Abstract
Three similar cases of bilateral neoplastic disease of the kidney were associated with congenital pulmonary cystic disease and fetal macrosomia. These cases are compared to several in the literature. We suggest that these three cases represent a spectrum of abnormal morphogenesis that affects both the kidney and the lung. Case 1 had bilateral multilocular cysts of the kidney in association with hamartomatous pulmonary cysts, This case is compared with Case 2 who had bilateral multilocular renal cysts, with one area of mesoblastic nephroma and multiple pulmonary cysts. These cases are compared to Case 3 who demonstrated markedly hyperplastic renomegaly with medullary dysplasia (similar to what is seen in the Beckwith-Wiedemann syndrome) in association with classical bilateral cystic adenomatoid malformation of the lungs. All three cases were overgrown at birth, and we suggest that these cases illustrate similarities in the development of kidneys and lungs. Embryologically the kidney and lung begin their development around the same time. During the 5th week of gestation, the ureteric bud invades the unsegmented mesoderm that becomes the metanephric system, and the lung bud invades the splanchnic mesoderm which provides the stimulus for its growth. The predominant pattern of a congenital kidney or lung neoplasm may reflect the timing of a prenatal neoplastic event.
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Graham, J., Boyle, W., Troxell, J. et al. BILATERAL NEOPLASTIC KIDNEY DISEASE, PULMONARY CYSTIC DISEASE, AND FETAL MACROSOMIA: A SPECTRUM OF DEVELOPMENTAL ABNORMALITIES. Pediatr Res 18 (Suppl 4), 305 (1984). https://doi.org/10.1203/00006450-198404001-01272
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DOI: https://doi.org/10.1203/00006450-198404001-01272