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Ranganath et al.
Abdominal Masses in Children
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Residents Section
Pattern of the Month
Residents
inRadiology
Shashi H. Ranganath1,2
Edward Y. Lee1
Ronald L. Eisenberg2
Ranganath SH, Lee EY, Eisenberg RL
2
Department of Radiology, Beth Israel Deaconess
Medical Center, Harvard Medical School, 330 Brookline
Ave, Boston, MA 02215. Address correspondence to
R. L. Eisenberg (rleisenb@bidmc.harvard.edu).
WEB
This is a Web exclusive article.
AJR 2012; 199:W1W16
0361803X/12/1991W1
American Roentgen Ray Society
Ranganath et al.
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Fig. 2Hepatic abscess in 13-year-old boy with right upper quadrant pain, fever, and elevated liver function
tests. Culture of drainage fluid was positive for Escherichia coli.
A, Transverse ultrasound image of liver shows large cystic mass (arrows) with internal mobile echogenic debris (D).
B, Contrast-enhanced axial CT image shows complex fluid collection (arrows) with internal septations of
varying thickness in right hepatic lobe.
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Fig. 4Hepatic mesenchymal hamartoma in 15-month-old girl with progressively increasing abdominal
distention and palpable right upper quadrant mass.
A, Transverse ultrasound image of liver shows large mass with areas of cystic (C) and solid (arrows)
components.
B, Contrast-enhanced axial CT image of liver shows large mass with multiple cysts (C) of variable size and solid
components (arrows).
Ranganath et al.
because an increased risk of malignancy and, particularly in symptomatic patients, mass effect
from large lesions.
Choledochal cystCholedochal cysts are a spectrum of congenital developmental abnormalities of the biliary system in which there is fusiform dilatation of the extrahepatic and
intrahepatic bile ducts. The Todani classification of choledochal cysts is as follows:
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Type 1: Segmental or diffuse fusiform dilatation of the common bile duct (most frequent)
Type 2: Diverticulum of an extrahepatic duct
Type 3: Choledochocele
Type IVa: Multiple extrahepatic bile duct cysts with intrahepatic biliary involvement
Type IVb: Multiple extrahepatic bile duct cysts alone
Type 5: Cystic dilatation of the intrahepatic bile ducts (Caroli disease)
Pediatric patients with choledochal cysts typically present with jaundice, abdominal pain,
and a mass. Ultrasound and MRI are the two best imaging modalities to detect and characterize choledochal malformations in children. MRI using an MRCP protocol with thick-slab
T2-weighted turbo spin-echo (TSE) and HASTE in the coronal, coronal oblique, and axial
planes is the MRI protocol for evaluating choledochal cysts. The findings can show a choledochal cyst that is separate from the gallbladder but communicates with the biliary ducts and
shows abrupt changes in caliber at the junction between the dilated segment and normal ducts
(Fig. 5). Dilatation of multiple intrahepatic bile ducts is typically seen in Caroli disease (Fig.
6). Typical complications of choledochal cysts include infection, stone formation, and bile
duct carcinomas. The current treatment of choice is surgical resection.
Kidney
HydronephrosisHydronephrosis, characterized by a dilated renal pelvis that communicates with dilated calyces, is the most frequent abdominal mass in neonates and infants. Im-
C
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aging evaluation plays an important role by differentiating a marked hydronephrosis that may
mimic a true intrarenal cystic mass. In the pediatric population, the three most frequent
causes of hydronephrosis are ureteropelvic junction (UPJ) obstruction, ureterovesicular junction obstruction, and renal duplication. Additionally, patients with prune belly syndrome can
also present with bilateral varying degrees of hydronephrosis and hydroureters.
UPJ obstruction is the most frequent cause of hydronephrosis in the pediatric population.
UPJ obstruction typically produces severe hydronephrosis, which ends abruptly at the UPJ
and is associated with a normal caliber of the downstream ureter (Fig. 7). In severe cases of
UPJ obstruction, the renal pelvis is disproportionately enlarged compared with the calyces.
Theoretic causes for UPJ obstruction include abnormal smooth muscle arrangement of the
proximal ureter that impairs distensibility, abnormal innervation of the proximal ureter impairing peristalsis, and a crossing vessel or fibrous scar at the UPJ. UPJ obstruction can be
evaluated with ultrasound and 99mTc-MAG3 (mercaptoacetyltriglycine) renography. Ultrasound can show structural dilatation of the renal collecting system, whereas 99mTc-MAG3
renography provides functional information of renal excretion in patients with UPJ obstruction. After injection of 99mTc-MAG3, sequential images of the kidney are obtained. In cases
of UPJ obstruction, there is no drainage from the dilated renal collecting system into ureter
and bladder despite hydration and diuretic washout. The current treatment of choice for UPJ
obstruction is pyeloplasty to resect the narrowed segment at the UPJ or to reroute the crossing
vessel. In pediatric patients with UPJ obstruction, pyeloplasty is indicated when there is persistent or worsening renal collecting system obstruction or deteriorating renal function on serial
follow-up studies, such as ultrasound and 99mTc-MAG3 renography.
Another cause of hydronephrosis in pediatric patients is ureterovesicular junction obstruction.
Megaureter is the most frequent type of ureterovesicular junction obstruction, in which the renal
collecting system and ureter are dilated because of a functional aperistalsis of the distal ureter at the
juxtavesical ureteral segment. Other less frequent causes of ureterovesicular junction obstruction
include ureterocele and distal ureteral stricture. The amount of hydroureteronephrosis depends
Ranganath et al.
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Fig. 8Ureteropelvic duplication in 19-day-old girl with prenatal diagnosis of hydronephrosis and duplex kidney.
A, Longitudinal ultrasound image of right kidney shows markedly dilated upper pole (UP) moiety and mildly dilated
lower pole (LP) moiety. Dilated upper pole ureter (UU) and lower pole ureter (LU) are also seen. Upper pole and
lower pole collecting systems are separated by band of renal cortex (arrow).
B, Longitudinal ultrasound image of bladder (BL) shows upper pole ureter terminating in bladder as ureterocele (UC).
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Fig. 10Renal abscess in 15-year-old boy who presented with fever, elevated WBC count, and right flank pain.
A, Transverse ultrasound image of right kidney shows avascular heterogeneous area in upper and mid zones.
B, Contrast-enhanced axial CT image shows heterogeneous lesion with multiple internal septations of varying
thickness in right kidney.
Ranganath et al.
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Multilocular cystic nephromaA multilocular cystic nephroma is a rare nonhereditary benign cystic renal neoplasm. It typically occurs in boys between 3 months and 2
years old, or in women between 40 and 50
years old. Multilocular cystic nephromas can
range in size from a few centimeters to a
mean of approximately 10 cm. In children,
multilocular cystic nephroma typically presents as a palpable abdominal mass (Fig.
12A). On ultrasound, the lesion is usually a
well-defined multiloculated anechoic mass
with echogenic internal septa (Fig. 12B). Although CT is not usually necessary after ultrasound detection, multilocular cystic nephroma may be incidentally detected on CT
Fig. 11Mutlicystic dysplastic kidney in 2-weekobtained for other reasons. On CT, it appears old boy with prenatal diagnosis of cystic right
as a cystic mass with water attenuation, well- renal mass. Transverse ultrasound image of right
circumscribed margins, and internal septa- kidney shows numerous cysts (C) of various sizes
recognizable normal renal corticomedullary
tions (Fig. 12C). Because imaging studies without
architecture.
cannot reliably distinguish multilocular cystic nephroma from a multilocular cystic renal cell carcinoma or cystic Wilms tumor (Fig. 13),
surgical excision is the currently accepted management of choice.
Cystic Wilms tumorWilms tumor is the most frequent primary renal neoplasm in the pediatric population. It accounts for approximately 95% of all pediatric renal malignancies and
typically occurs in children younger than 5 years old. Pediatric patients with Wilms tumor
B
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Fig. 12Multilocular cystic nephroma in 10-monthold boy who presented with palpable left-sided
abdominal mass.
A, Abdominal radiograph shows opacity (M) centered
in left side of abdomen that produces mass effect on
adjacent colon (arrow).
B, Transverse ultrasound image of left kidney shows
cystic mass (C) with echogenic septa (arrow). Blood
flow within these echogenic septa is also seen.
C, Contrast-enhanced axial CT image shows large
water-attenuation mass with well-circumscribed
borders and mildly enhancing internal septations
(arrows).
C
AJR:199, July 2012
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Fig. 13Cystic Wilms tumor in 1-year-old boy who presented with rapidly enlarging right-sided abdomen and
increasing irritability.
A, Transverse ultrasound image of right kidney shows large cystic mass (C) arising from right kidney (arrows).
B, Contrast-enhanced axial CT image shows large cystic mass (C) with well-circumscribed borders and without
internal solid components arising from right kidney (arrows).
present with a palpable abdominal mass, pain, hematuria, or hypertension. Wilms tumor may
be associated with clinical syndromes or anomalies, including Beckwith-Wiedemann syndrome, Drash syndrome, hemihypertrophy, cryptorchidism, and sporadic aniridia. Approximately 510% of children with Wilms tumors have bilateral or multicentric tumors. Although
Ranganath et al.
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the majority of Wilms tumors are solid renal masses, a small percentage may be purely cystic,
referred to as cystic Wilms tumor.
On ultrasound, cystic Wilms tumors are anechoic intrarenal masses (Fig. 13A). Although
the diagnosis of cystic Wilms tumor and vascular extension can be made on ultrasound, CT
may be used to confirm the renal origin of the tumor, its margins, and the local and distant
metastases (Fig. 13B). The current management of Wilms tumor involves multimodal therapy, including surgery, chemotherapy, and radiation therapy for selected patients. Such multimodal therapy results in dramatic improvement in outcome in children with Wilms tumor,
with overall 5-year survival rates of 90%.
Rhabdoid tumorMalignant rhabdoid tumor is a rare and aggressive renal neoplasm in children. It is often associated with early onset of local and distant metastases as well as resistance
to chemotherapy. The most frequent sites of metastases are lungs, abdominal lymph nodes, liver,
brain, and bone. Although there is no pathognomonic imaging feature of malignant rhabdoid
tumor of the kidney in children, it typically presents as a large renal mass composed of both
cystic and solid components. The most helpful imaging feature is a peripheral subcapsular crescent-shaped fluid collection, which may be due to either hemorrhage or necrosis (Fig. 14). However, the definitive diagnosis of rhabdoid tumor is only made by histologic examination. Treatment includes both surgery and chemotherapy, but the prognosis is generally poor.
Pancreas
Pancreatic pseudocystA pancreatic pseudocyst is a collection of pancreatic fluid and inflammatory exudates encapsulated by fibrous tissue. It usually develops as a result of posttraumatic or inflammatory injury to the pancreas, when unabsorbed fluid collections organize and
form a fibrous capsule within 46 weeks after initial injury. On ultrasound, pancreatic pseudocysts are usually well-circumscribed unilocular smooth-walled cystic masses, often with irregularly thickened walls (Fig. 15A). They are most frequently located in the body or tail of the
pancreas. Pancreatic pseudocysts can sometimes be complicated by hemorrhage or infection,
which is manifested by septations, internal echoes, and fluid-debris levels on imaging studies. On
CT, pancreatic pseudocysts are typically round or oval homogeneously hypodense lesions with
near-water attenuation (Fig. 15B). However, the attenuation and complexity of the lesion can
increase when there is associated hemorrhage or superimposed infection. In pediatric patients,
ultrasound is usually used to follow pancreatic pseudocysts, which may require surgical decompression if they continue to grow and cause symptoms.
Pancreatic cystadenomaPancreatic cystadenoma is a benign neoplasm arising from acinar
cells of the pancreas. It is composed of numerous small cysts that contain proteinaceous fluid
and are separated by septa of connective tissue. Depending on the size of the individual cysts,
pancreatic cystadenoma can have a variable appearance on ultrasound and CT. On ultrasound,
Fig. 15Pancreatic pseudocyst in 17-year-old girl with history of pancreatitis who presented with new onset
of epigastric pain.
A, Longitudinal ultrasound image of epigastric region shows large cystic mass (C) with irregular walls (arrows).
B, Contrast-enhanced axial CT image shows large cystic mass (C) arising from pancreas. Mild surrounding
inflammatory changes and fluid (arrows) are also seen adjacent to this large pancreatic pseudocyst.
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Fig. 16Mucinous cystadenoma of pancreas in 17-year-old girl who presented with epigastric pain.
A, Transverse ultrasound image of pancreas shows large cystic mass with multiple internal septations (arrows)
in pancreatic body and tail.
B, Contrast-enhanced axial CT image shows large low-attenuation cystic mass with several mildly enhancing
internal septations (arrows) arising from body and tail of pancreas.
numerous small cysts can appear as solid, partially solid, or multicystic with septations (Fig.
16A). CT shows similar findings and can show enhancement of the septa separating numerous
small cysts (Fig. 16B). For small cystadenomas in asymptomatic patients, conservative management with follow-up imaging is sufficient. However, large cystadenomas that cause common
bile duct obstruction or atrophy of the pancreas distal to the tumor require complete surgical
excision and follow-up.
Spleen
Splenic cystSplenic cysts can be either congenital or acquired, although they appear
identical on imaging studies. Congenital splenic cysts (true or epidermoid cysts) contain an
inner cellular lining. Acquired splenic cysts (false or pseudocysts) are usually posttraumatic
and due to liquefactive necrosis. Both congenital and acquired splenic cysts usually appear as
anechoic round lesions with imperceptible walls on ultrasound, although they can show internal echoes if they contain debris or hemorrhage. Posttraumatic splenic cysts often have thicker walls that may be calcified, unlike the imperceptible walls of congenital cysts. There is no
internal enhancement on contrast-enhanced CT images (Fig. 17).
Splenic abscessA splenic abscess is a collection of pus within the splenic parenchyma.
On ultrasound, it is typically a hypoechoic lesion containing low-level internal echoes that
represent debris and internal septations. On CT, splenic abscesses typically appear as complex fluid collections that are often associated with internal septations, peripheral contrast
rim enhancement, and surrounding inflammatory changes (Fig. 18). Splenic abscesses can be
divided into microabscesses ( 1.5 cm), which are often due to fungal infection (Fig. 19), and
larger abscesses (> 1.5 cm), which are typically due to bacterial infection (Fig. 18). Splenic
abscesses in children also can be due to cat scratch disease, which is a regional, infectious
lymphadenitis caused by a pleomorphic gram-negative bacillus. Children affected with cat
scratch disease may present with multiple hypoechoic lesions on ultrasound in the spleen or
hypoattenuating lesions on CT of the spleen, representing underlying granulomatous inflammation with abscess formation. Imaging evaluation with ultrasound or CT can be helpful in
pediatric patients with splenic abscess for guiding surgical drainage procedures in managing
large splenic abscesses, particularly when the abscesses are not responding to medical management with either antibacterial or antifungal medications.
Vascular malformationSplenic vascular malformations are characterized by abnormal
vascular channels lined with a single layer of dysplastic endothelium. Capillary and venous
malformations of the spleen have a similar imaging appearance and may be solitary or multiple. Multiple vascular malformations in the spleen often occur in patients with Klippel-Trenaunay-Weber or Beckwith-Wiedemann syndrome. For assessment of the underlying vascular
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Ranganath et al.
component of splenic vascular malformation, Doppler ultrasound is a helpful imaging technique. Splenic vascular malformation may be an incidental finding on CT performed for other
reasons in pediatric patients. On CT, splenic vascular malformations are often multiple, homogeneous, and hypodense and are sometimes associated with central or peripheral calcification.
Lymphatic malformations are rare benign cystic splenic lesions that can be solitary or
multiple. On contrast-enhanced CT images, they typically have low attenuation and do not
enhance (Fig. 20).
Adrenal Gland
Adrenal hemorrhageAdrenal hemorrhage, which typically occurs in infants during the
perinatal period, is usually due to birth trauma, anoxia, neonatal sepsis, or dehydration. The
right adrenal gland is more often affected than the left. Left adrenal gland hemorrhage is associated with renal vein thrombosis. Clinical findings of neonatal adrenal hemorrhage include a palpable mass, anemia, and jaundice due to underlying hyperbilirubinemia.
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Fig. 21Neonatal adrenal hemorrhage in 5-day-old boy with prenatal diagnosis of cystic mass of right upper
quadrant.
A, Longitudinal ultrasound image of suprarenal region shows cystic mass with internal debris (arrow), likely
representing blood product and consistent with adrenal hemorrhage. K = kidney.
B, Follow-up ultrasound image obtained 3 months later shows interval decrease in size of adrenal hemorrhage
(arrows). K = kidney.
The imaging appearance depends on the stage of the hemorrhage. On ultrasound, acute adrenal hemorrhage typically presents as a suprarenal cystic mass with variable echogenicity and
complexity. In the subacute stage, adrenal hemorrhage becomes more hypoechoic because of
liquefaction of the hemorrhage (Fig. 21). Later, adrenal hemorrhage becomes hyperechoic as the
clot retracts and calcification develops. Follow-up ultrasound is helpful for documenting interval
resolution of adrenal hemorrhage in neonates because sonographic differentiation between neonatal adrenal hemorrhage and neuroblastoma can be difficult or impossible. In contrast to neonatal adrenal hemorrhage, which decreases in size within 12 weeks, neuroblastoma remains stable
or increases in size on follow-up ultrasound.
Cystic neuroblastomaNeuroblastoma is the most frequent malignant tumor in infancy and
most often originates from the adrenal gland. Although cystic neuroblastoma, which can arise
from the adrenal gland or any neural crest element of the abdomen, is a rare form of this neoplasm,
it should be considered in the differential diagnosis of a cystic adrenal mass, particularly in neonates. On ultrasound, cystic neuroblastoma typically presents as an anechoic or complex echogenic suprarenal mass that may have associated calcification (Fig. 22). Because the imaging appearance of cystic neuroblastoma is often very similar to that of adrenal hemorrhage in neonates,
follow-up ultrasound is currently recommended for management. Although adrenal hemorrhage
rapidly decreases in size and usually resolves within several weeks, cystic neuroblastoma in the
neonate is unlikely to show any substantial interval change in size on follow-up ultrasound.
Mesentery
Mesenteric Cyst or Lymphatic Malformation
A mesenteric lymphatic malformation, also known as mesenteric cyst, arises from the small
or large bowel mesentery. It develops from a proliferation of lymphatic tissue that fails to communicate with the central lymphatic system. A mesenteric cyst can range in size from a few millimeters to 40 cm. On imaging studies, mesenteric lymphatic malformations can be unilocular or
multilocular cystic lesions. They sometimes show thin septations, fine calcifications, and variable attenuation or internal echogenicity depending on the composition of the internal fluid (Fig.
23). Complications of mesenteric lymphatic malformations include hemorrhage and infection.
CSF Collection
A collection of CSF at the distal end of a ventriculoperitoneal shunt is termed a CSFoma and
typically occurs secondary to adhesion of the ventriculoperitoneal shunt catheter in the peritoneal cavity and subsequent blockage of peritoneal absorption of the shunted CSF. Children with
CSFomas typically present with abdominal distention or shunt malfunction, which manifests as
increased intracranial pressure. The diagnosis can be made by ultrasound or CT, which typically
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show an anechoic abdominal cystic mass located near the tip of the ventriculoperitoneal
shunt catheter (Fig. 24). Although CSFomas
sometimes resolve spontaneously, shunt revision or aspiration of the lesion should be considered as other treatment options, particularly in symptomatic children.
Abscess
Mesenteric abscesses are localized collections of pus within the mesentery. In children, they are often secondary to infectious
or inflammatory processes of the large and
small bowel, such as inflammatory bowel
disease or ruptured appendicitis. Ultrasound
and CT can show a fluid collection that is often complex with internal gas, air-fluid level,
or septations (Fig. 25).
Cystic Teratoma
Intraabdominal mesenteric cystic teratomas are extremely rare. Radiographs often show a
large abdominal opacity that displaces bowel loops. Calcifications associated with mesenteric
cystic teratoma can sometimes be seen. On ultrasound, the mass has variable cystic and solid
components (Fig. 26A). CT shows similar imaging findings, but this modality better shows the
fat and calcifications that are often associated with an intraabdominal mesenteric cystic teratoma (Fig. 26B).
Bowel
Duplication Cyst
Enteric duplication cyst is a rare focal congenital cystic malformation of the gastrointestinal tract. They can occur anywhere along the mesenteric border of the bowel but most frequently involve the jejunum or ileum. Although affected patients may be asymptomatic, the
typical clinical presentation includes a palpable abdominal mass, abdominal distention, vomiting (secondary to bowel obstruction), or hemorrhage (secondary to peptic ulceration due to
the presence of gastric mucosa in some patients). Pathologically, duplication cysts are characterized by a well-developed coat of smooth muscle, epithelial lining that represents some part
of the alimentary tract, and contiguity with some part of the alimentary tract.
Fig. 23Mesenteric cyst in 14-year-old girl who presented with abdominal pain.
A, Transverse ultrasound image of midabdomen shows large anechoic cystic mass (C) with imperceptible wall.
B, Contrast-enhanced axial CT image shows well-circumscribed cystic mass (C) without enhancement in
midabdomen.
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Fig. 26Mesenteric cystic teratoma in 9-month-old girl who presented with palpable abdominal mass.
A, Longitudinal ultrasound image of midabdomen shows complex mass composed of cystic (C) and solid
(arrows) components.
B, Contrast-enhanced axial CT image shows cystic component (C), fat component (F), and calcification (arrow).
On ultrasound, duplication cysts typically have a spherical, ovoid, or dumbbell shape (Fig.
27). They have a characteristic bowel wall signature, which includes echogenic mucosa, hypoechoic muscular layer, and echogenic serosa (Fig. 27). On CT, gastrointestinal duplication
cysts typically appear as round masses with water attenuation and a mildly enhancing wall
(Fig. 28). Information regarding complications of gastrointestinal duplication cysts is limited,
particularly in the pediatric population. However, reported complications include perforation,
intussusception, bowel obstruction, and volvulus. The current treatment of choice for gastrointestinal duplication cysts in symptomatic pediatric patients is surgical excision, whereas
there is currently no standardized evidence-based guideline for managing incidentally detected gastrointestinal duplication cysts in asymptomatic pediatric patients.
Conclusion
Focal cystic abdominal masses are frequent disorders that can be symptomatic or incidental findings in pediatric patients. Imaging evaluation can provide precise information regard-
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Ranganath et al.
Fig. 28Colonic duplication cyst. Contrastenhanced axial CT image in 2-year-old boy with
right lower quadrant pain to evaluate of possible
appendicitis shows round cystic mass (C) within right
colon surrounded by oral contrast material.
ing their location, appearance, size, and mass effect on adjacent abdominal structures. This
information is crucial for early and correct diagnosis, which, in turn, can lead to optimal patient management.
Selected Reading
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tumors of the kidney in children: CT findings.
AJR 1995; 164:697700
3. Decter RM. Renal duplication and fusion anomalies. Pediatr Clin North Am 1997; 44:13231341
4. Elsayes KM, Menias CO, Dillman JR, et al. Vascular malformation and hemangiomatosis syndromes: spectrum of imaging manifestations.
AJR 2008; 190:12911299
5. Han TI, Kim MJ, Yoon HK, et al. Rhabdoid tumour of the kidney: imaging findings. Pediatr
Radiol 2001; 31:233237
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tumors. RadioGraphics 1999; 19:431451
7. Koumanidou C, Vakaki M, Papadaki M, et al.
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8. Murphy BJ, Casillas J, Ros PR, et al. The CT ap-
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