Primary epithelioid hemangioendothelioma of the kidney: the first case report in a child and literature review.

Epithelioid hemangioendothelioma is a distinct vascular tumor with low malignant biologic behavior that is very rare in the kidney. Only 3 adult patients have been reported to date in English-language reports. We report the case of a 9-year-old boy who presented with painless gross hematuria. Magnetic resonance imaging and positron emission tomography-computed tomography demonstrated a primary mass in the left kidney. The pathologic diagnosis of the tumor was epithelioid hemangioendothelioma after radical nephrectomy. To the best of our knowledge, this is the first reported case of primary renal epithelioid hemangioendothelioma in the pediatric age group.