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Differential diagnosis of cystic renal tumors especially multilocular cystic nephroma. 

Differential diagnosis of cystic renal tumors especially multilocular cystic nephroma. 

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The following is a report of the unusual case of a multilocular cystic nephroma in an 8-year-old boy who was transferred to our unit with a palpable abdominal tumor. The patient suffered from thoracic pain and night sweating. The laboratory values were normal. Abdominal sonography showed a huge kidney tumor on the right side consisting of numerous...

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Space occupying lesions of the retroperitoneum originate from the kidneys, the adrenal glands and the sympathetic chain. Cystic lesions often are benign, whereas solid lesions are often malignant. Cystic lesions of the kidney are simple cysts, multicystic dysplastic kidneys and multiloculated cystic nephroma. Multicystic dysplastic kidneys are unilateral diseases characterized by multiple round or oval shaped cysts, which don't communicate with each other. Multilocular cystic nephromas are characterized by multiple irregular cysts of different size, separated by septa of various thickness. Solid tumors of the kidneys compromise Wilms tumor, mesoblastic nephroma and infiltrations of the kidneys in malignant systemic disorders such as leukemia and lymphoma. Wilms tumors and mesoblastic nephromas have an inhomogenous, liver like echogenicity often with small cysts without calcifications. In the border zones of the tumors normal renal tissue can be displayed in most cases. Leukemic infiltrations of the kidneys are characterized by an increase of the size of the kidneys. The internal echogenicity of the kidneys is focally or diffusely increased or decreased. Cystic lesions of the adrenal glands are rare. Solid lesions of the adrenal gland are adrenal hemorrhages, neuroblastomas, ganglioneuroblastomas and phaeochromocytomas. Adrenal hemorrhages are initially echogenic. They decrease in size and get more and more cystic before they disappear. The differentiation of congenital neuroblastoma and adrenal hemorrhage is difficult in the individual case. In contrast to adrenal hemorrhages neuroblastomas are good perfused with color Doppler. Neuroblastomas in elder patients show inhomogeneous delineation and echogenic internal echoes. They often contain calcification with acoustic shadowing and rarely have internal cysts. Phaeochromocytomas are round good definable tumors without calcification. They are localized in the region of the adrenal glands or the renal hilus. They may contain small cysts, whereas calcifications are usually absent.
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Color and spectral Doppler allow flow measurement in renal arteries and veins. Normal values for the flow velocities and resistance indices are necessary for the quantification of kidney perfusion. Flow velocities decrease from the hilus to the periphery of the kidney: They are highest in the renal arteries and veins and lowest in the interlobar arteries and veins. Additionally the flow velocities increase with age and weight of the patient. If pathologic flow parameters are estimated the location of the sample volume and the age and weight of the patient have to be considered. Renal flow parameters may be influenced by hemodynamic, cardiovascular and renal or urinary tract diseases. Cardiovascular malformations which influence renal perfusion are leakages of the aortic ‘Windkessel’ such as an open duct, common arterial trunk and left heart obstructions such as hypoplastic left heart syndrome and coarctation of the aorta. Leakages of the aortic ‘Windkessel’ are characterized by a decrease of the diastolic amplitude whereas left heart obstruction reduce the systolic amplitude. Acute renal failure can be caused pre-renal, renal and post-renal. These conditions may be associated with low diastolic flow, sometimes retrograde diastolic flow, which causes low volume flow and renal failure. Examples for pre-renal failure are cardiovascular anomalies and shock. Examples of renal failure are reno-parenchymal diseases such as glomerulonephritis, pyelonephritis and cystic kidney disease. Post-renal abnormalities are obstructive uropathies.
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The authors report an unusual presentation of congenital mesoblastic nephroma as a multilocular cystic renal lesion. Prenatal sonography revealed a unilateral, encapsulated, multilocular cytic mass with solid components measuring 5.7 × 5.4 × 4.3 cm in the left renal fossa. There was no increase in vascularity and no signs of hydrops fetalis. On the forth postnatal day left-sided radical nephrectomy was performed and histopathological examination revealed cellular type congenital mesoblastic nephroma. A multicystic appearance is rare as the vast majority of prenatally diagnosed congenital mesoblastic nephroma cases presented in the literature are of the classic type with solid homogenous or heterogenous appearence. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2012.