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Renal Masses. Robert D. Thomas MD Pediatric Radiology. Balls Cyst Hematoma Abscess Tumor Dromedary hump. Beans Duplication/anomaly Compensatory hypertrophy Hydronephrosis Pyelonephritis/edema Hematoma PCKD Tumor Vascular occlusion/trauma. Renal Masses. Newborn Hydronephrosis

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renal masses

Renal Masses

Robert D. Thomas MD

Pediatric Radiology

renal masses1
Balls

Cyst

Hematoma

Abscess

Tumor

Dromedary hump

Beans

Duplication/anomaly

Compensatory hypertrophy

Hydronephrosis

Pyelonephritis/edema

Hematoma

PCKD

Tumor

Vascular occlusion/trauma

Renal Masses
renal masses by age
Newborn

Hydronephrosis

MCDK

AR-PCKD

Anomalies

Tumors

Mesoblastic nephroma

Nephroblastomatosis

Childhood

Cysts

Hydronephrosis, MCDK

Anomalies

Hematoma

Tumors

Wilms

Lymphoma

Angiomyolipomas

Renal Masses by Age
hydronephrosis bean
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
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 obstruction1
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 obstruction2
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
Multicystic Dysplastic KidneyBean 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
multicystic dysplastic kidney
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 kidney1
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)
multicystic dysplastic kidney2
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
r p mass in neonate
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
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 hamartoma1
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 hamartoma2
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
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
Wilms Tumor
  • Most common solid abdominal mass in childhood
  • Most common renal malignancy in child
  • 8% of all childhood cancer
wilms tumor1
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 tumor2
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)
wilms tumor3
Wilms 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
wilms tumor4
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

NR versus Wilms at MRI
nephroblastomatosis
Nephroblastomatosis
  • Treatment
    • Confluent disease treated with chemotherapy
wilms tumor5
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
wilms tumor6
Wilms Tumor
  • Pathology
    • 5% bilateral
    • 7% unilateral and multicentric
    • Metachronous cases may occur up to 10 years later
    • 10% unfavorable histology
wilms tumor7
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 tumor8
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
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
wilms tumor radiology1
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 radiology2
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
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
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
Renal Lymphoma
  • Usually late in NHL
  • Nodules, masses, diffuse infiltration
  • Unilateral/bilateral
  • US – hypoechoic
  • CT – hypodense
  • Leukemia usually diffuse/bilateral
multilocular cystic nephroma
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
Clear Cell Sarcoma
  • Identical age group to Wilms
  • Very aggressive
  • Not distinguishable from Wilms by imaging
  • Bone mets common
other lesions to ponder
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 ponder1
Other lesions to ponder
  • Duplication
  • Hematoma/renal trauma
  • Pyelonephritis
    • Focal bacterial
    • Xanthogranulomatous
  • Autosomal recessive polycystic kidney dz
    • Infantile form