A child with sickle cell trait and macroscopic hematuria: answer

Abstract

The differential diagnosis of macroscopic hematuria in patients with sickle cell disease (hgb SS), and more rarely in sickle cell trait (hgb AS), includes papillary necrosis, renal infarction, membranoproliferative glomerulonephritis associated with the hepatitis C virus, interstitial nephritis due to analgesic abuse, pyelonephritis, nephrolithiasis and, very rarely, a renal malignancy, such as renal medullary carcinoma. Acute papillary necrosis is often suspected and is frequently the cause, but further studies are mandatory if the hematuria persists. In this case, the renal ultrasound (Fig. 1) demonstrated an echogenic mass in the upper pole of the left kidney. The computed tomography (CT) scan with intravenous contrast (Fig. 2) revealed a heterogeneous mass in the same location. A tissue diagnosis was required to confirm the diagnosis, and the patient underwent unilateral nephrectomy. Figure 3 shows the gross pathology of the tumor found in the upper pole of the left kidney. The pathologic diagnosis was renal cell carcinoma, papillary cell type, collecting-duct sub-type. Figure 4 shows the microscopic pathology. Our patient had no evidence of metastases and appears to be completely well after 6 months of follow-up.