Abstract
Bilateral nephromegaly with a histological picture of diffuse Wilms' tumor or so-called bilateral nephroblastomatosis is a rare condition. We present an additional case documented by urography. To our knowledge, this is the first report of diffuse nephroblastomatosis recognized at 2 hours of age. A few cases are reported but urographic documentation is available in only 4 of those and in our own. The review of the urographic findings reveals features which seem to be nearly pathognomonic of this condition. Urographic appearance similar to that of adult type polycystic disease without the typical radiolucent cysts, should raise the suspicion of a diffuse bilateral process in the kidneys. Exclusion of other infiltrative diseases, like leukemia and glycogen storage disease, should lead to radiologic diagnosis of nephroblastomatosis. Renal biopsy is then indicated.
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Anderson, E. E., Harper, J. M., Small, M. P., Atwill, W. H.: Bilateral diffuse Wilms' tumor: A 5 year survival. J. Urol.99, 707 (1969)
Bolande, R. P.: Congenital mesoblastic nephroma of infancy. Perspectives in pediatric pathology p. 227. Year Book Medical Publishers, Inc. 1973
Gowdey, J. F., Neuhauser, E. B. D.: The roentgen diagnosis of diffuse leukemic infiltration of the kidneys in children. Amer. J. Roentgenol.60, 13 (1948)
Gwinn, J. L., Landing, B. H.: Cystic diseases of the kidneys in infants and children. Radiol. Clin. N. Amer.6, 191 (1968)
Gyepes, M. T., Burko, H.: Diffuse bilateral Wilms' tumor simulating multicystic renal disease. Radiology82, 1029 (1964)
Harper, J. M., Anderson, E. E.: Bilateral diffuse Wilms tumor: A case report. J. Urol.91, 220 (1964)
Hou, L. T., Holman, R. L.: Bilateral nephroblastomatosis in a premature infant. J. Path. Bact.82, 249 (1961)
Javapadour, N., Bush, I. M.: Induction and treatment of Wilms' tumor by transplantation of renal blastema in a new experimental model J. Urol.107, 931 (1972)
Lee, F. A.: Radiology of the Beckwith-Wiedemann syndrome. Radiol. Clin. N. Amer.10, 261 (1972)
Lee, F. A., Gwinn, J. L.: Four uncommon pediatric conditions associated with bilateral nephromegaly. Ann. Radiol.12, 285 (1969)
Liban, E., Kozenitzky, I. L.: Metanephric hamartomas and nephroblastomatosis in siblings. Cancer25, 885 (1970)
Neuhauser, E. B. D.: Case records of the Massachusetts General Hospital, Case No. 46372. New Eng. J. Med.263, 557 (1960)
Nogrady, M. B., Dunbar, J. S.: Delayed concentration and prolonged excretion of urographic contrast medium in the first month of life. Amer. J. Roentgenol.104, 289 (1968)
Perlman, M., Goldberg, G. M., Bar-Ziv, J., Danovitch, G.: Renal hamartomas and nephroblastomatosis with fetal gigantism: A familial syndrome. J. Pediat.83, 414 (1973)
Perlman, M., Levine, M., Wittels, B.: Fetal gigantism, renal hamartomas and nephroblastomatosis syndrome: An additional subject with typical Wilms' tumor. Cancer, 1974 (Submitted for publication)
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Bar-Ziv, J., Hirsch, M. & Perlman, M. Bilateral nephroblastomatosis. Pediatr Radiol 3, 85–88 (1975). https://doi.org/10.1007/BF01000119
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DOI: https://doi.org/10.1007/BF01000119