2019-40 / NOVEMBER 25 (CONTRIBUTOR: LEVENTE KUTHI)
10-year-old girl with 15 cm renal mass and multiple pulmonary metastases. She received neoadjuvant chemotherapy according to the SIOP Umbrella Protocol followed by radical nephrectomy. The immunophenotype was as follows: diffusely positive for vimentin, INI1, CD99, focally positive for cyclin D1, PAX2, S100, and negative for EMA, WT1, AE1/AE3, TLE1, ERG, FLI1, CD56, BCL2, PAX8, desmin. FISH analysis was carried out using EWSR1/FLI1 TriCheck Probe, which identified an EWSR1 rearrangement, but the FLI1 was intact.
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1. What is the correct diagnosis?
a. Clear cell sarcoma of the kidney
b. Wilms’ tumor
c. Ewing sarcoma
d. Desmoplastic small round cell tumor
e. Synovial sarcoma