Transcript
Page 1: Pediatric renal tumors radiology

PEDIATRIC RENAL TUMORS

Presenter- Dr. Bipin JosephModerator- Dr. Anto Richie

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Neonate – Mesoblastic nephroma – Multicystic dysplastic kidney

6 months to 5 years of age –WILMS TUMOR – Nephroblastomatosis – Medullary tumors – Multilocular cystic nephroma

> 5 years – Renal cell cancer

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WILMS TUMOR

Epidemiology 85% of renal masses 6-7% of all childhood cancers Approximately 500 cases/year Deletion of tumor suppressor

genes on short arm of chromosome

11 11p13 locus (WT1 gene) 11p15 locus (WT2 gene)

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Risk Factors Aniridia

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)

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Gross Features Large cortical mass(400 to 500

gm) Hemorrhage & necrosis(90%) Pseudocapsule Spares collecting system Calcification & fat <10%

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Favorable Histology (85%) Triphasic composition – Metanephric blastema – Immature stroma – Tubular elements Good prognosis if triphasic

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“Unfavorable” Histology (15%) Anaplastic changes – Nuclear enlargement »> 3x size –Hyperchromatic nuclei – Atypical mitotic figures Implies poor prognosis & resistance to conventional therapy

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Wilms Tumor: Clinical Features Mean age: 36 m (range, 6 m-4

yr) Symptoms – Mass: 90% – Pain, fever, hematuria: 30% –Hypertension: 75% - 90% – Aniridia, hemihypertrophy – Budd-Chiari syndrome

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Wilms Tumor: US Findings

Well-defined margins Solid, intrarenal mass Tumor matrix –Homogeneous: 50% –Heterogeneous : 50% IVC thrombus (5-10%) Relatively avascular

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Wilms Tumor

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Wilms Tumor

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Wilms Tumor

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Wilms Tumor: Venous Extension • 5-10% of cases • Tumor thrombus above IVC alters surgical approach – Cardiopulmonary bypass

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Wilms Tumor: Caval Thrombus

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Tumor Thrombus

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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

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Wilms Tumor: Treatment En bloc resection of affected

kidney Excision of nodes and lung

nodules Postop chemotherapy Preoperative chemotherapy for

invasive disease or bilateral Wilms, then

surgery For all stages 2-year survival of

90%

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NEPHROBLASTOMATOSIS

Defined as "the presence of nephrogenic

rests or nephrogenic blastema beyond 36

weeks gestation” Important because it is a

precursor to Wilms tumor Patient age < 2 years

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Nephrogenic Rests

Location2 types by location Perilobar – Peripheral cortex or columns of Bertin Intralobar – Deep cortex – Greater risk ofWilms tumor (4-

5%)

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Nephrogenic Rests

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Nephroblastomatosis: Imaging

Two basic appearances: –Confluent peripheral tumor –Focal peripheral cortical mass

USG: hypoechoic

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Diffuse Nephroblastomatosis

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Focal Nephroblastomatosis:Discrete Cortical Nodules

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Nephroblastomatosis: Treatment

Controversial Chemotherapy in some centers Close imaging surveillance

forenlarging masses in others Renal sparing surgery

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MESOBLASTIC NEPHROMA

5% of all renal tumors Tumor of the neonate – mean age, 2 months(first 6

m) Incidental mass May be diagnosed in utero - polyhydramnios

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Clinically palpable flank mass Hypertension hematuria hypercalcemia

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Mesoblastic Nephroma: Path

Gross Cortical tumor Unencapsulated Hemorrhage & necrosis uncommon Histology – mature spindle cells

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Mesoblastic Nephroma: Imaging

Usually homogeneous cystic changes rare Well circumscribed Looks like Wilms but age is the clue

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Mesoblastic Nephroma

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Mesoblastic Nephroma:Therapy and Prognosis

Most behave in benign fashion Cured by nephrectomy Metastases & recurrence very

rare –Associated with atypical

cellular histology Overall prognosis excellent

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TUMORS OF MEDULLARY ORIGIN 6% of renal neoplasms

Rhabdoid tumor Clear cell sarcoma

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Malignant rhabdoid tumor

– infants – associated with CNS tumors

(PNET) Large masses Infiltrating margins

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Rhabdoid Tumor:

Heterogeneous mass Peripheral low density crescent Ill-defined margins

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Rhabdoid tumor – Poor prognosis – 80% have metastases at

diagnosis »lung, liver, brain, nodes –20% survival @ 18 months

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Clear cell sarcoma

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

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Clear Cell Sarcoma

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

Clear cell sarcoma –Survival rates 60%-70%

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Cystic Masses

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

> 5 years of age –Simple renal cysts (rare)

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MULTILOCULAR CYSTIC RENAL TUMORThe term “multilocular cystic renal tumor”encompasses two histologically distinct

cystictumors: 1. cystic nephromas composed of epithelial

lined cysts separated by fibrous septa with mature tubules

2. cystic partially differentiated nephroblastomas composed of cysts with septa containing foci of blastemal cells.

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Multilocular Cystic Renal Tumor:

Clinical Findings Biphasic age distribution – Boys 3 months to 2 years – Women > 40 years Usually asymptomatic mass Pain & hematuria from ureteral

prolapse of cyst

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Multilocular Cystic Renal Tumor

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

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Multilocular Cystic Renal Tumors

Cystic, fluid-filled mass Varying thickness septations Avascular, except septa

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Multilocular Cystic Renal Tumor

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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

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MCDK: Gross Pathology & Imaging

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

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ANGIOMYOLIPOMA Fat-containing Masses Any age, but more often after 5 years Associated with tuberous sclerosis 80% of tuberous sclerosis patients

have angiomyolipomas also are associated (although to a

lesser extent) with von Hippel-Lindau syndrome and neurofibromatosis.

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Angiomyolipoma: USG

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Angiomyolipoma: CT

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Because of the increased risk of hemorrhage with large angiomyolipomas, the current recommendation is to treat an angiomyolipoma

greater than 4 cm with surgical resection or selective catheter embolization. In

Thus, ultrasound surveillance is currently recommended every 2–3 years before puberty and yearly thereafter to detect any increase in the size of the mass.

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OSSIFYING RENAL TUMOR OF INFANCY Ossifying 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 cells

usually presents as an abdominal mass that may be accompanied by hematuria

On 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

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OSSIFYING RENAL TUMOR OF INFANCY

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TUMORS OF OLDER CHILDREN-- RENAL CELL CANCER Peak incidence: 15-19 yr

Clinical findings pain, hematuria, mass

Imaging findings: solid renal mass same as adult

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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 syndrome

in children who have undergone prior chemotherapy for other renal tumors

Painless hematuria, flank pain, or a palpable mass are the usual presenting symptoms of RCC.

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Most common metastatic diseases Neuroblastoma Leukemia Lymphoma

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NEUROBLASTOMA

arise 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.

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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 hydronephrosis

These hematogenous metastases appear as well-circumscribed hyperechoic renal lesions on ultrasound and hypoenhancing masses on contrastenhanced CT

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Wilms' tumor vs Neuroblastoma Wilms'

<10% are calcified; more often a curvilinear pattern

Occasional local para-aortic adenopathy (less common than with neuroblastoma)

IVC invasion has high positive predictive value

Mets to lungs common

Neuroblastoma Often calcified; scattered

pattern throughout mass Large regional adenopathy High predictive value:

encasement of great vessels, spinal canal invasion, paravertebral mass

Moderate predictive value: extension across midline, displacement of great vessels

Mets to liver, bone common Elevated urine

catecholamines

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LEUKEMIA

Leukemic involvement of the kidneys occurs most often from acute lymphoblastic leukemia- the most common childhood malignancy.

Both ultrasound and CT in kidneys with leukemic infiltration show diffuse bilateral renal enlargement with loss of the normal corticomedullarydifferentiation The affected renal cortex is relatively hypoenhancing on contrast enhanced CT

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LYMPHOMA

Lymphomatous involvement of the kidneys usually occurs by direct extension of retroperitoneal lymphoma or by hematogenous spread, most often from non-Hodgkin lymphoma

Lymphoma of the kidneys can be difficult to detect on ultrasound.

On CT, renal lymphomatous lesions are hypoenhancing compared with the adjacent normal renal parenchyma

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Summary:Neonate Mesoblastic nephroma Multicystic dysplastic kidney

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

> 5 years Renal cell cancer

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Infants & Young Children:Which Solid Renal Tumor?

Homogeneous solid mass? Wilms, mesoblastic nephroma Subcapsular or peripheral solid tumor? Nephroblastomatosis Large cystic areas or bone mets? Clear cell Peripheral fluid/cystic collection? Rhabdoid Infiltrative margins? Rhabdoid/clear cell sarcoma Well circumscribed margins? Wilms, mesoblastic nephroma

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THANK YOU