泌外课件-老也疯狂~.pptVIP

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 Identical age group to Wilms Very aggressive Not distinguishable from Wilms by imaging Bone mets common 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 ponder Duplication Hematoma/renal trauma Pyelonephritis Focal bacterial Xanthogranulomatous Autosomal recessive polycystic kidney dz Infantile form 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 Most common solid abdominal mass in childhood Most common renal malignancy in child 8% of all childhood cancer 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 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 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 Tumor Nephroblastomatosis ImagingAppearance Nodules Subcapsular hypodense plaques US – iso, hypo, hyperechoic (rela