299

Wilms Tumor (Nephroblastoma) in

the Adult Patient: Clinical and
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Radiologic Manifestations

Farhad Kioumehr1 Wilms tumor (nephroblastoma), a primary renal neoplasm containing primitive bIas-
Sachiko 1. Cochran2 tema and embryonic glomerulotubular structures, is seen rarely in adults. To identify
Lester Layfield3 clinical and radiologic criteria for preoperative diagnosis of adult Wilms tumor, we
studied 29 cases reported in the literature from 1975 to 1987 (all patients were 15 years
Issa Yaghmai1
old or older) and four newly diagnosed cases. The mean age of patients was 30 years;
Caroline Ngo1
80% were less than 35 years old. Each patient presented with a large, rapidly growing,
Stanley R. Smith4 abdominal mass (average duration of symptoms, <2 months), had no constitutional
symptoms (e.g., weight loss, fever), and otherwise were healthy (80%). Twenty-sIx
patients had IV or retrograde pyelograms. Twenty-three showed a nonspecific mass
effect. In seven (78%) of nine patients, abdominal CT scans showed a large, inhomo-
geneous mass with large areas of low density and increased enhancement of the
compressed remaining normal renal parenchyma, which resembled a pseudocapsule.
In five (63%) of eight patients, sonograms showed a complex mass with large cystic
components. In 18 (82%) of 22 patients, arteriograms showed a hypovascular mass with
fine wavy or zigzag (creeping-vine) neovascularfty.
We conclude that a rapidly growing renal mass in a young patient (<35 years old)
that is shown to be complex and cystic by CT or sonography and that is hypovascular
with fine, wavy neovascularity on arteriography is suggestive of adult Wilma tumor (75-
80%). An awareness of this constellation of findings may be helpful in diagnosing this
unusual tumor before surgery.

Wilms tumor (nephroblastoma) in adults is rare and represents 0.5% of all renal
neoplasms [1 ]. Nephroblastoma is only one of 53 synonyms for this tumor. The
number of cases reported in the literature is difficult to determine owing to
controversy over the pathologic diagnosis. There is also uncertainty about whether
all the reported cases actually represent adult Wilms tumors or whether they are
sarcomatoid renal carcinomas instead [2].
Kilton et aI. [31 compiled the following criteria for the diagnosis of adult Wilms
tumor: (1) presence of a primary renal neoplasm, (2) presence of a primitive
blastematous spindle or round-cell component, (3) formation of abortive or em-
Aeceived August 30, 1988; accepted after revi-
sion October 3, 1988. bryonal tubular or glomeruloid structures, (4) absence of tumor diagnostic of renal
Department of Radiology, Olive View Medical cell carcinoma, and (5) age over 1 5 years.
Center, 14445 Olive View Dr., Sylmar, CA 91342. Previous papers have reported that adult Wilms tumor cannot be recognized
Address reprint requests to F. Kioumehr. specifically before surgery. To identify clinical and radiologic findings that might
2Department of Radiology, UCLA School of
suggest the diagnosis of adult Wilms tumor before surgery, we reviewed the cases
Medicine, 10833 Le Conte Ave., Los Angeles, CA
reported in the literature and our four new cases.
90024.
3Department of Pathology, UCLA School of
Medicine, Los Angeles, CA 90024.
Department of Aadiology, Bums Clinic Medical Materials and Methods
Center P.C., 560 W. Mitchell Ave., Petoskey, MI
49770. We reviewed all 29 cases of adult Wilms tumor reported in the literature from 1975 to
1987 [1-14]. Also, a computer search of the pathologic records at multiple medical centers
AJR 152:299-302, February 1989
0361 -803X/89/1 522-0299 (Olive View, UCLA, Harbor/UCLA, LA County/USC Medical Center, and Bums Clinic Medical
© American Roentgen Ray Society Center) identified four new cases of adult Wilms tumor. These 33 cases were reviewed by a
 300 KIOUMEHR ET AL. AJR:152, February 1989

pathologist to confirm that they were indeed adult Wilms tumor. Of sented to the UCLA Medical Center with a large, nontender, rapidly
these cases, 26 (79%) had IV or retrograde pyelograms, nine (27%) growing, left-sided abdominal mass. There was no weight loss or
had abdominal CT scans, eight (24%) had sonograms, and 23(70%) hematuna. An abdominal CT scan showed a large, inhomogeneous
had angiograms. mass with cystic and solid components, arising from the left kidney.
The clinical data that were collected included the patient’s age and A renal arteriogram showed a hypovascular mass with a few small,
sex, side of tumor, type and duration of symptoms, record of weight irregular vessels extending into the mass. No filling defect was seen
loss, and presence of hematuria and metastasis. Radiolgoic data on the inferior venacavogram. The fourth patient was a 68-year-old
included appearance of tumor on IV or retrograde pyelography, man in whom a right lower-pole renal mass was identified incidentally
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abdominal CT, sonography, or angiography. on IV pyelography. Sonography showed a complex, solid mass. The
Four new cases were analyzed. Two of these are illustrated in mass was hypovascular on renal artenography. Normal renal arteries
Figures 1 and 2. The third patient was a 1 5-year-old girl who pre- were stretched around the mass, which had a fine neovascularity.

Fig. 1.-18-year-oId woman with a rapidly growing abdominal mass. There was no weight loss or hematuria.
A, Enhanced abdominal CT scan shows a large, inhomogeneous, exophitic mass arising from left kidney and extending to anterior abdominal wall;
there is a huge cystic component anterioriy and a dilated calix posteriorly. Cystic component is denser than fluid in calix. Solid component shows less
enhancement than that seen in normal parenchyma (pseudocapsule) (arrows).
B, Sonogram shows a large, complex, mostiy cystic mass with septation involving lower pole of kidney (arrows) and a dilated upper-pole caiix.
C, Selective left renal arteriogram shows a hypovascular mass arising from lower pole of kidney and some neovasculanty extending into tumor (arrows).
No clot was seen on an inferior venacavogram (not shown).

Fig. 2.-18-year-old woman with an enlarging

right lower quadrant mass.
A, IV pyeiogram shows a large, radiolucent
mass in right lower pole. Cyst puncture revealed
clear, light amber fluid. Wall of cyst was smooth
on contrast examination. However, malignant
cells were found on cytologic examination.
B, Arteriogram shows an avascuiar mass with
no tumor vessels (arrows).
A B
 AJA:152, February 1989 WILMS TUMOR 301

No tumor thrombus was seen on inferior venacavography. The met- Follow-up data were available in 1 8 patients in whom there
astatic workup was negative. was radiologic, surgical, or pathologic information about the
renal vein and inferior vena cava. Metastases developed in
Results five of six patients in whom the renal vein and/or inferior vena
cava was positive for tumor thrombus (follow-up, 3 months
Clinical Findings
to 2 years). Six of 1 2 patients in whom the renal vein and/or
Twenty-six (79%) of the 33 patients reviewed were 35 inferior vena cava was negative for tumor thrombus were free
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years old or younger (average age, 30; range, 1 5-84). There of disease during follow-up (1 0 months to 71/2 years).
were 1 7 women and 1 6 men. In 18 patients, the tumor was
in the right kidney; in 1 5 patients, it was in the left kidney. In
1 0 (59%) of the 1 7 women, the tumor was on the left side; in Pathologic Findings
1 1 (69%) of the 16 men, the tumor was on the right side.
Most of the tumors were large, solitary, unilateral masses
Symptoms included abdominal pain of short duration re-
that were well circumscribed grossly but not encapsulated.
lated to a rapidly growing abdominal mass. Pain in the abdo-
Areas of hemorrhage were described grossly in eight of 33
men, flank, or back was noted in 22 (88%) of 25 cases and
cases, and necrosis was found in 1 3 cases. Although radio-
was not present in three cases (1 2%). (Data were not available
logic imaging showed inferior vena caval involvement in eight
in eight [24%] of 33 cases.) A palpable abdominal mass or
cases, invasion was documented pathologically in three. In
abdominal fullness was present in 1 9 (79%) of 24 cases and
the 26 cases in which slides or adequate pathologic descrip-
was absent in five cases (21 %). (No data were available in
tions were available, no cases appeared to have true anapla-
nine [27%] of 33 cases.) Duration of symptoms ranged from
sia as defined by the National Wilms’ Tumor Study criteria.
1 day to 8 months. In 1 7 (71 %) of 24 patients, the symptoms
Also, no rhabdoid or clear cell variants were reported. Most
lasted 3 months or less. Six patients presented with weight
cases appeared to be mixed blastemal and epithelial (13
loss. Hematuria was present in 1 5 (58%) of 26 patients and
cases) or blastemal predominant neoplasms (nine cases).
was absent in 1 1 patients (42%). (In seven of 33 [21 %] cases,
no data were available.)
Nine (28%) of 32 patients had metastases at the time of Discussion
presentation. In one patient, no data were available. The most
Wilms tumor is a childhood neoplasm; in 85% of cases, it
common locations were the lung and the liver.
occurs before the patient is 6 years old. Only 1 % of these
tumors occur in patients older than 1 5 years. Two hundred
Radiologic Findings
cases have been reported in adults, but it is unclear whether
Twenty-six (79%) of 33 patients had an IV or retrograde all of these cases were adult Wilms tumors; some of them
pyelogram. In 23 (88%) of the 26 a mass was shown in the may have been sarcomatoid renal carcinomas or some other
kidney. In two patients, these masses contained calcifications. renal neoplasm [1 , 2, 4]. The diagnostic criteria for Wilms
Three patients had no kidney function on the side of the tumor include the presence of abortive or embryonic glomer-
tumor. Two of these three patients had a clot in the inferior otubular structures within an immature spindle-cell stroma.
vena cava. These structures are not found in renal cell carcinoma. Some
Nine (27%) of 33 patients had abdominal CT scans. In renal cell carcinomas can have glandular elements and an
seven (78%) of the nine an inhomogeneous mass was seen abundance of sarcomatous or undifferentiated cells. These
with large areas oflow density and less contrast enhancement cases can be misinterpreted as Wilms tumors [2]. The ab-
than that seen in normal parenchyma; in these patients, the sence of fetal renal tissue points against the diagnosis of
compressed normal renal parenchyma appeared as a pseu- Wilms tumor [5]. The most common location of nephrogenic
docapsule. In the other two patients, the masses were ho- blastema is the subcapsular portion of the renal cortex [15],
mogeneous on CT. Three patients showed no thrombus in which explains this neoplasm’s common appearance as an
the inferior vena cava, two showed thrombus, and in four no exophitic mass. Microscopically, no difference exists between
data were available. Paraaortic lymph nodes (one case), pen- adult and childhood Wilms tumor. Both have epithelial,
nephnic fat involvement (one case), and liver metastases (one stromal, and blastemal elements [15].
case) also were shown by CT. Clinically, adult Wilms tumor usually presents in young
Eight (24%) of 33 patients had sonograms. Five (63%) of patients as a large, rapidly growing abdominal mass, but the
eight patients had a complex, largely cystic mass, with some disease can be seen at any age; the oldest patient reported
solid components. The remaining three patients had a solid was 84 years old [7]. In our study as well as that of Byrd et
echogenic mass. Two sonograms showed venous thrombus. al. [1 6], the mean age of patients was 30 years. This is in
Twenty-two (67%) of 33 patients had renal arteniograms. contrast to the study by Bailey et al. [7] in which patients
In 1 8 (82%) of these 22 patients masses were hypovascular; presented between the fourth and sixth decades. Renal cell
in two, masses were vascular; and in two, masses were carcinoma occurs in an older population than does adult
hypervascular. The hypovascular masses contained fine zig- Wilms tumor. Women are affected more often than men [8,
zag, wavy neovasculanity that extended into the tumor. 1 5-1 7]. Renal cell carcinoma, however, is seen predominantly
Inferior venacavograms were performed in nine (27%) of in men.
33 patients. Six (67%) of the nine were negative, one was Usually the tumor is very large and is palpable, in contrast
positive, and two were false positive. to renal cell carcinoma in which the tumors are generally
 302 KIOUMEHR ET AL. AJR:152, February 1989

smaller and often are less palpable at the time of presentation. mass in an otherwise healthy young patient that shows as a
IV pyelography showed a nonspecific mass effect or, on complex mass on sonography and as an inhomogeneous
rare occasions, a nonfunctioning kidney. A horseshoe kidney mass with variable enhancement and pseudocapsule on CT
was reported in one patient with adult Wilms [7]. One patient is highly suggestive of Wilms tumor. Hypovasculanity of the
had a large, calcified mass [9]. Calcifications are rare in adult mass on arteniography with a few zigzag lines of neovascu-
Wilms tumor but may be seen because of hemorrhage or lanity extending to the mass is also characteristic. This is in
necrosis within the lesion. contrast to renal carcinoma, which usually is solid and smaller
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Although the number of cases studied by high-resolution than Wilms tumor, is hypervascular and has arteniovenous
CT is limited, the findings in adult Wilms tumor are character- shunting on arteniography, occurs at an older age, and in-
istic. Abdominal CT shows a large, well-defined, exophitic, volves more constitutional symptoms. Multilocular cystic ne-
inhomogeneous mass that is cortical in origin. Large areas of phroma should also be considered in the differential diagnosis.
low density are present without contrast enhancement. After This benign renal tumor, which is seen more frequently in
IV contrast administration, the solid component shows van- children, also can occur in adults (predominantly women). The
able enhancement, and the remaining normal parenchyma tumor is well encapsulated and contains numerous noncom-
shows greater enhancement; the normal parenchyma ap- municating cysts. Occasionally, calcifications are present and
pears as a pseudocapsule around the tumors (75%). Nonen- the tumor is hypovascular on arteniography.
hancing areas representing foci of necrosis and hemorrhage
are common. These findings are similar to those in childhood ACKNOWLEDGMENTS
Wilms tumor [8, 1 5]. Renal cell carcinoma generally presents
We thank Paul Dickman for providing the UCLA/Harbor case,
as a smaller, infiltrative tumor when compared with Wilms
Howard Mindell for bringing a new case to our attention, and Sharon
tumor [17].
Simpson and Cynthia Shirley for editing and typing the manuscript.
Sonographic studies of adult Wilms tumor typically show a
large, complex mass with large cystic components. This is in
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