Renal clear cell sarcoma in the pediatric age: differential diagnosis and management

Abstract

Renal clear cell sarcoma is the second most frequent malignant renal tumor in pediatric age, after Wilms' tumor. It is a difficult to diagnose neoplasm, with nonspecific clinical presentation as palpable abdominal mass, abdominal pain and hypertension and hematuria. The imaging characteristics of this lesion are non-specific, so its diagnosis is made by anatomopathological study of the tumor. Its importance revolves around the diagnostic difficulty, since this tumor presents great histologic variability and few adequate immunohistochemical markers. Treatment includes neoadjuvant and post-surgical chemotherapy, with a prognosis that improves if the disease is diagnosed early.