Reach Us +44-7426-607635

Xanthogranulomatous Pyelonephritis (XGPN) Presenting as Renal Lump in Infant: Rare Presentation

Rahul Ghule*, Ram Daga, Jaydeep Date, Sujit Joshi and Subodh Shivde

Deenanath Mangeshkar Hospital and Research Centre, Pune, Maharshtra, India

*Corresponding Author:
Rahul Ghule
Deenanath Mangeshkar Hospital and Research Centre, Pune, Maharshtra, India
Tel: +918308804098
E-mail: [email protected]

Received date: October 22, 2015, Accepted date: November 11, 2015, Published date: November 16, 2015

Citation: Ghule R, Daga R, et al. Xanthogranulomatous Pyelonephritis (XGPN) Presenting as Renal Lump in Infant: Rare Presentation. J Rare Dis Diagn Ther. 2015, 1:3. doi:10.21767/2380-7245.100025

Copyright: © 2015 Ghule R, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

 
Visit for more related articles at Journal of Rare Disorders: Diagnosis & Therapy

Abstract

Background: Xanthogranulomatous pyelonephritis (XGPN) is an unusual suppurative granulomatous reaction to chronic renal infection. It usually presents with symptoms of infections. It is rare entity especially in childhood. Case: We are reporting case of xanthogranulomatous pyelonephritis in 2 month male baby presented as left renal mass without signs of infections. On imaging it shows multicystic, heterogeneous, non-enhancing left renal mass.
Conclusion: XGPN, itself is a rare entity presenting in infant/childhood of which presentation solely as renal lump without any signs and symptoms of urosepsis, UTI. Hence even in neonate/childhood one should consider it as one of D/D presenting as renal mass.

Keywords

Xanthogranulomatous pyelonephritis (XGPN); Renal lump; Urosepsis

Introduction

Xanthogranulomatous pyelonephritis (XGPN) is rare variant of chronic pyelonephritis. The most common associated factors are urinary tract obstruction and infection. It is presumed to be related to chronic bacterial infection, renal or lymphatic obstruction, alterations in lipid metabolism or an ineffective immune response to infection, nephrolithiasis [1]. It rarely appears in childhood. A very few cases in neonatal period have been reported in literature so far. It is important to recognize this rare entity in children because it may be confused with childhood renal malignancies particularly Wilm’s tumors [1].

Case

2 months old male baby presented with lump in left lumbar region, denied history of fever, weight loss and urinary infection. Haemogram was normal, no leukocytosis, occasional pus cells in urine analysis, no growth on urine culture. On examination, nutrition was good, per abdominal large lump palpable in left lumbar, iliac, extending to umbilical region. Systemic examination was normal. Ultrasound suggestive of large left renal mass with cystic/solid component, contrasts enhanced CT scan suggestive of multicystic, heterogeneous; non-enhancing left renal mass. Since a differential of renal malignancy was considered, patient underwent left nephrectomy. Histopathology was suggestive of xanthogranulomatous pyelonephritis. After 2 years, baby had multiple admissions for urinary infection. On evaluation, voiding cystourethrogram s/o vesicoureteric reflux on left side with dilation remaining ureteral stump (Figure 1-3).

raredisorders-coronal-veiw

Figure 1 CECT (coronal veiw ).

raredisorders-Cut-section-specimen

Figure 2 Cut section of specimen.

raredisorders-Cut-Histopathology

Figure 3 Cut Histopathology of XGPN. section of specimen.

Discussion

XGPN is an atypical form of severe chronic renal parenchymal infection [2]. The first descriptions of the distinctive gross and macroscopic features of this disease were made by Schlagenhaufen in 1916 [3-5], but it was Oberlin who introduced the term XGPN in 1935 [5]. In 1978, Malek and Elder classified and staged the disease [6]. The first pediatric case was described in 1963 by Avnet and Friedenberg [5,7,8]. It is an uncommon entity, accounting for between 0.6% of histologically documented cases of chronic pyelonephritis [9]. A review of recently published data indicates that about 80 cases of XGPN have been reported in pediatric age [10].The earliest age of presentation reported so far ranges from 21 days in English literature by Youngson GG [11], 48 days in Japanese literature by YazakiT [12], 40 days old by Anand Upasani et al [13]. We are reporting case of 60 days (2 month) male baby.

XGPN is classified into three stages: stage I - the lesion is confined to the kidney (nephric), stage II - when it extends to Gerota’s space (perinephric) and stage III - the extension to the perinephric space or retroperitoneal structures (paranephric) [14]. Both diffuse and focal forms of the disease have been reported, with the diffuse form being more common [14]. In children, the disease most frequently occur below 8 years of age with male predominance affecting left kidney predominantly [10]. In adults, females are affected more than males and right kidney has been noted to be involved more often than the left [10]. Presentation is varied from sepsis to asymptomatic renal lump but most of these children present with symptoms of chronic infection, like recurrent fever, weight loss, pallor and failure to thrive etc. Although bacterial culture from urine reveals E. coli, Proteus as the most common offending organism [2,15], but in our case it was sterile. Radiological features are not specific and mimic neoplasia. It is important to consider XGPN in order to avoid needless preoperative irradiation of a large abdominal mass, extensive lymph node dissection, or administration of myelosuppressive agents at the time of operation [16].

The present case is unique in way that, 2 month old male baby presented with left renal mass without any sign and symptoms of urosepsis, UTI. Urine was normal, there was no leukocytosis, radiology (ultrasound/CECT) suggestive of left renal mass without any peculiar feature of particular pathology and hence the presumptive diagnosis of renal neoplasm was made. In view of renal neoplasm, left radical nephrectomy was done. Final diagnosis was settled on histopathology as XGPN. After extensive search of literature, till now there are only 8-10 cases reported as XGPN presenting as renal mass or pseudotumour in infants.

Summary

XGPN is a relatively rare entity especially in infant. As it can be presented as renal lump without signs of infection, it should be included in the differential diagnosis of children with a renal mass. As the prognosis in adequately treated XGP is quite good, a clinical awareness and a high index of suspicion are required to achieve the correct preoperative diagnosis and appropriate management.

References

Select your language of interest to view the total content in your interested language

Viewing options

Post your comment

Share This Article

Flyer image
 

Post your comment

captcha   Reload  Can't read the image? click here to refresh