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Robert D. Thomas MD Pediatric Radiology
Renal Masses Robert D. Thomas MD Pediatric Radiology
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Renal Masses Balls Beans Cyst Hematoma Abscess Tumor Dromedary hump
Duplication/anomaly Compensatory hypertrophy Hydronephrosis Pyelonephritis/edema Hematoma PCKD Tumor Vascular occlusion/trauma
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Renal Masses by Age Newborn Childhood Hydronephrosis Cysts
MCDK AR-PCKD Anomalies Tumors Mesoblastic nephroma Nephroblastomatosis Childhood Cysts Hydronephrosis, MCDK Anomalies Hematoma Tumors Wilms Lymphoma Angiomyolipomas
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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)
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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.
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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
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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
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Multicystic Dysplastic Kidney Bean or Ball
Not a true “cystic disease” etiology is severe embryonic obstruction during metanephric stage of development So…it’s an obstruction Hallmark: non-function of the kidney Bilaterality not compatible with life due to severe pulmonary hypoplasia
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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%)
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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 24-48hrs)
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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
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Solid Renal Masses Beans and Balls!
Hematoma Abscess Tumor
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R/P mass in Neonate Renal Adrenal Hydronephrosis
Multicystic dysplastic kidney Solid Wilms tumor? Perinephric hematoma? Mesoblastic nephroma? Lymphoma? Adrenal Hemorrhage neuroblastoma
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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
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Mesoblastic 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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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
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Childhood 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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Wilms Tumor Most common solid abdominal mass in childhood
Most common renal malignancy in child 8% of all childhood cancer
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Wilms Tumor Demographics Presentation 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
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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 children’s siblings have a 30% chance of development of Wilms Nephroblastomatosis (Wilms precursor)
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Wilms Tumor Nephroblastoma (Wilms “in situ”)
Rests of metanephric blastema persisting after 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
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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
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NR versus Wilms at MRI NR Wilms Plaque-like Ovoid Lenticular
Homogeneous on all sequences Hypotense post gad Wilms Round/spherical Heterogeneous pre gad Heterogeneous post contrast
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Nephroblastomatosis Treatment
Confluent disease treated with chemotherapy
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Wilms Tumor Pathology Solid, necrosis, hemorrhage, 15% calcifications
Capsule usually intact Invades nodes, veins, rarely urothelium Decreasing 10’s 10% renal vein invasion 10% IVC extension 10% right atrial extension
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Wilms Tumor Pathology 5% bilateral 7% unilateral and multicentric
Metachronous cases may occur up to 10 years later 10% unfavorable histology
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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)
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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
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Wilms Tumor - Radiology
Nitwits (NWTS) don’t agree on optimal imaging – nonsense like IVP’s 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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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
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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
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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)
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Wilms Tumor - Treatment
Overall survival now 90% >90% 2 yrs with favorable histology, surgery, chemo and radiation High mortality with unfavorable histology
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Renal Lymphoma Usually late in NHL
Nodules, masses, diffuse infiltration Unilateral/bilateral US – hypoechoic CT – hypodense Leukemia usually diffuse/bilateral
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Multilocular 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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Clear Cell Sarcoma Identical age group to Wilms Very aggressive
Not distinguishable from Wilms by imaging Bone mets common
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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
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Other lesions to ponder
Duplication Hematoma/renal trauma Pyelonephritis Focal bacterial Xanthogranulomatous Autosomal recessive polycystic kidney dz Infantile form
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