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Renal Masses

HENDRA MD
Pediatric Radiology

Renal Masses
Balls
Cyst
Hematoma
Abscess
Tumor
Dromedary hump

Beans
Duplication/anomaly
Compensatory
hypertrophy
Hydronephrosis
Pyelonephritis/edema
Hematoma
PCKD
Tumor
Vascular
occlusion/trauma

Renal Masses by Age


Newborn
Hydronephrosis
MCDK
AR-PCKD
Anomalies
Tumors

Mesoblastic
nephroma
Nephroblastomatosis

Childhood
Cysts
Hydronephrosis,
MCDK
Anomalies
Hematoma
Tumors

Wilms
Lymphoma
Angiomyolipomas

Hydronephrosis
(Bean)
Calyceal/Pelvic obstruction
Congenital (intrinsic/extrinsic)
TB
Tumor
Ureter
Physiologic (full bladder)
Congenital (1 megaureter, ectopic ureter,
retrocaval)
Inflammatory (TB, Crohn, PID, etc)
Intraluminal (stone, clot, tumor, stricture)

Congenital UPJ obstruction


#1 cause of renal mass in newborn
Associations

Ipsilateral reflux
Lower moiety of duplication
Most common cause of obstruction with
horseshoe kidney

Causes
Stricture, disordered peristalsis, ischemia,
redundant urothelium, crossing vessel, etc.

Congenital UPJ obstruction


Imaging:
Mass in plain films
US dilated pelvo-calyceal system
(communicating cysts): dilatation-fluid equal to
cortical thickness
NM obstructive pattern w/o lasix response
Pitfalls
US may underestimate hydro due to
oliguria/dehydration in newborn
MCDK may look like UPJ if only a couple cysts
present

Congenital UPJ obstruction


Work-up
VCUG:

co-existant ipsilateral reflux*,


urethral obstruction, contralateral reflux
Scintigraphy: site of obstruction & renal
function
*obstruction

to reflux at UPJ, dilution of


contrast in dilated renal pelvis, delay in
drainage from renal pelvis

Multicystic Dysplastic Kidney


Bean or Ball
Not a true cystic disease
etiology is severe embryonic

obstruction during metanephric stage of


development
Soits an obstruction
Hallmark: non-function of the kidney
Bilaterality not compatible with life due
to severe pulmonary hypoplasia

Multicystic Dysplastic Kidney


2nd most common renal mass in newborn
Types

Pelvoinfundibular atresias at ureter, pelvis,


infundibulae

Most common, grape-like collection of cysts and


dysplastic glomeruli, atrophied tubules

Hydronephrotic-atresia of proximal ureter alone

Uncommon (5%)

Multicystic Dysplastic Kidney


Imaging
US

- Isolated cysts without a definable


pelvis and without normal renal tissue
IVP lack of function
NM absence of perfusion & lack of
function (may have minimal activity 2448hrs)

Multicystic Dysplastic Kidney


Work-up
US: frequent contralateral UPJ, reflux,
VCUG: opposite reflux/obstruction
MAG3, DTPA renogram
Management
Usually observation (natural history of involution)
Nephrectomy for GI obstruction/respiratory
compromise, hypertension
?malignancy probably not increased over baseline

Solid Renal Masses


Beans and Balls!
Hematoma
Abscess
Tumor

R/P mass in Neonate


Renal
Hydronephrosis
Multicystic dysplastic kidney
Solid

Wilms tumor?
Perinephric hematoma?
Mesoblastic nephroma?
Lymphoma?

Adrenal

Hemorrhage
neuroblastoma

Mesoblastic Nephroma
(Fetal renal hamartoma)
Most common neonatal renal neoplasm
Present as an asymptomatic mass
Not Wilms tumor
Characteristics
Benign appearing spindle cells with dysplastic
nephrons
Large (8-30cm), arise in medulla
Blends with normal parenchyma
May penetrate capsule and invade locally
Rare hypercellular forms may metastasize

Mesoblastic Nephroma
(Fetal renal hamartoma)
Imaging
Non-calcified

abdominal mass
Look like uterine leiomyoma by US
CT vascular and entrapped collecting
system excretes contrast

Mesoblastic Nephroma
(Fetal renal hamartoma)
Management
Nephrectomy
No

chemo or radiation (usually no mets)


Cellular form

Age >3months at surgery are more likely to


need chemo/radiation

Childhood Renal Tumors


Wilms tumor & nephroblastomatosis
Renal lymphoma/leukemia
Renal cell carcinoma
Multilocular cystic nephroma
Clear cell sarcoma
Rhabdoid tumor
Angiomyolipoma (and tuberous sclerosis)

Wilms Tumor
Most common solid abdominal mass in

childhood
Most common renal malignancy in child
8% of all childhood cancer

Wilms Tumor
Demographics
Male=female
1% familial
7.8 per 1,000,000 children
Peaks between 2.5 to 3 years
80% occur between 1-5 years
Presentation

Asymptomatic mass most common


Other: pain, hematuria, hypertension, fever

Wilms Tumor
Associated conditions
8% have overgrowth disorders, genital
anomalies, aniridia
Drash, Beckwith-Wiedemann, Soto, NF,
KTW, Bloom, WAGR, 45X, etc
5% bilateral & higher incidence of above

These childrens siblings have a 30% chance of


development of Wilms

Nephroblastomatosis

(Wilms precursor)

Wilms Tumor
Nephroblastoma (Wilms in situ)
Rests of metanephric blastema persisting after 3436 weeks gestational age
Present in most cases of bilateral Wilms, 15%
unilateral disease
Intralobular NR

Younger age
Drash & sporadic aniridia
Metachronous Wilms

Perilobular NR

BWS, Tr18, hemihypertrophy


Synchronous Wilms

Wilms Tumor
Nephroblastomatosis
ImagingAppearance

Nodules
Subcapsular hypodense plaques

US

iso, hypo, hyperechoic (relatively


insensitive)
CT w contrast better for surveillance
MRI ? Able to distinguish Wilms from
nephroblastomatosis

NR versus Wilms at MRI


NR
Plaque-like
Ovoid
Lenticular
Homogeneous on all
sequences
Hypotense post gad

Wilms
Round/spherical
Heterogeneous pre
gad
Heterogeneous post
contrast

Nephroblastomatosis
Treatment
Confluent

disease treated with


chemotherapy

Wilms Tumor
Pathology
Solid,

necrosis, hemorrhage, 15%


calcifications
Capsule usually intact
Invades nodes, veins, rarely urothelium
Decreasing 10s

10% renal vein invasion


10% IVC extension
10% right atrial extension

Wilms Tumor
Pathology
5%

bilateral
7% unilateral and multicentric
Metachronous cases may occur up to 10
years later
10% unfavorable histology

Wilms Tumor
Pathology
Lung

mets up to 20% at diagnosis


Liver mets 10% of patients
Bone mets rare (lytic)
Bilateral tumors may have different grades
of histology (favorable vs unfavorable)

Wilms Tumor
Staging
I

limited to kidney, completely resected


II- outside kidney, completely resected
III confined to abdomen
IV hematogenous mets
V bilateral initial/during treatment

Wilms Tumor - Radiology


Nitwits (NWTS) dont agree on optimal

imaging nonsense like IVPs persist


IVP distortion of collecting system, nonfunction (vascular compression)
US

CDS excellent for venous tumor thrombi in IVC


Echotexture similar to liver
Sharply marginated

Wilms Tumor - Radiology


CT
15%

contain calcifications
Round, hetergeneous, low density
Displaces vessels, does NOT encase
(DDX from neuroblastoma)
Best for opposite kidney evaluation, nodes,
lungs

Wilms Tumor-Radiology
MRI
Becoming

preferred over CT
Prolonged T1 and T2, heterogeneous post
gad
Excellent for NR of 4 mm size
Angio
Plays

a role for partial nephrectomy

Wilms Tumor - Surveillance


Patients with syndromes associated

with Wilms
US easiest, MRI may be best
Arbitrary 3-6 month scans
Continue until about 10 years old (<1%
incidence after 10)

Wilms Tumor - Treatment


Overall survival now 90%
>90% survival @ 2 yrs with favorable
histology, surgery, chemo and radiation
High mortality with unfavorable histology

Renal Lymphoma
Usually late in NHL
Nodules, masses, diffuse infiltration
Unilateral/bilateral
US hypoechoic
CT hypodense
Leukemia usually diffuse/bilateral

Multilocular Cystic Nephroma


Indistinguishable from cystic partially

differentiated nephroblastoma/cystic
Wilms
Young boys and adult women
Anechoic cysts with regular septa
Rx - nephrectomy

Clear Cell Sarcoma


Identical age group to Wilms
Very aggressive
Not distinguishable from Wilms by

imaging
Bone mets common

Other lesions to ponder


Simple cyst
Were considered rare prior to ultrasound
But, the differential diagnosis is:
Prior trauma or infection
Obstructed upper pole moiety of duplication
Early presentation of familial cystic disease

Other lesions to ponder


Duplication
Hematoma/renal trauma
Pyelonephritis
Focal

bacterial
Xanthogranulomatous
Autosomal recessive polycystic kidney

dz

Infantile

form

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