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

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
DOI: 10.1016/j.ejcped.2023.100122 Publication Date: 2023-11-04T03:55:51Z
ABSTRACT
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.
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