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Renal Masses

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Dromedary hump. Beans. Duplication/anomaly. Compensatory hypertrophy. Hydronephrosis ... Stricture, disordered peristalsis, ischemia, redundant urothelium, ... – PowerPoint PPT presentation

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Title: Renal Masses


1
Renal Masses
  • Robert D. Thomas MD
  • Pediatric Radiology

2
Renal Masses
  • Balls
  • Cyst
  • Hematoma
  • Abscess
  • Tumor
  • Dromedary hump
  • Beans
  • Duplication/anomaly
  • Compensatory hypertrophy
  • Hydronephrosis
  • Pyelonephritis/edema
  • Hematoma
  • PCKD
  • Tumor
  • Vascular occlusion/trauma

3
Renal Masses by Age
  • Newborn
  • Hydronephrosis
  • MCDK
  • AR-PCKD
  • Anomalies
  • Tumors
  • Mesoblastic nephroma
  • Nephroblastomatosis
  • Childhood
  • Cysts
  • Hydronephrosis, MCDK
  • Anomalies
  • Hematoma
  • Tumors
  • Wilms
  • Lymphoma
  • Angiomyolipomas

4
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)

5
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.

6
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 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

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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)

17
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

21
Solid Renal MassesBeans and Balls!
  • Hematoma
  • Abscess
  • Tumor

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R/P mass in Neonate
  • Renal
  • 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 gt3months at surgery are more likely to need
    chemo/radiation

31
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
  • 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

35
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)

36
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

37
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
  • 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 10s
  • 10 renal vein invasion
  • 10 IVC extension
  • 10 right atrial extension

46
Wilms Tumor
  • Pathology
  • 5 bilateral
  • 7 unilateral and multicentric
  • Metachronous cases may occur up to 10 years later
  • 10 unfavorable histology

47
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)

48
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) 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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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 (lt1 incidence
    after 10)

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Wilms 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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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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