Department of Pathology, Mahatma Gandhi Medical College & Research Institute, Pillaiyarkuppam, Puducherry, India.
BMC Pediatr. 2010 Jul 6;10:47. doi: 10.1186/1471-2431-10-47.
We 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.
An 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.
The 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.
我们报告了一例男性儿童病例，其表现为黄色肉芽肿性肾盂肾炎（XGPN），伴有延伸至下腔静脉的肾静脉血栓形成。这是一种罕见的表现。XGPN 是一种罕见的肾脏感染类型，其特征为伴有巨细胞和泡沫状组织细胞的肉芽肿性炎症。发病高峰在第六至第七个十年，女性多见。XGPN 在儿童中很少见。
一名 11 岁男性儿童，因高热寒战、右侧腰痛和进行性脓尿病史 2 个月就诊。他有膀胱结石病史，4 年前接受了手术治疗。在我们的病例中，进行了右肾被膜下切除术。手术标本用福尔马林固定和石蜡包埋。切片用常规苏木精和伊红染色。大体上；肾脏增大，包膜粘连，肾周组织增厚。肾盂肾盏系统扩张，充满脓液铸型。组织学评估显示肾实质和肾周脂肪弥漫性坏死。可见中性粒细胞、浆细胞、泡沫状巨噬细胞片和偶尔的多核巨细胞。肾静脉部分被炎症性血栓阻塞，血栓由纤维蛋白、血小板和混合炎症细胞组成。血栓局部黏附于静脉壁并发生组织化。
临床表现和大体外观，以及组织学模式、细胞学特征，都提示诊断为 XGPN 合并下腔静脉血栓形成。我们的病例表明，即使在存在肾静脉和下腔静脉血栓形成的儿童年龄组中，也应考虑 XGPN 的诊断。
