Diagnostic MRI characteristics of pediatric clear cell sarcoma of the kidney and rhabdoid tumor of the kidney: A retrospective multi-center SIOP-RTSG Radiology panel study

• DOI: 10.1016/j.ejcped.2023.100122
• Publication Date: 2023-11-04T03:55:51Z
• Authors: Justine N. van de...; Jens-Peter Schenk; Tom A. Watson; Ana Coma; Carlo Morosi; Norbert Graf; Tanzina Chowdhury; Gema L. Ramírez-V...; Filippo Spreafico; Kristina Dzhuma; Lidwine B. Mokkink; Ronald R. de Krijger; Marry M. van den ...; Annemieke S. Litt...
• Keywords: Wilms tumor; Neoplasms. Tumors. Oncology. Including cancer and carcinogens; Pediatrics; 03 medical and health sciences; Magnetic resonance imaging; 0302 clinical medicine; Oncology; Dentistry (miscellaneous); Rhabdoid tumor; Kidney neoplasms; Radiology; Clear cell sarcoma; RC254-282

IntroductionClear cell sarcoma of the kidney (CCSK) and rhabdoid tumor (RTK) are rare malignant pediatric renal tumors, both accounting for 1-5% diagnoses. Within International Society Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG) protocols diagnostic invasive procedures to determine histology discouraged. MRI has become preferred imaging modality, however, non-invasive discrimination CCSK RTK remains challenging. Therefore, this study aims identify MRI-characteristics RTK, in largest series patients date.Material MethodsFive SIOP-RTSG national review radiologists identified MRIs histologically proven CCSKs RTKs. Scan-protocols were based on MRI-guidelines following protocols. Radiologists assessed their cases using a validated case report form (CRF).ResultsRetrospectively, 59 (n=38 CCSK, n=21 RTK). showed high median volume (576 cm3, range 54-4414), which was lower RTKs (290 20-761) (p=0.006*10-3). Fifty-two percent (11/21) RTK-patients disease at other locations, predominantly lungs brain, compared 8% CCSK-patients (3/38). ill-defined margins (12/21, 57%) infiltrative growth pattern (13/21, 62%). appeared hyper-intense T2-weighted (27/38, 71%) with characteristics band-like enhancement (13/38, 34%), whereas often hypo-intensity (11/21, 52%). The overall mean ADC-value 1.11*10-3mm2/s (range 0.57-2.07*10-3 mm2/s), 0.72*10-3mm2/s 0.53-0.90*10-3 mm2/s) RTK.ConclusionsThis retrospective suggests small size, an aggressive may be characteristic RTK. typical relatively ADC-values. Identified used future studies focusing validation from especially WT, aiming early diagnosis.