Xanthogranulomatous Pyelonephritis in a male child with renal vein thrombus extending into the inferior vena cava: a Case Report

Abstract

BackgroundWe present a case of Xanthogranulomatous pyelonephritis (XGPN) in a male child with renal vein thrombus extending into the inferior vena cava. This is a rare presentation. XGPN is a rare type of renal infection characterised by granulomatous inflammation with giant cells and foamy histiocytes. The peak incidence is in the sixth to seventh decade with a female predominance. XGPN is rare in children.Case presentationAn 11 year old male child presented with a history of high grade fever and chills, right flank pain and progressive pyuria for two months. He had a history of vesical calculus for which he was operated four years back. In our case, a subcapsular right nephrectomy was performed. The surgical specimens were formalin fixed and paraffin embedded. The sections were stained with routine Hematoxylin & Eosin stain. Grossly; the kidney was enlarged with adherent capsule and thickening of the perinephric tissue. The pelvicalyceal system was dilated and was filled with a cast of pus. Histological evaluation revealed diffuse necrosis of the renal parenchyma and perinephric fat. Neutrophils, plasma cells, sheets of foamy macrophages and occasional multinucleate giant cells were seen. The renal vein was partially occluded by an inflammatory thrombus with fibrin, platelets and mixed inflammatory cells. The thrombus was focally adherent to the vein wall with organization.ConclusionsThe clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristics addressed the diagnosis towards XGPN with a vena caval thrombus. Our case illustrates that the diagnosis of XGPN should be considered even in paediatric age group when renal vein and vena caval thrombi are present.

Xanthogranulomatous pyelonephritis presenting as a pseudotumour in a 5-year-old boy

“…The exact aetiology of XGPN is unknown, but is generally accepted that the disease process is associated with long-term obstruction and infection 1 3 4 10 14–16. Other factors have also been implicated and include altered immune response and intrinsic disturbance of leucocyte function, alterations of lipid metabolism, lymphatic obstruction, malnutrition, arterial insufficiency and venous occlusion 4 10 16…”

Infectious Diseases and the Kidney in Children

“…A case series by Quinn et al showed an incidence of 6/1,000 surgically proven chronic pyelonephritis. Case reports have associated it with renal vein thrombi [212]. It presents with anorexia, weight loss, fever, chills, and dull, persistent flank pain.…”

Xanthogranulomatous pyelonephritis presenting as a pseudotumour

“…XGPN is often called "the great imitator.'' 14,15 Laboratory results show a microcytic anemia, leukocytosis, thrombocytosis, and increased C-reactive protein. 1,14 In our series, white blood cells and C-reactive protein rates are increased in one case (8.3%).…”

“…5,[11][12][13] Two forms of XGPN have been described, a diffuse form and a pseudo-tumoural (or focal) form, which is less frequent (15.4%). 1,14,15 The lower pole of the kidney is the most common location of focal XGPN. In our series, 50% of these lesions involved the lower pole.…”