Benign cyst or malignancy? A six-year-old with primitive small round cell sarcoma of the right buttock: a case report

Michelle L. Amit, Lori Barr, Erlyn Smith


A six-year-old male presents with mass on his right buttock. After surgical resection, he was found to have a primitive small round cell sarcoma, high grade with CIC-DUX4 fusion gene. Treatment started with compressed regimen of VDC/IE (compressed Ewing protocol). There was initial improvement in visual appearance of the buttock/wound site but recurrence of the tumor occurred despite extensive surgical resection along with metastases to the lungs and bone which progressed and eventually led to death. This case adds to the collective knowledge regarding the CIC-DUX4 type of pediatric neoplasms and provides insight for future chemotherapy protocols.


Primitive small round cell sarcoma, Pediatric sarcoma, Gluteal mass, Pelvis mass, CIC-DUX4, Metastasis

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