Wilms Tumor

Radiography
Conventional radiography is a noninvasive and economical way to demonstrate lung and
bone metastases. Plain radiographs are also used in following up radiation therapy and in
looking for pulmonary complications associated with chemotherapy.
A plain abdominal radiograph often shows displacement of the abdominal viscera and, in less
than 10% of cases, streaky or irregular calcification. Calcification is more apparent on CT
than on radiographs. The calcification usually is on the edge of the tumor, whereas the
calcification associated with a neuroblastoma is speckled throughout.
IVU shows an intrarenal mass confined within the renal outline (see the image below). The
mass is often associated with splaying, distortion, and displacement of the calyces. Threedimensional assessment can be achieved by using frontal and lateral radiographs. Upper and
posterior tumors increase calyceal distortion, as these sites offer little room for exophytic
expansion because of their rigid surrounding structures.

An IVU shows a nonfunctioning left kidney with a suggestion

of ill-defined mass in the left loin due to a biopsy-proven Wilms tumor. Note the functioning
right duplex renal collecting system. The chest radiograph in the same child shows a lung
metastatic deposit (arrow). Images courtesy Dr. Pedro Daltro and Dr. Edson Marchiori, Port
Allegre, Brazil. edmarchiori@gmail.com
With IVU, an inadequate dose of contrast medium often causes nondiagnostic results, with
subsequent errors in diagnosis. Relatively large doses of contrast agent (4 mL/kg) should be
used to obtain diagnostic IVUs.
Tumors are commonly large at presentation and often cross the midline. Because large tumors
may virtually replace the excretory renal tissue, IVU may show little opacification. This is
true in approximately 10% of children with a Wilms tumor, but it does not appear to affect
their prognosis.
Tumors at the lower pole and anterior tumors have more room for exophytic growth.
Therefore, their associated calyceal deformity is less pronounced than that observed with
upper and posterior tumors. A central tumor may cause hydronephrosis and calyceal
distortion.
In the developed world, conventional radiography (apart from chest radiography) has a
limited role in the workup of Wilms tumor to detect and follow up lung metastases. However,
in the developing world, much more reliance may be placed on conventional radiography and

perhaps ultrasonography, because these modalities are more readily available. Chest
radiography, IVU, and ultrasonography provide the best combination in parts of the world
where CT and MRI are not available.
A preoperative imaging protocol that relies predominantly on chest radiography and
abdominal ultrasonography does not reduce survival.[20] More sophisticated imaging,
particularly CT, is not required in most cases, and it is warranted only when results of chest
radiography or ultrasonography are not helpful for resolving relevant management problems.
Lung and bone metastases are easily missed on plain radiographs. Moreover, plain
radiographs have low specificity.

Kidney Angiomiolipoma Imaging

Radiography
Angiomyolipomas of sufficient size may be appreciated on a plain abdominal radiograph or
an IV urogram (see the images below). A large, extrarenal, exophytic component is present in
25% of cases; it may be visualized with both a plain abdominal radiograph and an IV
urogram.

Renal ultrasonogram obtained in a 12-year-old boy with

known tuberous sclerosis. Note the multiple echogenic tumors of varying sizes in both
kidneys. This oblique sagittal scan through the left kidney shows a 4-cm echogenic mass
(arrow) on the inferior aspect of the kidney that anteriorly displaces the renal sinus (S).

Renal ultrasonogram depicting many tumors in the right

kidney. The arrow marks an echogenic 1-cm lesion (same patient as in the previous image).

Selective right renal angiogram showing multiple avascular

tumors. The tumors are small (same patient as in the previous image).

Selective left renal angiogram showing 2 tumors, which are

larger than those in the previous image (same patient as in the previous image). The final
diagnosis was multiple renal angiomyolipomas in a patient with tuberous sclerosis.

Ultrasonogram obtained in a 48-year-old man who presented

with dyspepsia and right upper quadrant discomfort. The patient was referred for a
gallbladder ultrasonogram. The gallbladder was normal, but a solid 18-mm mass was present
in the upper pole of the right kidney; it was isoechoic relative to the renal sinus.

Part of an intravenous urogram series obtained in the same patient

as in the previous image. The radiograph shows a hypoattenuating exophytic mass (arrow).
If planar tomographic images are obtained before the administration of IV contrast material
and if a large quantity of fat is present within the tumor, radiolucency may be evident. This
finding suggests the diagnosis of angiomyolipoma; it is seen in more than 10% of cases. With
multiple large angiomyolipomas, particularly those in patients with tuberous sclerosis, an IV
urogram may demonstrate distortion of the renal collecting system that is indistinguishable
from polycystic renal disease. On CT scans, calcification is apparent within the tumor in as
many as 6% of cases.
Plain radiography and IV urography are not useful in the diagnosis of angiomyolipoma,
because neither modality has enough sensitivity to demonstrate fat within the tumor.
Moreover, there are other causes of the occurrence of fat within renal masses, although such
cases are rare. Multiple angiomyolipomas that distort the collecting system may be
indistinguishable from polycystic disease. A false-positive diagnosis may occur in cases

involving other renal tumors containing fatty tissue. Only larger angiomyolipomas contain a
sufficient amount of fat to be visible on plain radiographs.

RENAL CELL CARSINOMA

Radiography
Plain radiography

Plain radiographic findings often are unrevealing in patients with renal cell carcinoma, unless
the mass contains detectable calcification or is large enough to distort the normal renal
contour. Plain radiography has no role in the primary search for RCC or in the follow-up
observation of patients with RCC because of its limited sensitivity and specificity.
Intravenous urography

Intravenous urography (IVU) is also limited in depicting RCCs. Large lesions, which can
distort the renal contour or the collecting system, may be detected with IVU. If RCC is
suggested, further imaging with CT or MRI is necessary to confirm a solid mass and to stage
the disease. If the lesion appears to be a cyst, US is the next best test in the patient's workup.
Findings of RCC are nonspecific and include mass effect on the collecting system, distortion
of the renal contour, enlargement of a portion of the kidney, and calcifications. If good
nephrotomograms are obtained at peak renal enhancement, most RCCs are less attenuating
than surrounding renal parenchyma. Renal vein invasion may be inferred if contrast material
excretion by the affected kidney is poor or absent. Alternatively, this finding may result from
extensive involvement of the kidney or ureteral obstruction caused by mass effect.
Degree of confidence

In any patient with normal IVU findings and persistent hematuria or other results suggestive
of RCC, CT or MRI should be performed.
False positives/negatives

Plain radiography and IVU have substantial limitations in the detection of RCCs. These
techniques should not be used as the primary modalities in the workup of suspected RCC.

Case 1. Large renal cell carcinoma. Three-minute tomogram.

Case 1. Large renal cell carcinoma. Delayed intravenous urographic image.

Case 3. Small renal cell carcinoma. Tomogram.

Case 3. Small left renal cell carcinoma is subtle on this intravenous urographic
image.

PAPILLOMA
Radiography

Calcification in a sloughed papilla is characteristically ring shaped and may be the only
abnormal radiologic finding in cases of necrosis in situ. Calcification is common in patients
with analgesic-induced papillary necrosis; it has not been reported in patients who have
papillary necrosis associated with hemoglobinopathy.
Persistent streaking of contrast from the polar fornix is almost diagnostic of renal papillary
necrosis. Necrosis in situ is difficult to diagnose because necrotic tissue does not slough.
Filling defects within the pelvocalyceal system and the ureter are nonspecific findings.
Opacification of the collecting system is poor when renal function is impaired.
Medullary calcification may occur in patients with hyperparathyroidism, renal tubular
acidosis, and medullary sponge kidney, as well as patients with hypercalcemia. The presence
of ringlike calcifications of up to 5-6 mm in diameter is characteristic of sloughed papillae.
Findings of medullary calcification (nephrocalcinosis) are nonspecific. Calcification may be
the only abnormal radiologic finding in papillary necrosis in situ.
Plain radiography

On plain radiography, the kidneys are normal in size and contour except in the late stage of
disease, during which they shrink and demonstrate a wavy contour as a result of the
prominence of the septal cortex around the atrophied centrilobular cortex.
Sloughed papillae may calcify; on plain radiography they may be observed as curvilinear or
ringlike calcification measuring up to 5-6 mm in diameter. The appearance of calcification
implies that a change in urine bacteriology has occurred such that Proteus organisms
predominate.
On plain radiographs, tiny calcifications may be observed in the region of the liver, spleen,
adrenal glands, and lymph nodes in patients with abdominal tuberculosis.
Excretory urography findings

Urographic findings depend on the stage of the disease. The kidneys are of normal size, and
the contour is smooth until the late stage, in which the kidneys shrink and demonstrate a
wavy contour. (See the images below.)

Excretory urography in a patient with diabetes. A film

obtained at 5 minutes shows horns from the calices, ring shadows, and an eggin-a-cup appearance (ring sign) characteristic of renal papillary necrosis.

Excretory urography in a 53-year-old man with

analgesic-induced nephropathy. A film obtained at 15 minutes after
administration of contrast shows a wavy renal outline with tracks of contrast
extending from fornix, ring shadows caused by the sloughing of papillae, and an
egg-in-a-cup appearance characteristic of renal papillary necrosis. Note the

bamboo spine, characteristic of ankylosing spondylitis.

Excretory urography in a patient with renal papillary necrosis and pyeloureteritis
cystica. Note the bilateral loss of the renal mantle with contrast tracking from the
renal fornix in the lower pole of the right kidney. Note also the multiple smooth
filling defects in the ureter, caused by ureteritis cystica.

In the early stage, papillary swelling may be the only abnormal finding; papillary necrosis
may be difficult to diagnose.
Later, necrosis of the papillae, in association with disruption of the urothelial lining, causes
tracking of contrast from the fornix parallel to the long axis of the papillae. These

developments may produce the lobster claw sign. Cavitation of renal papillae then occurs;
such cavitation may be incomplete (medullary) or complete (papillary) and may be either
central or eccentric.
Shrinkage and sloughing of the necrotic papillae cause forniceal widening and calyceal
clubbing.
Sloughed papillae cause a filling defect in the pelvocalyceal system and in the ureter.
Retrograde pyeloureterography

Findings in this modality are similar to those of excretory urography. Minor abnormal
papillary findings may be demonstrated readily when urographic findings are indeterminate.
Degree of confidence

Retrograde pyelography is sensitive, especially in the presence of renal impairment or for

patients in whom urographic findings are inconclusive. The procedure cannot help in the
assessment of renal function or the renal parenchyma.
In the early stage, when papillary swelling may be the only abnormal radiologic finding,
swelling is difficult to recognize. Necrosis in situ cannot be diagnosed unless calcification has
occurred.
False positives/negatives

Pyelosinus extravasation, which may occur with forceful injection of a large volume of
contrast, may mimic the tracking of contrast from the fornix in papillary necrosis.
Inadvertent injection of air bubbles may produce filling defects, but the defects appear
smooth and rounded and therefore may be differentiated from the irregular filling defects
found in sloughed papillae.