Clear cell sarcoma of the kidney with inferior vena cava tumor thrombus in a pediatric patient: a case report.

backgroundClear cell sarcoma of the kidney (CCSK) is a rare and highly aggressive pediatric renal malignancy with a marked propensity for metastatic spread. Cases of CCSK associated with inferior vena cava (IVC) tumor thrombus (IVCTT) are exceptionally uncommon in the literature. We report a case of CCSK with IVCTT in a 15-month-old male infant. CASE DESCRIPTION: We reported a case admitted in May 2020 for a 3-day history of fever and hematuria. Abdominal CT revealed an unevenly enhanced mass and low-density shadows within the IVC. The diagnosis of CCSK was confirmed via needle biopsy. The patient received 4 cycles of adjuvant chemotherapy. The initial surgery lasted 10 h with 600 mL blood loss, and primary closure was achieved. Postoperative management included 6 cycles of radiotherapy and 5 cycles of chemotherapy. In March 2022, CT detected IVCTT recurrence, requiring surgical intervention involving thrombus removal and partial IVC resection. This procedure lasted 8 h with 300 mL blood loss, followed by 5 additional chemotherapy cycles. The patient showed no sign of IVC obstruction, including varicose veins or lower limb edema, and maintained renal function throughout follow-up. However, Intracranial metastases were detected 15 months postoperatively. After the family opted against additional treatment, the patient succumbed to the disease.

conclusionsThe management of CCSK associated with IVCTT should include consideration of IVC thrombectomy. In case of recurrent IVCTT with preserved collateral circulation, combined thrombus excision and partial IVC resection may be warranted.