hemorrhage and tumor rupture are oncologic emergencies requiring a rapid and appropriate response. An 11-year-old boy visited our hospital with abdominal distension of 1 month's duration. Read More

J Pediatr Hematol Oncol 2022 Mar;44(2):e496-e499

Department of Pediatrics.

We describe a 21-month-old male with relapsed clear cell sarcoma of the kidney receiving enteral nutrition who experienced recurrent, ketotic hypoglycemia. During relapse therapy, he had recurrent hypoglycemia episodes, in the setting of hematochezia and diarrhea. Evaluation revealed low carnitine levels. Read More

Curr Oncol Rep 2021 02 16;23(3):33. Epub 2021 Feb 16.

Aflac Cancer and Blood Disorders Center, Children Healthcare of Atlanta, Atlanta, GA, USA.

Purpose Of Review: Pediatric renal tumors account for 7% of new cancer diagnoses in children. Here, we will review results from recently completed clinical trials informing the current standard of care and discuss targeted and immune therapies being explored for the treatment of high risk or relapsed/refractory pediatric renal malignancies.

Recent Findings: Cooperative group trials have continued to make improvements in the care of children with pediatric tumors. Read More

Clin Perinatol 2021 03 11;48(1):71-81. Epub 2021 Jan 11.

Maine Children's Cancer Program, Department of Pediatrics, Maine Medical Center, Tufts School of Medicine, 100 Campus Drive, Suite 107, Scarborough, ME 04074, USA. Electronic address:

Renal tumors are rare in the neonatal period. Although some may be detected prenatally, a greater proportion present after birth, most often with a palpable abdominal mass with or without other associated symptoms. Cross-sectional imaging is typically followed by radical nephrectomy to make a specific histologic diagnosis to determine the need for additional therapy. Read More

Pediatr Blood Cancer 2021 05 12;68(5):e28860. Epub 2021 Jan 12.

Department of Paediatric Haematology and Oncology, St. Anna Children's Hospital, Medical University, Vienna, Austria.

Introduction: Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce. Read More

Cytopathology 2021 May 5;32(3):378-382. Epub 2021 Jan 5.

Department of Histopathology, PGIMER, Chandigarh, India.

Zhonghua Bing Li Xue Za Zhi 2020 Dec;49(12):1308-1310

Shengli Clinical Medical College of Fujian Medical University; Department of Pathology, Fujian Provincial Hospital, Fuzhou 350001, China.

J Pediatr Surg 2021 Mar 29;56(3):580-586. Epub 2020 Jul 29.

Department of Urology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, 136 Zhongshan 2nd RD, Yuzhong District, Chongqing 400014, China; Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatric, 136 Zhongshan 2nd RD, Yuzhong District, Chongqing 400014, China. Electronic address:

Background: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor. This study aims to describe the clinical characteristics and outcomes of CCSK patients in one of the largest pediatric medical centers in China.

Methods: We included all patients diagnosed with CCSK between January 2008 and March 2019 at the Children's Hospital of Chongqing Medical University, China. Read More

Pediatr Radiol 2020 12 30;50(13):1907-1920. Epub 2020 Nov 30.

Department of Radiology, Pediatric Section, Baylor Scott and White Health-Temple, McLane Children's Medical Center, Texas A&M Health Science Center, 2401 S. 31st St., MS-01-W256, Temple, TX, 76508, USA.

Fetal abdominal tumors are rare, usually benign, and cause a great deal of anxiety for expectant parents and the physicians counseling them. In this paper the author reviews the most common fetal abdominal tumors in the liver (hemangioma, mesenchymal hamartoma, hepatoblastoma, metastases) and the kidney (congenital mesoblastic nephroma, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma), and suprarenal mass lesions (adrenal neuroblastoma, adrenal hemorrhage, and subdiaphragmatic extralobar pulmonary sequestration). The author describes the imaging approach, imaging appearance and differentiating features of tumors, and differences between fetal and childhood appearances of tumors. Read More

Surg Pathol Clin 2020 Dec 9;13(4):695-718. Epub 2020 Oct 9.

Department of Pathology, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Box 120, Aurora, CO 80045, USA. Electronic address:

Molecular characterization has led to advances in the understanding of pediatric renal tumors, including the association of pediatric cystic nephromas with DICER1 tumor syndrome, the metanephric family of tumors with somatic BRAF mutations, the characterization of ETV6-NTRK3-negative congenital mesoblastic nephromas, the expanded spectrum of gene fusions in translocation renal cell carcinoma, the relationship of clear cell sarcoma of the kidney with other BCOR-altered tumors, and the pathways affected by SMARCB1 alterations in rhabdoid tumors of the kidney. These advances have implications for diagnosis, classification, and treatment of pediatric renal tumors. Read More

Pediatr Dev Pathol 2020 Nov-Dec;23(6):424-430. Epub 2020 Aug 13.

Ospedale Pediatrico Bambino Gesu, Istituto Ricovero e Cura a Carattere Scientifico, Rome, Italy.

Introduction: Somatic internal tandem duplication of 3' of ( ITD) has been found in clear cell sarcomas of the kidney (CCSK), soft tissue undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of infancy (URCS/PMMTI), and a subgroup of central nervous system high-grade neuroepithelial tumors (CNS-HGNET). ITD+ tumors share morphologic features. Expression of OLIG2 and epidermal growth factor receptor (EGFR) has been reported in CNS-HGNET with ITD. Read More

Diagn Pathol 2020 Jul 23;15(1):95. Epub 2020 Jul 23.

Department of Urology, Ondokuz Mayis University School of Medicine, Samsun, Turkey.

Background: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa. Here, we present an unusual case of primary DSRCT of kidney.

Case Presentation: The patient was an 8-year-old girl with a large renal mass which was confused with primitive neuroectodermal tumor (PNET) in the needle biopsy. Read More

Int J Surg Pathol 2021 Apr 10;29(2):194-197. Epub 2020 Jul 10.

Department of Pathology, 89063UT Southwestern Medical Center, Dallas TX, USA.

Primary renal sarcoma with gene fusion is a rare tumor with only 7 cases reported in the English literature. The morphologic features of this tumor strikingly overlap with clear cell sarcoma of the kidney and synovial sarcoma. Accurate diagnosis can be challenging. Read More