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Pictorial Essay
Differential Diagnosis of Perinephric Masses
on CT and MRI
Antonio Westphalen1, Benjamin Yeh, Aliya Qayyum, Anil Hari, Fergus V. Coakley

asses in the perinephric space, including the peripelvic space of


the renal hilum, may be due to tumor, fluid, inflammation, or various proliferative diseases. More broadly, abnormal
tissue at the periphery of the kidney may be
subcapsular or perinephric in origin, and
these processes are not always distinguishable, so the differential diagnosis for perinephric masses includes subcapsular disease.

Radiologic evaluation of renal and perinephric masses is increasingly important; for


example, more than half of renal cell carcinomas are discovered incidentally during imaging performed for unrelated reasons [1].
The objective of this pictorial essay is to provide a timely review of those diseases that
may result in perinephric or subcapsular
masses, with an emphasis on key clinical or
radiologic differentiating features.

Fig. 1.58-year-old man presenting with gross hematuria and left flank pain. Contrastenhanced axial portal phase CT image shows large heterogeneous and necrotic renal
cell carcinoma (asterisk) invading perinephric space. Significant thickening of renal fascia also is seen.

Tumors
Renal Cell Carcinoma

Although renal cell carcinoma is common,


a perinephric pattern of spread is seen only occasionally, typically in bulky tumors (Fig. 1).
Lymphoma

Primary renal lymphoma is rare, and renal


lymphoma usually is seen in widespread dis-

Fig. 2.63-year-old man with non-Hodgkins lymphoma. Contrast-enhanced axial CT


image shows bilateral perinephric (arrows) and right peripelvic (asterisk) masses.

Received October 10, 2003; accepted after revision June 3, 2004.


1

All authors: Department of Radiology, University of CaliforniaSan Francisco, 505 Parnassus Ave., Rm. M-372, Box 0628, San Francisco, CA 94143-0628. Address correspondence to F. V. Coakley.

AJR 2004;183:16971702 0361803X/04/18361697 American Roentgen Ray Society

AJR:183, December 2004

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Westphalen et al.

Fig. 3.43-year-old male renal transplant recipient with biopsy-confirmed posttransplantation lymphoproliferative disorder in hilum of transplanted kidney.
A, Axial T1-weighted image shows hypointense mass (arrow) in hilum of allograft. Note small nonspecific pocket of fluid (asterisk) adjacent to allograft.
B, Axial T2-weighted image obtained at same level as A shows same lesion (arrow) as predominantly hypointense. Small nonspecific pocket of fluid (asterisk) adjacent to
allograft is also seen on this image.
C, Gadolinium-enhanced axial gradient-echo T1-weighted image obtained with fat suppression at same level as A and B shows minimal enhancement in lesion (arrow)
and fluid pocket (asterisk).

ease. Renal involvement is found in 1550%


of patients with lymphoma at autopsy but in
only 38% of patients at CT staging [2, 3].
Perinephric lymphoma is one of the described patterns of renal involvement and
typically is homogeneous, hypovascular,
mildly enhancing, and associated with non-

obstructive encasement of retroperitoneal


vessels [3] (Fig. 2).
Posttransplantation Lymphoproliferative Disorder

Posttransplantation lymphoproliferative disorder is a lymphomalike condition associated


with Epstein-Barr virus infection that occurs

Fig. 4.76-year-old man with history of malignant melanoma. Contrast-enhanced


axial CT image of abdomen shows nodular left perinephric metastasis (arrow). Diagnosis was confirmed by concordant progression of perinephric lesion and widespread metastases elsewhere.

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as a complication in 2% of the recipients of


solid-organ transplants. In kidney transplant
recipients, posttransplantation lymphoproliferative disorder has a predilection for occurring
in the renal hilum and typically is hypointense
on both T1- and T2-weighted images, with
minimal contrast enhancement [4] (Fig. 3).

Fig. 5.77-year-old woman with right upper quadrant pain. Contrast-enhanced axial portal phase CT image shows large heterogeneous mass (arrows), predominantly composed of fat in right upper quadrant, invading perinephric space and
renal hilum. Surgical resection established diagnosis of myxoid liposarcoma.

AJR:183, December 2004

CT and MRI Diagnosis of Perinephric Masses

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Fig. 6.67-year-old woman with angiomyolipoma of left kidney presenting as acute


left flank pain. Unenhanced axial CT image shows perirenal hematoma (arrow) originating from ruptured angiomyolipoma (asterisk).

Metastases

Fluid

Metastases to the kidney are common


findings at autopsy, typically in the setting of
widely disseminated disease, but rarely are
recognized on imaging [3, 5] (Fig. 4). In a
study performed at Georgetown University
Medical Center, perinephric spread was seen
in six of 27 patients with radiologically evident metastases to the kidney [5].

Hematomas

Retroperitoneal Tumors

Retroperitoneal tumorsparticularly sarcomas (Fig. 5), multiple myelomas, Castlemans disease tumors, and bulky primary
renal tumorsmay involve the perinephric
space by direct contiguous extension [2].

Described causes of spontaneous (nontraumatic) perinephric hematomas include angiomyolipoma, renal cell carcinoma, polycystic
kidney disease, and bleeding diathesis [2, 6].
The underlying tumor may be identifiable on
imaging, even in cases of acute bleeding (Fig.
6). Retroperitoneal leakage from a ruptured
abdominal aortic aneurysm also may result in
perinephric hematoma.

stone usually suggests the correct diagnosis.


Delayed CT scans may show extravasation of
excreted contrast material [2, 6] (Fig. 7).
Abscesses

A perinephric abscess may be the result of


urinary tract infection or may arise from infection of a preexisting perinephric hematoma or
urinoma. Clinical and radiologic clues include a
history of diabetes mellitus or immunosuppression therapy, clinical manifestations of sepsis,
thick enhancing wall, and intralesional gas [2, 6].

Urinomas
Lymphangiomatosis

Perinephric urinomas may be due to an obstructive forniceal rupture or trauma. The history of trauma or the finding of an obstructing

Renal lymphangiomatosis is a rare benign


malformation of the perinephric lymphatic

Fig. 7.67-year-old man with flank pain after aortic


aneurysm repair.
A, Contrast-enhanced axial arterial phase CT image
shows fluid collection in left perirenal space (arrow)
and adjacent to aorta. Appearance of aorta is due to
tortuosity and postoperative changes. Perinephric hematoma (asterisk) also is evident.
B, Contrast-enhanced axial delayed CT image obtained at same level as A shows excreted contrast
material passing from left renal pelvis (arrow) into
perinephric fluid collection (asterisk), confirming diagnosis of urinoma.

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Westphalen et al.

B
Fig. 8.Pregnant 31-year-old woman with hereditary lymphangiomatosis. Diagnosis was confirmed by aspiration of chyle from perinephric cystic lesions.
A, Longitudinal sonogram shows cystic structures around right renal pelvis (asterisk) and kidney (arrow).
B, Axial fast spin-echo T2-weighted image shows multiple bilateral perirenal (arrows) and peripelvic (asterisks) cystic structures.
C, Coronal single-shot fast spin-echo T2-weighted image shows same perirenal (arrows) and peripelvic (asterisks) cysts seen in B.

Fig. 9.51-year-old woman with xanthogranulomatous pyelonephritis and history


of staghorn calculus and chronic urinary tract infection. Axial late arterial phase
contrast-enhanced CT image shows enlarged right kidney with abscesslike lowdensity cavity (asterisk) in renal hilum. Staghorn calculus is noted (arrowheads)
and was also seen on unenhanced images (not shown). Perirenal space extension
of inflammatory process (arrow) also is visible.

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Fig. 10.68-year-old man with extramedullary hematopoiesis due to myelofibrosis.


Contrast-enhanced axial CT image obtained during portal venous phase shows
large hypodense, hypovascular mass encasing right renal hilum, distorting collecting system (asterisk). Lesion (arrows) is also seen in perirenal space bilaterally.
Note preservation of renal contours. Appearance is nonspecific. Diagnosis was established by biopsy.

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CT and MRI Diagnosis of Perinephric Masses

Fig. 11.51-year-old man with retroperitoneal fibrosis. Contrast-enhanced axial


delayed CT image shows hypodense, nonenhancing mass extending into right renal
hilum (white arrow) with associated hydronephrosis (asterisk) and renal atrophy.
Subtle extension of retroperitoneal process anterior to left kidney (black arrow)
also is present. Appearance is nonspecific, and differential includes malignancy
such as lymphoma. Diagnosis was confirmed by biopsy.

system resulting in characteristic unilocular


or multilocular thin-walled perinephric cysts.
Pregnancy may exacerbate the condition
(Fig. 8). The diagnosis can be confirmed by
the aspiration of chyle [6].
Inflammation
Xanthogranulomatous Pyelonephritis

Xanthogranulomatous pyelonephritis is a
rare inflammatory and locally aggressive tumorlike renal mass that usually arises in the
setting of renal stones or chronic infection

Fig. 12.24-year-old woman with 2-year history of Rosai-Dorfman disease. Contrastenhanced axial delayed CT image shows that left renal hilum is encased by large lobulated hypodense and hypovascular mass and that pelvicaliceal system (asterisk) is
compressed. Appearance is nonspecific. Diagnosis was established by biopsy.

by Proteus, Escherichia coli, or Pseudomonas bacteria. The histopathologic finding of


lipid-laden foamy macrophages is diagnostic for this condition. The typical imaging
appearances are of an ill-defined renal mass
with some combination of stones, hydronephrosis, parenchymal renal abscesses, and
perinephric extension [2] (Fig. 9).
Pancreatitis

Fluid dissecting between fascial planes in


the perinephric space is seen in patients with

pancreatitis. The left or right kidney may be


affected, depending on whether the inflammatory fluid tracks from the tail or the head
and neck of the pancreas, respectively [6].
Proliferative Diseases
Extramedullary Hematopoiesis

Extramedullary hematopoiesis may occur


in chronic anemia, blood dyscrasias such as
leukemia, and replacement of the normal bone
marrow by tumor or bone overgrowth. Renal

Fig. 13.48-year-old man with Erdheim-Chester disease presenting with flank and extremity pain.
A, Contrast-enhanced axial CT image shows left perinephric hypovascular mass (arrow) associated with fat stranding. Note moderate hydronephrosis (asterisk). Appearance is nonspecific.
B, Conventional radiograph of right and left femurs shows typical cortical and medullary sclerosis. Findings are bilateral and symmetric. Erdheim-Chester disease was
raised as possible diagnosis in view of femoral abnormalities and was confirmed by biopsy of perinephric tissue.

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Westphalen et al.

Fig. 14.33-year-old woman with acute renal failure from cortical necrosis after
severe antepartum hemorrhage. Contrast-enhanced axial CT image shows lack of
enhancement of renal cortex (arrow) with normal renal medulla enhancement.
Note slight enhancement of renal capsule (arrowheads).

extramedullary hematopoiesis may be perinephric (Fig. 10). It is typically hypodense on


CT and hypointense on T1-weighted and
mildly hyperintense on T2-weighted imaging.
The patients clinical history may indicate the
correct diagnosis.
Retroperitoneal Fibrosis

Retroperitoneal fibrosis usually is idiopathic. The typical appearance is of a hypodense mass on CT or a hypointense mass on
T1- and T2-weighted imaging. The mass infiltrates the retroperitoneum and encases the major vessels. Vascular occlusion is uncommon,
although obstruction of the inferior vena cava
is a recognized complication. Contrast enhancement is minimal and is more apparent on
delayed images. Perinephric involvement is
rarely seen in isolation, so the diagnosis is usually straightforward [2] (Fig. 11).
Rosai-Dorfman Disease

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a benign systemic histiocytic proliferative
disorder presenting with lymphadenopathy,
fever, leukocytosis, elevated erythrocyte
sedimentation rate, and polyclonal hypergammaglobulinemia. Renal involvement is
characterized on CT by infiltrative hypodensity at the periphery of the kidney as a result
of the accumulation of histiocytes. The imaging findings are often more suggestive of
a subcapsular rather than a perinephric process [7] (Fig. 12).

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Fig. 15.9-month-old boy with nephroblastomatosis. Axial contrast-enhanced CT


image shows that kidneys are bilaterally enlarged due to cortical hypodense, nonenhancing, soft-tissue mass (arrows). Note distorted renal parenchyma centrally.

Erdheim-Chester Disease

Conclusion

Erdheim-Chester disease (lipoid granulomatosis) is a relentlessly progressive multisystem disorder characterized radiologically
by bilateral symmetric medullary osteosclerosis with cortical thickening of long tubular
bones, sparing the axial skeleton. Retroperitoneal and perinephric infiltration may occur
[8]. Long-bone abnormalities are crucial findings for recognition of this disease (Fig. 13).

A variety of conditions may result in perinephric and subcapsular disease, with potentially overlapping radiologic features; however,
close attention to and knowledge of the associated clinical and imaging features can facilitate a
confident and specific diagnosis in many cases.

Subcapsular Disease
Renal Cortical Necrosis

Renal cortical necrosis is characterized by


the destruction of the renal cortex with sparing
of the renal medulla and is the cause of approximately 2% of cases of acute renal failure.
Renal cortical necrosis is usually seen as a
complication of obstetric catastrophes. In the
acute setting, CT shows nonenhancement of
the renal cortex, with the exception of a thin
subcapsular rim, and normal medullary enhancement (Fig. 14). In chronic cases, renal
cortical eggshell calcification may occur [9].
Nephroblastomatosis

Nephroblastomatosis is the presence of


multiple or diffuse nephrogenic rests, embryonal tumors that result from abnormal renal histogenesis and are precursors of
Wilms tumor. Nephroblastomatosis is characterized by the presence of multiple welldefined round or ovoid foci in the periphery
of the renal cortex (Fig. 15).

References
1. Jayson M, Sanders H. Increased incidence of serendipitously discovered renal cell carcinoma.
Urology 1998;51:203205
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The perirenal space: relationship of pathologic
processes to normal retroperitoneal anatomy. RadioGraphics 1996;16:841854
3. Bailey JE, Roubidoux MA, Dunnick NR. Secondary
renal neoplasms. Abdom Imaging 1998;23: 266274
4. Ali MG, Coakley FV, Hricak H, Bretan PN.
Complex posttransplantation abnormalities of renal allografts: evaluation with MR imaging. Radiology 1999;211:95100
5. Choyke PL, White EM, Zeman RK, Jaffe MH, Clark
LR. Renal metastases: clinicopathologic and radiologic correlation. Radiology 1987;162: 359363
6. Haddad MC, Hawary MM, Khoury NJ, AbiFakher FS, Ammouri MF, Al-Kutoubi AO. Radiology of perinephric fluid collections. Clin Radiol
2002;57:339346
7. Brown WE, Coakley FV, Heaney M. Renal involvement by Rosai-Dorfman disease: CT findings. Abdom Imaging 2002;27:214216
8. Yun EJ, Yeh BM, Yabes AP, Coakley FV, Kane
CJ. Erdheim-Chester disease: case report and review of associated urological, radiological and
histological features. J Urol 2003;169:14701471
9. Sallomi DF, Yaqoob M, White E, Finn R. Case report: the diagnostic value of contrast-enhanced
computed tomography in acute bilateral renal
cortical necrosis. Clin Radiol 1995;50:126127

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