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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 Apr 1. 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 2021 Mar 9. Epub 2021 Mar 9.

Department of Pediatrics, Nippon Medical School.

Clear cell sarcoma of the kidney (CCSK) is a rare aggressive pediatric renal tumor. Intratumoral hemorrhage and tumor rupture are considered as oncologic emergencies, requiring a quick and appropriate response. Herein, we report the case of an 11-year-old boy, who visited our hospital with abdominal distension that started a month earlier. Read More

J Pediatr Hematol Oncol 2021 Mar 3. Epub 2021 Mar 3.

Department of Pediatrics Department of Pediatrics, Division of Genetics, Walter Reed National Military Medical Center Department of Patient Services, National Institutes of Health, Bethesda, MD.

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 Feb 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 May 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

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

World J Clin Cases 2020 Jun;8(11):2332-2338

Department of Surgical Oncology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310053, Zhejiang Province, China.

Background: Transcatheter arterial chemoembolization (TACE) is a common treatment for inoperable malignant renal tumors. However, a series of complications may follow the TACE treatment. Spinal cord injury caused by the embolization of intercostal or lumbar arteries is extremely rare. Read More

Pract Lab Med 2020 Aug 16;21:e00164. Epub 2020 May 16.

Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.

Objectives: To identify oncogenic driver mutations in congenital mesoblastic nephroma (CMN) cases lacking - fusion and discuss their diagnostic value.

Design: The institutional pathology database was queried for cases with a morphologic diagnosis of CMN. Cases positive for rearrangement or with unavailable blocks were excluded. Read More

Mod Pathol 2020 09 5;33(9):1669-1677. Epub 2020 May 5.

Department of Pathology, Hospital Bambino Gesu, Rome, Italy.

Until recently, undifferentiated round cell sarcomas (URCS) in infants have been considered a wastebasket diagnosis, composed of various pathologic entities and lacking consistent genetic alterations. The recent identification of recurrent BCOR internal tandem duplications (ITD) and less common alternative YWHAE-NUTM2B/E fusions in half of infantile URCS and the majority of so-called primitive myxoid mesenchymal tumors of infancy (PMMTI) suggests a common pathogenesis with clear cell sarcoma of the kidney which also harbors the same genetic alterations. These tumors also share a similar morphology and immunoprofile, including positivity for BCOR, cyclin D1, and SATB2. Read More

Int J Clin Exp Pathol 2020 1;13(4):771-777. Epub 2020 Apr 1.

Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College Bengbu, Anhui, P. R. China.

Background: Clear cell sarcoma of the kidney (CCSK) is a rare malignant tumor in children with uncertain histologic and immunohistologic traits. It mostly reveals atypical clinical symptoms similar to other familiar pediatric renal neoplasms, including abdominal mass, abdominal pain, hematuria, etc. Therefore, the lack of specificity in clinical symptoms may induce some challenging and controversial diagnoses. Read More

J Clin Pathol 2020 Jun 11;73(6):314-317. Epub 2020 Mar 11.

Anatomical Pathology, University of Cape Town, Cape Town, South Africa.

BCL-6 transcriptional corepressor () gene is located at Xp11.4 and encodes a protein which is involved in transcriptional repression in association with BCL-6 and epigenetic silencing through polycomb repressive complex 1 (PRC1). mutations are being identified in an increasing number of tumours which are diverse in their anatomical location and clinical setting. Read More

J Pediatr Hematol Oncol 2020 05;42(4):251-255

Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.

Background: Almost all pediatric patients with renal tumors are diagnosed with nephroblastoma (Wilms tumor), clear cell sarcoma, or malignant rhabdoid tumor. The choice of treatment is important for relapsed and refractory patients with nephroblastoma. Furthermore, clear cell sarcoma of the kidney (CCSK) and malignant rhabdoid tumor of the kidney (MRTK) have a poor prognosis compared with nephroblastoma. Read More

Res Rep Urol 2019 13;11:311-317. Epub 2019 Nov 13.

Oncology Practice, Hyatt Medical Center, Amman, Jordan.

Several case reports have provided most of the information available on perivascular epithelioid cell tumors, but with no consensus regarding diagnosis or treatment paradigms. Sunitinb is a VEGFR multitargeted tyrosine-kinase inhibitor that is regarded as a first-line treatment of clear cell-type metastatic renal-cell carcinoma after cytoreductive surgery. Our case was a 29-year-old male who presented with a metastatic left renal tumor and underwent left partial nephrectomy followed by adjuvant tyrosine kinase-inhibitor treatment. Read More

Pediatr Blood Cancer 2020 04 26;67(4):e28151. Epub 2019 Dec 26.

Section of Hematology/Oncology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.

Clear cell sarcoma of the kidney (CCSK) is the second most common malignant pediatric renal tumor. Two of the recurrent somatic alterations reported in CCSK are BCL-6 corepressor (BCOR) internal tandem duplication (ITD) and YWHAE-NUTM2B/E gene fusion. A minority of patients with CCSKs have other rare somatic alterations. Read More

Int J Surg Pathol 2020 Aug 26;28(5):477-481. Epub 2019 Dec 26.

Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan.

. 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. Read More

Anat Rec (Hoboken) 2020 10 18;303(10):2561-2577. Epub 2019 Nov 18.

Centre for Cancer Research, Hudson Institute of Medical Research, Clayton, Victoria, Australia.

Despite the rarity of renal tumors in children, many different types of malignant and nonmalignant renal neoplasms have been described. Therefore, the correct diagnosis and clinical management of these patients can represent a challenge. Here we provide a comprehensive review of the commonly diagnosed pediatric renal malignancies, including nephroblastoma (commonly known as Wilms tumor), clear cell sarcoma of the kidney, rhabdoid tumor of the kidney, several subtypes of renal cell tumors (often collectively termed renal cell carcinoma), and congenital mesoblastic nephroma. Read More

BMC Cancer 2019 Nov 1;19(1):1034. Epub 2019 Nov 1.

Department of Pathology, The First Hospital and College of Basic Medical Sciences, China Medical University, No. 155 Nanjing North Street, Shenyang, 110001, China.

Background: Clear cell sarcoma of the kidney (CCSK) is the second most common renal tumor in children following Wilms' tumor. CCSK is extremely rare in adults, with only 25 adult cases reported in the medical literature.

Case Presentation: We reported a 62-year-old man with a right renal mass presenting only with generalized pruritus who underwent radical right nephrectomy. Read More

Zhonghua Bing Li Xue Za Zhi 2019 10;48(10):799-801

Department of Pathology, Children's Hospital of Fudan University, Shanghai 201102, China.