A Rare Case of Renal Sarcoma Mimicking Metastatic Osteosarcoma.

Pediatric renal sarcomas are exceedingly rare entities that present diagnostic challenges. We report a remarkable case of a 14-month-old female with an 8 cm right renal mass, accompanied by disseminated bone metastases, posing intricate clinical and histopathological dilemmas. Initial suspicion leaned towards clear cell sarcoma of the kidney (CCSK), however, subsequent histological examination post-chemotherapy revealed high-grade osteosarcoma and further differential considerations arose, including primary renal osteosarcoma and osseous osteosarcoma with secondary renal involvement.

Expanding the Spectrum of Sarcoma with an Internal Tandem Duplication of BCOR: A Non-Pediatric Nasosinusal Case.

Introduction: Undifferentiated small round-cell sarcomas with BCL6 corepressor (BCOR) alterations, such as an internal tandem duplication (ITD) within exon 15, are typically described as a pediatric group of Ewing-like small round-cell sarcomas.

Case Presentation: In contrast to this notion, we report the case of a 71-year-old woman with a nasosinusal sarcoma featuring a BCOR ITD. To the best of our knowledge, this presence had not been previously documented in a sarcoma of the nasal and sinus cavities in an elderly patient.

Cytokine Signaling in Pediatric Kidney Tumor Cell Lines WT-CLS1, WT-3ab and G-401.

Renal tumors comprise ~7% of all malignant pediatric tumors. Approximately 90% of pediatric kidney tumors comprise Wilms tumors, and the remaining 10% include clear cell sarcoma of the kidney, malignant rhabdoid tumor of the kidney, renal cell carcinoma and other rare renal tumors. Over the last 30 years, the role of cytokines and their receptors has been considerably investigated in both cancer progression and anti-cancer therapy.

Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2.

Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children's Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT.

18 F-FDG PET/CT in a Case of Clear Cell Sarcoma of the Kidney.

18 F-FDG PET/CT was performed in a 1-year-old girl who had a heterogeneous mass in the right abdominal cavity revealed by abdominal ultrasound. A heterogeneous mass with internal necrosis, cystic changes, and hemorrhage in the right kidney, accompanied by a slight increase of FDG uptake, was observed in FDG PET/CT. Malignant renal tumor was considered, and Wilms tumor was preferentially suspected.

CT radiomics to differentiate between Wilms tumor and clear cell sarcoma of the kidney in children.

Background: To investigate the role of CT radiomics in distinguishing Wilms tumor (WT) from clear cell sarcoma of the kidney (CCSK) in pediatric patients.

Methods: We retrospectively enrolled 83 cases of WT and 33 cases of CCSK. These cases were randomly stratified into a training set (n = 81) and a test set (n = 35).

[Renal tumors in children and adolescents].

Clinical/methodological Issue: Diagnosis and therapy of renal tumors in children and adolescents are standardized by study protocols from major international societies. Imaging plays a central role, and in Europe patients between the ages of 6 months and 14 years with renal tumors are referred to neoadjuvant chemotherapy without histological confirmation according to the study protocol due to the frequency of Wilms tumor.

Standard Radiologic Methods: Ultrasound is used worldwide as the primary investigative procedure for suspected renal tumors.

Clear cell sarcoma of kidney: A mimicker of Wilms' tumor.

The differential diagnosis for an abdominal mass in a 2-year-old child is broad and includes lesions of renal, hepatic, gastrointestinal, adrenal, and lymphatic origins. Of these, Wilms' tumor and neuroblastoma are the most common tumors, where Wilms' tumor represents about 92% of renal masses in children. Non-Wilms' renal tumors, rhabdoid tumors, and clear cell sarcoma of the kidney (CCSK) are uncommon.

A rare case of renal tumor in children: Clear cell sarcoma with an unusual presentation.

Clear cell sarcoma of the kidney is the most frequently misdiagnosed renal tumor in children. We report the case of a 6-year-old boy with clear cell sarcoma of the kidney with an unusual presentation, including a primary tumor of the left kidney with metastasis in the homolateral psoas muscle. The renal tumor was revealed by abdominal mass without hematuria.

A cystic non-Wilms renal tumor in a Nepalese boy: A rare case of clear cell sarcoma.

Introduction: Clear cell sarcoma of the kidney (CCSK) is an uncommon childhood malignancy known for its aggressive behavior and tendency to recur and metastasize to the brain and bones.

Case Presentation: We report a four-year-old boy evaluated for non-specific abdominal pain with an incidental radiological finding of a right lower pole cystic renal mass initially thought to be cystic Wilms' tumor. A pretherapy core biopsy of the mass suggested a clear cell tumor.

Sclerosing epithelioid fibrosarcoma of the kidney.

A woman in her 20s presented with headache and back pain and was found to have a left renal mass with bony metastases. She underwent nephrectomy, and on histopathology was initially diagnosed with stage 4 clear cell sarcoma of the kidney. She underwent palliative radiation and chemotherapy; however, the disease progressed and she came to our centre.

Tolerability of ifosfamide-containing regimen in patients with high-risk renal and INI-1-deficient tumors.

Background: Outcomes for children with high-risk renal (HRR) and INI-1-deficient (INI-) tumors are unacceptably poor. Concerns about excessive toxicity-as many are infants and/or undergo nephrectomy-have resulted in decreased chemotherapy dosing and omission of the nephrotoxic drug ifosfamide in collaborative group studies. As cause of death for children with these cancers remains overwhelmingly more from progressive disease rather than treatment toxicity, we examined the tolerability of an intensive ifosfamide-containing regimen.

Brain metastases with unusual MRI features in an infant with relapsed clear cell sarcoma of the kidney: A case report.

Clear cell sarcoma of the kidney (CCSK) is a rare primary renal tumor in children. It is known for its propensity to metastasize to bones and lungs at initial diagnosis. Distant metastatic relapses occur in about 15-16% of the patients, with the brain being the most frequent site of relapse.

Pediatric BCOR-Altered Tumors From Soft Tissue/Kidney Display Specific DNA Methylation Profiles.

In the pediatric population, BCL6-correpresor gene (BCOR)-upregulated tumors include primitive myxoid mesenchymal tumors/undifferentiated sarcomas (PMMTI/UND), clear cell sarcomas of the kidney (CCSK), and high-grade neuroepithelial tumors (HG-NET). We investigated DNA methylation (DNAm) and copy number variation (CNV) profiling in these tumors (N = 34) using an Illumina EPIC BeadChip to better define the potential use of these tools to confirm diagnosis and predict outcomes. Twenty-seven tumors from 25 patients (age range, 0-10 years), showed molecular confirmation of genetic abnormalities as follows: BCOR internal tandem duplication in 14 PMMTI/UND, 8 CCSK, and 3 HG-NET and YWHAE fusions in 2 PMMTI/UND.

Molecular Signature of Biological Aggressiveness in Clear Cell Sarcoma of the Kidney (CCSK).

Clear cell sarcoma of the kidney (CCSK) is a rare pediatric renal tumor with a worse prognosis than Wilms' tumor. Although recently, BCOR internal tandem duplication (ITD) has been found as a driver mutation in more than 80% of cases, a deep molecular characterization of this tumor is still lacking, as well as its correlation with the clinical course. The aim of this study was to investigate the differential molecular signature between metastatic and localized BCOR-ITD-positive CCSK at diagnosis.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.

Background: Peritoneal malignant tumors in children are rare but commonly associated with disease progression and poor outcome. The successful treatment experience of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in adult peritoneal carcinoma has been applied to pediatric peritoneal malignancy in recent years. However, patients with desmoplastic small round cell tumor (DSRCT) accounted for the majority of patients treated with CRS and HIPEC in previous studies.

Clear-Cell Sarcoma With an Unusual Presentation Mimicking Metastatic Melanoma.

Clear-cell sarcoma (CCS) was first described in 1968. It partly overlaps morphologically, immunohistochemically, and ultrastructurally with malignant melanoma (MM), hence its name "soft tissue melanoma." Nevertheless, there are sufficient cytogenetic differences between cutaneous melanoma and clear-cell sarcoma to consider clear-cell sarcoma as a separate entity.

Clinical relevance of BCOR internal tandem duplication and TP53 aberration in clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) is the second most common pediatric renal malignancy, characterized by BCOR internal tandem duplication (ITD), YWHAE rearrangement, BCOR-CCNB3 fusion, and lack of other consistent structural alteration. We accidentally identified TP53 deletion in CCSK, which was often associated with adverse clinical outcomes. In this study, we assessed the incidence as well as the clinical relevance of these molecules in CCSK patients.