Clear cell sarcoma of the kidney: An unusual presentation and review of the literature

Purpose: To describe a child with clear cell sarcoma of the kidney (CCSK) with an unusual presentation, including a primary tumor of the left kidney with metastases to the fight kidney and soft tissues of the lower extremities, and to review the literature. Patient and Methods: An 8-month- old infant presented with hypertension, an abdominal mass, and soft tissue masses in the left thigh and right foot. Imaging studies revealed a large left-sided renal tumor, left paravertebral soft tissue masses, and left thigh mass. At laparotomy, a lesion was noted in the lower pole of the contralateral kidney. CCSK with metastases to the contralateral kidney and to the soft tissues of left thigh, right foot, and left paravertebral region was diagnosed on histopathologic examination. Results: Multimodal oncologic treatment included surgery, chemotherapy, and radiotherapy. Three months after completion of therapy, a soft tissue lesion in the left arm and, later, soft tissue lesions involving multiple parts of the body developed. The patient died 18 months after diagnosis without clinical or radiographic evidence of bone involvement. Conclusions: In a review of the literature, CCSK is most commonly associated with bone and lung metastases. Soft tissue involvement is uncommon. Metastasis to the contralateral kidney at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of CCSK.