Title: Renal Masses
1Renal Masses
- Robert D. Thomas MD
- Pediatric Radiology
2Renal Masses
- Balls
- Cyst
- Hematoma
- Abscess
- Tumor
- Dromedary hump
- Beans
- Duplication/anomaly
- Compensatory hypertrophy
- Hydronephrosis
- Pyelonephritis/edema
- Hematoma
- PCKD
- Tumor
- Vascular occlusion/trauma
3Renal Masses by Age
- Newborn
- Hydronephrosis
- MCDK
- AR-PCKD
- Anomalies
- Tumors
- Mesoblastic nephroma
- Nephroblastomatosis
- Childhood
- Cysts
- Hydronephrosis, MCDK
- Anomalies
- Hematoma
- Tumors
- Wilms
- Lymphoma
- Angiomyolipomas
4Hydronephrosis(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)
5Congenital 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.
6Congenital 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
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11Congenital 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
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15Multicystic Dysplastic KidneyBean 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
16Multicystic 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)
17Multicystic 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 24-48hrs)
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20Multicystic 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
21Solid Renal MassesBeans and Balls!
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23R/P mass in Neonate
- Renal
- Hydronephrosis
- Multicystic dysplastic kidney
- Solid
- Wilms tumor?
- Perinephric hematoma?
- Mesoblastic nephroma?
- Lymphoma?
- Adrenal
- Hemorrhage
- neuroblastoma
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27Mesoblastic 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
28Mesoblastic Nephroma(Fetal renal hamartoma)
- Imaging
- Non-calcified abdominal mass
- Look like uterine leiomyoma by US
- CT vascular and entrapped collecting system
excretes contrast
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30Mesoblastic Nephroma(Fetal renal hamartoma)
- Management
- Nephrectomy
- No chemo or radiation (usually no mets)
- Cellular form
- Age gt3months at surgery are more likely to need
chemo/radiation
31Childhood Renal Tumors
- Wilms tumor nephroblastomatosis
- Renal lymphoma/leukemia
- Renal cell carcinoma
- Multilocular cystic nephroma
- Clear cell sarcoma
- Rhabdoid tumor
- Angiomyolipoma (and tuberous sclerosis)
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33Wilms Tumor
- Most common solid abdominal mass in childhood
- Most common renal malignancy in child
- 8 of all childhood cancer
34Wilms Tumor
- Demographics
- Malefemale
- 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
35Wilms 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)
36Wilms Tumor
- Nephroblastoma (Wilms in situ)
- Rests of metanephric blastema persisting after
34-36 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
37Wilms 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
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40NR 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
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43Nephroblastomatosis
- Treatment
- Confluent disease treated with chemotherapy
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45Wilms 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
46Wilms Tumor
- Pathology
- 5 bilateral
- 7 unilateral and multicentric
- Metachronous cases may occur up to 10 years later
- 10 unfavorable histology
47Wilms 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)
48Wilms 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
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50Wilms Tumor - Radiology
- Nitwits (NWTS) dont agree on optimal imaging
nonsense like IVPs persist - IVP distortion of collecting system,
non-function (vascular compression) - US
- CDS excellent for venous tumor thrombi in IVC
- Echotexture similar to liver
- Sharply marginated
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54Wilms 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
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56Wilms 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
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58Wilms 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 (lt1 incidence
after 10)
59Wilms Tumor - Treatment
- Overall survival now 90
- gt90 survival _at_ 2 yrs with favorable histology,
surgery, chemo and radiation - High mortality with unfavorable histology
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61Renal Lymphoma
- Usually late in NHL
- Nodules, masses, diffuse infiltration
- Unilateral/bilateral
- US hypoechoic
- CT hypodense
- Leukemia usually diffuse/bilateral
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65Multilocular Cystic Nephroma
- Indistinguishable from cystic partially
differentiated nephroblastoma/cystic Wilms - Young boys and adult women
- Anechoic cysts with regular septa
- Rx - nephrectomy
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67Clear Cell Sarcoma
- Identical age group to Wilms
- Very aggressive
- Not distinguishable from Wilms by imaging
- Bone mets common
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69Other 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
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75Other lesions to ponder
- Duplication
- Hematoma/renal trauma
- Pyelonephritis
- Focal bacterial
- Xanthogranulomatous
- Autosomal recessive polycystic kidney dz
- Infantile form
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