XANTHOGRANULOMATOUS PYELONEPHRITIS: radiologic

review.

Poster No.: C-0557

Congress: ECR 2014
Type: Educational Exhibit
Authors: 1 2 1
M. Barral , J. M. Sánchez Crespo , J. C. Pérez Herrera , J. L.
3 1 1
Ortega Garcia , F. J. Hidalgo Ramos , G. Porcuna Cazalla ;
1 2 3
Puerto Real/ES, Vejer de la Frontera, Cádiz/ES, Jerez de la
Frontera/ES
Keywords: Infection, Calcifications / Calculi, Diagnostic procedure,
Ultrasound, CT, Conventional radiography, Kidney, Abdomen
DOI: 10.1594/ecr2014/C-0557

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Learning objectives

- To illustrate radiological findings of xanthogranulomatous pyelonephritis (XP) on

different imaging techniques.

- To learn when to include XP as a diagnostic possibility, according to the clinical context

and the radiological features.

Background

Xanthogranulomatous pyelonephritis is an uncommon chronic suppurative renal infection

characterized by destruction of renal parenchyma and replacement with lipid-containing
macrophages (fig. 1).

Two forms of XP are well known, a diffuse form (85%) and a focal form (15%), the latter
also known as "tumefactive" form.

th th
It predominantly affects adults in the 5 through 7 decades of life and female gender
is more frequently affected.

Typically, there is a unilateral and diffuse affectation of the kidney and extension to the
perirenal spaces is common. Most patients have nephrolithiasis, and staghorn calculi are
found in approximately one-half of patients.

Patients are usually symptomatic, but with non-specific clinical manifestation; most of
them have recurrent low-grade fever, malaise, flank pain, hematuria and prior urinary
tract infection (fig. 2).

Laboratory studies show an elevated erythrocyte sedimentation rate, anemia, and

leukocytosis.

More than 80% of patients with XP have pyuria and over 60%, positive urine cultures,
being P. mirabilis and E. coli the most common organisms implicated. The percentage
of sterile urine cultures found is explained by the complete obstruction of the kidney and
bladder seen in some cases.

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An early diagnosis of this pathology brings excellent results after surgery.

Images for this section:

Fig. 1: Microscopic features of xanthogranulomatous pyelonephritis. Original (a) and

greater (b) magnification show lipid-laden macrophages (blue arrow) intermingled with
leukocytes and plasma cells (yellow arrow) in the renal medulla. Red arrow shows a
glomerulus in a thinned renal cortex.

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Fig. 2: Frequent clinical findings of XP.

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Findings and procedure details

Imaging findings

# Diffuse Xanthogranulomatous Pyelonephritis

CT technique deserves a special attention because it will be very helpful for diagnosis and
surgical planning on this form of XP. It frequently demonstrates characteristic features
and depicts extension of the inflammatory process beyond the kidney.

In the other hand, abdominal radiography, intravenous urography and ultrasound images
will show less conclusive features.

• Abdominal radiography

The radiographic finding of a large staghorn calculus is present in most cases, but this
feature by itself is nonspecific. Additional findings include enlargement of the affected
kidney and, if extrarenal extension exists, obscuration of the ipsilateral psoas muscle (fig.
3, 4, 9).

• Excretory urography

An important decrease in renal function on the pathological side is observed, with a delay
on the contrast material excretion or with no excretion (fig. 3).

• Ultrasound

This technique demonstrates an enlarged kidney, with multiple fluid collections replacing
the normal corticomedullary differentiation (fig. 6). These collections are either anechoic
or hypoechoic areas and correspond to dilated calyces and areas of parenchymal
destruction.

An echogenic pelvis from a central staghorn calculus may be seen.

Perirenal extension is observed as hypoechoic fluid masses.

• CT

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Typical findings are diffuse enlargement of kidney, replacement of renal parenchyma by
multiple low-attenuation rounded masses (dilated calyces or focal areas of parenchyma
destruction) and a central calcification within a contracted renal pelvis (fig. 4, 7, 8, 9).
Extrarenal extension may be seen, involving the perirenal space, pararenal spaces,
ipsilateral psoas muscle, posterior abdominal wall, skin or bowel (fig. 5, 7).

Less common CT features are absence of calculi (up to 10% of cases), important pelvic
dilatation or renal atrophy.

• MR

This technique does not give any advantage compare to CT. Features are similar to the
ones described on CT paragraph; enlarged kidney, replacement of renal parenchyma
by abscess cavities with intermediate signal intensity on T1-weighted images and high
signal intensity on T2-weighted images. Calculi are better depicted with CT but may be
seen at MR imaging as areas of signal void within the renal pelvis (fig. 10).

# Focal Xanthogranulomatous Pyelonephritis

This infrequent form of XP is confined to a part or pole of the kidney. Findings may
not be similar to the ones described for diffuse XP and no single radiological sign is
pathognomonic.

The definitive diagnosis of focal XP is most often given after histologic examination of
the surgical specimen.

• Ultrasound

Ultrasonographic features of focal XP and those of renal tumors or abscesses are

extensive overlap.

The focal disease may be seen as a hypoechoic mass with an associated calculus.

• CT

It is the imaging modality of choice.

A focal intrarenal mass with fluidlike attenuation and rim enhancement is seen, often with
an associated calculus (fig. 11). Extension to extrarenal spaces is possible as well.

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Differential diagnosis

CT examination demonstrates highly specific intrarenal findings in the majority of cases

of diffuse form of XP.

Differentiating XP from other types of renal infection or a renal neoplasm becomes very
difficult when atypical findings are seen or in case of focal form of XP.

Differential diagnosis for diffuse form of XP must include pyonephrosis (fig. 8) and
hydronephrosis.

Imaging findings of focal XP may imitate those of neoplastic diseases (such as renal cell
carcinoma, lymphoma, or leukemia in adults and Wilm tumor in children) and other focal
inflammatory renal parenchymal diseases (such as renal tuberculosis, renal abscess,
and malakoplakia).

Treatment and prognosis

Treatment consists on total nephrectomy for diffuse XP.

The treatment of focal XP is controversial. In selected cases, partial nephrectomy has

been tried. There are also reports of a few patients who recovered from the disease after
antibiotic therapy.

The prognosis is excellent, and XP does not recur after surgery.

Images for this section:

Page 7 of 17
Fig. 3: 43-year-old woman with recurrent right flank pain. Abdominal radiography (a)
demonstrates a large staghorn calculus in the left kidney. On excretory urogram (b) and
delayed phase (c), nonfunction of the left kidney is demonstrated.

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Fig. 4: 37-year-old man with a 2-weeks history of progressive right flank pain, malaise
and high-grade fever. On abdominal radiography, a large staghorn calculus in the right
kidney is observed without being able to delimitate its upper pole profile, neither the left
kidney profile. CT scan with contrast material in venous (b) and excretory (c) phases
show a horseshoe kidney with typical features of xanthogranulomatous pyelonephritis; a
diffuse enlargement of the right kidney, a replacement of renal parenchyma by multiple
low-attenuation rounded masses and a central calcification. On the excretory phase, no
contrast material excretion is demonstrated on the right kidney, being excretion on the
left kidney normal (white arrow).

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Fig. 5: CT scan of the same patient of figure 4 reveals posterior perirenal (green arrow)
and pararenal (white arrow) extension of the inflammatory process.

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Fig. 6: 38-year-old woman with left flank pain, fever and altered renal function. Ultrasound
technique of the right kidney (a) is normal. Left kidney (b, c) is enlarged, with some fluid
collections replacing the normal corticomedullary differentiation and with an echogenic
pelvis associated to a posterior acoustic shadowing from a central staghorn calculus.

Page 11 of 17
Fig. 7: Same patient as in figure 6. CT scan confirms typical features of diffuse XP in
the left kidney. Extrarenal affection is seen as well on (d, e) with a fluid collection in the
perirenal space (green arrow) and infiltration of the lumbar quadrate muscle (blue arrow).

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Fig. 8: 59-year-old woman with right flank pain and high-grade fever. On CT scan (a-c),
right kidney is enlarged, with a staghorn calculus (specially in the lower pole of the kidney)
and multiple fluid collections replacing the normal parenchyma. Left kidney has a normal
size, shows a staghorn calculus and fluid collections as well. The diagnosis of suspicious
was bilateral xanthogranulomatous pyelonephritis, obtaining after right nephrectomy an
histological result of XP complicated by pyonephrosis.

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Fig. 9: Same patient as in figure 8 after three years. Abdominal radiography (a, b)
demonstrates changes secondary to right nephrectomy and staghorn calculus on the left
kidney. CT scan (c, d) confirms same features, and shows the presence of multiple fluid
collections replacing the normal corticomedullary differentiation.

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Fig. 10: Same patient as in figure 8 and 9. MRI; T2-weighted images on axial (a-c) and
coronal (d-g) planes. Replacement of renal parenchyma by abscess cavities with high
signal intensity on T2-weighted images. Calculi are seen as areas of signal void within
the renal pelvis and calyces.

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Fig. 11: 62-year-old woman with right flank pain. CT scan shows a mass with areas of
fluidlike with an associated calculus located in the upper pole of the right kidney. The
diagnosis of suspicious was renal cell carcinoma. After nephrectomy, histological result
was focal xanthogranulomatous pyelonephritis.

Page 16 of 17
Conclusion

- CT is the gold standard technique in the diagnosis of XP for two reasons: it demonstrates
highly specific intrarenal findings in the majority of cases (diffuse XP) and shows
extrarenal extension, useful for surgical planning.

- It will be difficult to differentiate XP from other types of renal infection or a renal neoplasm
when atypical findings are seen or in case of focal form of XP.

Personal information

mariabare2001@hotmail.com

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