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Computed tomography of the abdomen in case 1. Computed tomography of the abdomen shows massive ascites and enlarged kidneys with no evidence of cysts.
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Chest radiography revealed cardiac enlargement and bilateral pleural effusions, and an abdominal computed tomography (CT) scan revealed massive ascites and bilateral kidney enlargement (Fig. 1).
Renal biopsy findings in case 1. (a) periodic acid-Schiff (PAS) stain; magnification 10x; (b) PAS stain; 200x. Light microscopy images show tubular dilatation and atrophy. Cystic tubules show irregularities and thinning of the tubular epithelial cells. The glomeruli show global sclerosis and arterial intimal thickening, consistent with the patient's age, without any other remarkable changes such as crescent formation.
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There were no remarkable glomerular changes except for global sclerosis consistent with the patient's age (Fig. 2).
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Serial changes of abdominal computed tomography (CT) at (a) 2 years prior to admission, (b) 1 year prior to admission and (c) at admission in case 2. There is no evidence of kidney enlargement at 2 or 1 year prior to admission; however, CT at admission shows kidney enlargement compared with the previous CT scans.
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Serial CT images are shown in Fig. 3.
Magnetic resonance imaging (MRI) of the abdomen: (a) axial section of the abdomen (T1-weighted image) and (b) coronal section of the abdomen (T2-weighted image) in case 2. No apparent microcysts are seen in the enlarged kidneys, including the coronal images, however, a closer observation shows multiple cysts along the corticomedullary boundary in the left kidney in the coronal section (arrows) (b).
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Magnetic resonance images (MRI) are shown in Fig. 4.
Renal biopsy findings in case 2. (a) periodic acid-methenamine-silver (PAM) stain; original magnification 10x, (b) PAS stain original magnification 200x. Light microscopy images show tubular dilatation and atrophy. Cystic tubules show irregularities and thinning of the tubular epithelial cells. Of seven glomeruli, two are globally sclerotic, one is segmentally sclerotic, and two show glomerular cysts. The other glomeruli are intact.
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The distal tubules were also dilated, consistent with MCKD, and there was mild interstitial fibrosis and inflammatory cell infiltration (Fig. 5).
Immunohistochemical findings in both cases. Immunohistochemical staining for CD10, cytokeratin 7 (CK7), epithelial membrane antigen (EMA), E-cadherin, and cytokeratin (34 beta E12) in the interstitium of both cases. Original magnification 10x. The tubular basement membrane (TBM) of dilated tubules in both cases is negative for CD10. In case 1, the TBM of dilated tubules is positive for CK7, EMA, E-cadherin, and CK (34 beta E12). In case 2, the TMB of dilated tubules is positive for CK7 and EMA and weakly positive for E-cadherin and CK (34 beta E12).
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We also performed immunohistochemical staining in both cases for CD10, cytokeratin 7 (CK7), epithelial membrane antigen (EMA), E-cadherin, and cytokeratin (34 beta E12) to identify the level of tubular dilatation (Fig. 6).