Nodular renal blastoma in kidney with multicystic dysplasia. Report of a case.
The clinico-pathologic association of nodular renal blastema, multicystic kidney and obstructive uropathy has been recently identified. We report on a female patient diagnosed as having unilater multicystic dysplasia by prenatal ultrasonography. The patient was nephrectomized at the age of 6 1/2 months. Examination of the resected kidney revealed multiple unilocular cysts in the cortex and hypoplasia of the homolateral ureter; histological study confirmed the presence of multiple cysts limited to the renal cortex, and revealed, among them, multiple cortical metanephric blastema cells islands. Our case supports a relationship between nodular renal blastema, cortical cysts and obstructive uropathy; ureter hypoplasia could cause intraluminal back pressure, with consequent abnormal development of the ampullae, normally endowed in nephronic anlagens induction, cystic tubular ectasia and persistence of nodular renal blastema. The peripheral location of renal nodular blastema and cysts supports a late error in nephrogenesis, at the time of formation of the last generation of nephrons.
Corsi
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III Cattedra di Anatomia e Istologia Patologica, Università La Sapienza, Rome.
Boldrini
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Caione
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Bosman
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D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging*; D006801:Humans; D006984:Hypertrophy; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities* / Q000196:embryology / Q000473:pathology; D007680:Kidney Neoplasms / Q000196:embryology; D009392:Nephrectomy; D007690:Polycystic Kidney Diseases / Q000000981:diagnostic imaging* / Q000196:embryology / Q000473:pathology / Q000601:surgery; D013234:Stem Cells / Q000473:pathology; D014463:Ultrasonography; D014513:Ureter / Q000002:abnormalities; D009396:Wilms Tumor / Q000196:embryology