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PEDIATRIC RENAL TUMORS Presenter- Dr. Bipin Joseph Moderator- Dr. Anto Richie

Pediatric renal tumors radiology

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Text of Pediatric renal tumors radiology

PEDIATRIC RENAL TUMORS

PEDIATRIC RENAL TUMORSPresenter- Dr. Bipin JosephModerator- Dr. Anto Richie

Neonate Mesoblastic nephroma Multicystic dysplastic kidney

6 months to 5 years of ageWILMS TUMOR Nephroblastomatosis Medullary tumors Multilocular cystic nephroma

> 5 years Renal cell cancer

WILMS TUMOREpidemiology85% of renal masses6-7% of all childhood cancersApproximately 500 cases/year Deletion of tumor suppressor genes on short arm of chromosome 1111p13 locus (WT1 gene) 11p15 locus (WT2 gene)

Risk FactorsAniridia

WAGR syndrome (aniridia, genital abnormalities, retardation) (WT1 gene)

Beckwith-Wiedemann syndrome (hemihypertrophy, omphalocele, macroglossia, hypoglycemia) & isolated hemihypertrophy (WT2 gene)

Drash syndrome (nephritis & male pseudohermaphrodism) (WT 1 gene)

Gross Features Large cortical mass(400 to 500 gm)Hemorrhage & necrosis(90%)PseudocapsuleSpares collecting systemCalcification & fat 3x size Hyperchromatic nuclei Atypical mitotic figures Implies poor prognosis & resistance to conventional therapy

Wilms Tumor: Clinical FeaturesMean age: 36 m (range, 6 m-4 yr)Symptoms Mass: 90% Pain, fever, hematuria: 30% Hypertension: 75% - 90% Aniridia, hemihypertrophy Budd-Chiari syndrome

Wilms Tumor: US Findings

Well-defined marginsSolid, intrarenal massTumor matrix Homogeneous: 50% Heterogeneous : 50%IVC thrombus (5-10%)Relatively avascular

Wilms Tumor

Long n transverse10

Wilms Tumor

Wilms tumor in a 4-year-old boy with an abdominal mass. (a) CT scan shows a left renal mass withheterogeneous enhancement (thick arrow) and multiple hepatic metastases (thin arrows). (b) CT scan obtained at ahigher level again shows multiple hepatic metastases in addition to tumor thrombus within the portal veins (arrows).11

Wilms Tumor

Wilms tumor in a 3-year-old boy with anabdominal mass. Gadolinium-enhanced coronal fatsuppressedT1-weighted MR image shows a large,well-defined mass in the right kidney (arrows) that enhancesless than adjacent renal parenchyma and containsmultiple hypointense hemorrhagic foci. The hypointensearea in the medial spleen is due to partialvolume artifact.12

Wilms Tumor: Venous Extension 5-10% of cases Tumor thrombus aboveIVC alters surgicalapproach Cardiopulmonary bypass

Wilms Tumor: Caval Thrombus

Tumor Thrombus

Bilateral Wilms Tumor

5-10% of patients (synchronous disease)

Dominant renal mass & small contralateral tumor or bilateral large masses

Alters therapy initial chemotherapy delayed surgery

Wilms Tumor: Treatment En bloc resection of affected kidney Excision of nodes and lung nodules Postop chemotherapyPreoperative chemotherapy for invasive disease or bilateral Wilms, then surgery For all stages 2-year survival of 90%

NEPHROBLASTOMATOSIS

Defined as "the presence of nephrogenic rests or nephrogenic blastema beyond 36 weeks gestationImportant because it is a precursor to Wilms tumorPatient age < 2 years

Nephrogenic RestsLocation2 types by locationPerilobar Peripheral cortex or columns of BertinIntralobar Deep cortex Greater risk ofWilms tumor (4-5%)

Most cases of nephroblastomatosis occur sporadically.However, perilobar nephroblastomatosis is associated with Beckwith-Wiedemannsyndrome, hemihypertrophy, and Perlman syndrome. Intralobar nephroblastomatosis usuallyoccurs as a single poorly marginated mass that can be found anywhere in the renal lobe. It isassociated with Drash syndrome, sporadic aniridia, and WAGR (Wilms tumor, aniridia, genitourinaryanomalies, and mental retardation) syndrome.

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Nephrogenic Rests

wilms20

Nephroblastomatosis: Imaging

Two basic appearances: Confluent peripheral tumor Focal peripheral cortical mass

USG: hypoechoic

nephroblastomatosis can present as a single hypoechoic nodule or multiplenodules or as nephromegaly with diffuse hypoechogenicity and obscuration of the corticomedullaryjunction21

Diffuse Nephroblastomatosis

CT, perilobar nephroblastomatosis appears as peripheral renal masses with low attenuation andmild contrast enhancement22

Focal Nephroblastomatosis:Discrete Cortical Nodules

Small size, peripheral location BUTneed biopsy to rule out Wilms23

Nephroblastomatosis: Treatment

Controversial Chemotherapy in some centers Close imaging surveillance forenlarging masses in others Renal sparing surgery

MESOBLASTIC NEPHROMA

5% of all renal tumorsTumor of the neonate mean age, 2 months(first 6 m)Incidental massMay be diagnosed in utero - polyhydramnios

Clinically palpable flank massHypertensionhematuria hypercalcemia

Mesoblastic Nephroma: Path

GrossCortical tumor Unencapsulated Hemorrhage & necrosis uncommon Histology mature spindle cells

Mesoblastic Nephroma: Imaging

Usually homogeneouscystic changes rareWell circumscribedLooks like Wilms but age is the clue

usg- a mesoblastic nephroma appears as a solid-tissue mass with smooth margins. often have an echogenicity similar to that ofnormal muscle28

Mesoblastic Nephroma

Axial contrast-enhanced CT image shows large hypodense mass (asterisk) arising from right kidney. Enhancement pattern is variableMesoblastic nephromas show intermediate tolow signal intensity on T1-weighted imagesand are hyperintense on T2-weighted images.MR may be the most useful imaging modalityfor confirming the renal origin of the mass29

Mesoblastic Nephroma:Therapy and Prognosis

Most behave in benign fashionCured by nephrectomy Metastases & recurrence very rare Associated with atypical cellular histologyOverall prognosis excellent

TUMORS OF MEDULLARY ORIGIN

6% of renal neoplasms

Rhabdoid tumorClear cell sarcoma

Malignant rhabdoid tumor

infants associated with CNS tumors (PNET)Large masses Infiltrating margins

Rhabdoid Tumor:

Heterogeneous mass Peripheral low density crescent Ill-defined margins

Rhabdoid tumorin 3-year-old boy whopresented with palpableabdominal mass. Axialcontrast-enhanced CTimage shows large massarising from left kidneycausing protrusion ofleft abdominal wall.Mass has predominantlyhypodense centersuggestive of necrosis(asterisk). There is rim ofnormally enhancing renalparenchyma (arrows)posteriorly. Patientalso was found to havecerebellar neoplasm.34

Rhabdoid tumor Poor prognosis 80% have metastases at diagnosis lung, liver, brain, nodes 20% survival @ 18 months

Clear cell sarcoma

1st 4 years of life bone metastases (60-70%)Infiltrative aggressive tumors

Clear Cell Sarcoma

Heterogeneous massCystic changes (70%) Ill-defined margins Bone metastases

Clear cell sarcoma Survival rates 60%-70%

Clear cell sarcoma in 2-year-old girl who presented with palpable right flank mass.A, Axial contrast-enhanced CT image shows large heterogeneous mass (M) arising from right kidney.B, Axial contrast-enhanced CT image obtained slightly more inferiorly shows thin rim of renal parenchyma(arrows) remains. Hypodense areas (asterisk) are suggestive of necrosis. M = heterogeneous mass.38

Cystic Masses

< 5 years of age Multilocular cystic renal tumor Multicystic dysplastic kidney

> 5 years of age Simple renal cysts (rare)

MULTILOCULAR CYSTIC RENAL TUMORThe term multilocular cystic renal tumorencompasses two histologically distinct cystictumors: cystic nephromas composed of epithelial lined cysts separated by fibrous septa with mature tubules cystic partially differentiated nephroblastomas composed of cysts with septa containing foci of blastemal cells.

Imaging cannotdistinguish between the two types of multilocular cystic renal tumor40

Multilocular Cystic Renal Tumor:

Clinical Findings Biphasic age distribution Boys 3 months to 2 years Women > 40 years Usually asymptomatic massPain & hematuria from ureteral prolapse of cyst

Multilocular Cystic Renal Tumor

Composed of cysts & septa Encapsulated Mean diameter 7 to 10 cm

Multilocular Cystic Renal Tumors

Cystic, fluid-filled mass Varying thickness septationsAvascular, except septa

Multilocular Cystic Renal Tumor

On CT, multilocularcystic renal tumor appears as a well-circumscribed cystic mass containing multiple thin minimallyenhancing septations (Fig. 6B). Occasionally, septal or wall calcifications can be seen44

Axial contrast-enhanced CT image of left kidney in excretory phase reveals large multiseptated cystic massarising from left kidney with classic finding of herniation (asterisk) of tumor into collecting system. Rim ofnormal left renal tissue (arrows) remains anteriorly.45

Multilocular Cystic Renal Tumor:Differential Diagnosis

Multicystic dysplastic kidney Non-functioning Entire kidney involved Conservative treatment, not surgery Found in neonates (day 1) Typically involutes

MCDK: Gross Pathology & Imaging

Cysts of varying sizesRandomly arranged Non-communicating No normal parenchyma Associated atresia of ureter or pelvis

ANGIOMYOLIPOMA

Fat-containing MassesAny age, but more often after 5 yearsAssociated with tuberous sclerosis80% of tuberous sclerosis patients have angiomyolipomasalso are associated (although to a lesser extent) with von Hippel-Lindau syndrome and neurofibromatosis.

Angiomyolipoma: USG

Angiomyolipomasin 9-year-old girl whopresented with tuberoussclerosis. Sagittalposterior ultrasoundimage of left kidney showsmultiple small echogenicfoci (arrows) scatteredthrough renal cortexand hyperechoic mass(curved arrow) in lowerpole. These echogenicareas are consistent withmacroscopic fat. Asteriskdenotes posterioracoustic shadow from rib.Imaging:Bilateral fatty, renal massesSolitary lesions, very rareOn ultrasound, an angiomyolipoma often appears as a well-circumscribed hyperechoicmass. However, small angiomyolipomas may not be reliably diagnosed by ultrasound49

Angiomyolipoma: CT

ngiomyolipomasin 17-year-old girl whopresented with knowntuberous sclerosis. Axialcontrast-enhancedCT image shows largepredominantly fattymasses (asterisks) inboth kidneys. Additionalsmaller hypodenselesions (arrows) are seenscattered throughoutcortex, presumablyrepresenting smallangiomyolipomas50

Because of the increased risk of hemorrhage with large angiomyolipomas, the current recommendation is to treat an angiomyolipomagreater than 4 cm with surgical resection or selective catheter embolization. InThus, ultrasound surveillance is currently recommended every 23 years before puberty and yearly thereafter to detect any increase in the size of the mass.

pediatric patients with tuberous sclerosis, angiomyolipomas may become locally aggressive.

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OSSIFYING RENAL TUMOR OF INFANCYOssifying renal tumor of infancy is an extremely rare lesion found in infants as young as 6days to 14 months. The tumor consists of an osteoid core, osteoblast, and spindle cellsusually presents as an abdominal mass that may be accompanied by hematuriaOn ultrasound, ossifying renal tumor of infancy presents as a heterogeneously echogenic intrarenal mass with posterior acoustic shadowing due to the calcified components of the tumor

OSSIFYING RENAL TUMOR OF INFANCY

Ossifying renaltumor of infancy in10-month-old girl whopresented with palpableleft flank mass. Axialcontrast-enhanced CTimage shows partiallycalcified soft-tissue mass(arrow) in medial leftkidney that extends intorenal collecting systemand causes obstructivehydronephrosis (H).53

TUMORS OF OLDER CHILDREN-- RENAL CELL CANCER

Peak incidence: 15-19 yr

Clinical findingspain, hematuria, mass

Imaging findings:solid renal masssame as adult

RENAL CELL CARCINOMA (RCC) Is extremely rare in children. It usually occurs in periadolescent children but may develop in younger children with von Hippel-Lindau syndromein children who have undergone prior chemotherapy for other renal tumorsPainless hematuria, flank pain, or a palpable mass are the usual presenting symptoms of RCC.

Renal cell carcinoma in 11-year-old boy who presented with hematuria.A, Sagittal posterior ultrasound image shows circumscribed heterogeneously echogenic mass (M) involvingupper pole of left kidney.B, Corresponding axial contrast-enhanced CT image shows left renal mass (M) as heterogeneously enhancingbut predominantly hypodense, focally distorting renal contour (arrow).56

Most common metastatic diseases

NeuroblastomaLeukemiaLymphoma

NEUROBLASTOMAarise from primitive neuroblasts in the neural crest of sympathetic ganglia.malignancy; 80% of cases occur in children under 5 years old. Neuroblastoma most frequently presents as an abdominal mass, with two thirds of children having metastatic disease at the time of presentation.

After leukemia and primary brain tumors, neuroblastoma is the third most common childhood

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Neuroblastoma often displaces the ipsilateral kidney,although it can involve the kidney by direct extension and may encase the renal arteries or ureters to cause hypertension or hydronephrosisThese hematogenous metastases appear as well-circumscribed hyperechoic renal lesions on ultrasound and hypoenhancing masses on contrastenhanced CT

Wilms' tumor vs NeuroblastomaWilms' 5 yearsRenal cell cancer

Infants & Young Children:Which Solid Renal Tumor?

Homogeneous solid mass? Wilms, mesoblastic nephroma Subcapsular or peripheral solid tumor? NephroblastomatosisLarge cystic areas or bone mets? Clear cellPeripheral fluid/cystic collection? Rhabdoid Infiltrative margins? Rhabdoid/clear cell sarcomaWell circumscribed margins? Wilms, mesoblastic nephroma

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