Cases reported "Kidney Diseases, Cystic"

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1/12. Solitary renal cyst, hypertension and renin.

    Solitary renal cysts may cause renin hypersecretion with associated hypertension by compressing surrounding tissue and by distortion of renal vessels. Selective measurements of plasma renin activity in the renal veins can predict the antihypertensive effect of decompression. An illustrative case is presented and its significance is discussed.
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2/12. Ivemark syndrome: asplenia with kidney collecting duct cysts and polysplenia with cerebellar cyst.

    Two newborns, one male and one female, from two different families, with Ivemark syndrome proven at autopsy are reported. One of them had asplenia and another had polysplenia. Both newborns had complex cardiac defects with isomerism of the lungs. The newborn with asplenia had dextrocardia, transposition of the great vessels, stenosis of the pulmonary artery, common atrioventricular canal and patent ductus arteriosus. The newborn with polysplenia had a common atrium, hypoplastic left ventricle and patent ductus arteriosus. The patient with asplenia had cystic dilated collecting ducts of the kidney and the patient with polysplenia had cerebellar cyst. These associate malformations have not been reported previously. Both cases were sporadic.
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3/12. Spontaneous remission of peripelvic renal cysts.

    Three cases are presented of spontaneous regression of peripelvic renal cysts. The lesions were manifested by lateral extrinsic pressure defects in the renal pelvis and major calices (all 3 cases), and selective renal angiography showing stretching of vessels plus sharply defined defects in the nephrographic phase (2 patients). In 1 case the mass disappeared following trauma to the flank, while in the remaining 2 cases it disappeared spontaneously.
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4/12. Spontaneously ruptured multilocular cystic nephroma.

    Multilocular cystic nephroma is a relatively rare benign tumor of the kidney. An adult case of spontaneously ruptured multilocular cystic nephroma was reported. A 33-year-old woman who presented with a right flank colic attack. The preoperative diagnosis was spontaneously ruptured renal tumor. A right nephrectomy was performed. A multilocular cystic lesion, 10 cm in diameter, was found in the removed specimen, which had a hematoma inside and direct tumor extension into the renal pelvis. On microscopic examination, the surface of loculi was covered by a layer of hobnail epithelium, and septa were composed of fibrous tissue with dilated vessels, lacking in normal renal components. There were no signs of malignancy. The lesion extending into the renal pelvis showed the same microscopic findings. The final diagnosis was multilocular cystic nephroma. Two respects, spontaneous rupture and tumor extension into the renal pelvis, were unique to the present case. There has been no report of spontaneously ruptured mutilocular cystic nephroma. The tumor extension into the renal pelvis, however, has been found in several reports. This growth pattern might be one of the characteristics of this benign renal tumor.
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5/12. Giant hydronephrosis mimicking progressive malignancy.

    BACKGROUND: Cases of giant hydronephroses are rare and usually contain no more than 1-2 litres of fluid in the collecting system. We report a remarkable case of giant hydronephrosis mimicking a progressive malignant abdominal tumour. CASE PRESENTATION: A 78-year-old cachectic woman presented with an enormous abdominal tumour, which, according to the patient, had slowly increased in diameter. Medical history was unremarkable except for a hysterectomy >30 years before. A CT scan revealed a giant cystic tumour filling almost the entire abdominal cavity. It was analysed by two independent radiologists who suspected a tumour originating from the right kidney and additionally a cystic ovarian neoplasm. Subsequently, a diagnostic and therapeutic laparotomy was performed: the tumour presented as a cystic, 35 x 30 x 25 cm expansive structure adhesive to adjacent organs without definite signs of invasive growth. The right renal hilar vessels could finally be identified at its basis. After extirpation another tumourous structure emerged in the pelvis originating from the genital organs and was also resected. The histopathological examination revealed a >15 kg hydronephrotic right kidney, lacking hardly any residual renal cortex parenchyma. The second specimen was identified as an ovary with regressive changes and a large partially calcified cyst. There was no evidence of malignant growth. CONCLUSION: Although both clinical symptoms and the enormous size of the tumour indicated malignant growth, it turned out to be a giant hydronephrosis. Presumably, a chronic obstruction of the distal ureter had caused this extraordinary hydronephrosis. As demonstrated in our case, an accurate diagnosis of giant hydronephrosis remains challenging due to the atrophy of the renal parenchyma associated with chronic obstruction. Therefore, any abdominal cystic mass even in the absence of other evident pathologies should include the differential diagnosis of a possible hydronephrosis. Diagnostic accuracy might be increased by a combination of endourological techniques such as retrograde pyelography and modern imaging modalities.
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6/12. Giant simple renal cyst complicated with hypertension.

    Solitary renal cysts are a common and usually asymptomatic occurrence in older patients. They may be associated with hypertension or abdominal disturbances, as they can be responsible for compression of surrounding tissues and distortion of renal vessels. This report presents an interesting case of a hypertensive patient with a solitary renal cyst of a marked size. Owing to the high risk of performing a surgical procedure in such a patient, a distinct therapeutic solution was opted for. Successful management of this case was achieved by a combination of percutaneous fluid aspiration and injection of alcohol and Vibramycin inside the cystic cavity. Percutaneous fluid evacuation combined with the administration of a sclerosing agent is suggested as a safe and effective alternative for cyst decompression and blood pressure normalisation.
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7/12. angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma.

    Renal angiomyolipoma (AML) is typically a solid lesion, composed of varying amounts of adipose, vascular, and muscular tissue, lacking an epithelial component. Although it is known that entrapped renal tubules may be observed in AML, presentation as a cystic mass has not been previously reported. We report the clinicopathologic and immunohistochemical features of four cystic renal AML. The lesions were found in 2 male and 2 female patients, ranging in age from 37 to 76 years, none with a history of hormonal therapy. One of the four patients had known tuberous sclerosis, and this patient and 1 other presented with bilateral cystic renal lesions. Grossly, the lesions measured between 1.3 and 4.5 cm in greatest dimension. Histologically, the lesions were each composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) a compact subepithelial "cambium-like" layer of cellular, mullerian-like AML stroma with prominent admixed chronic inflammation; and 3) muscle-predominant AML with associated dysmorphic blood vessels exterior to the cellular subepithelial stroma. Immunohistochemically, the stromal components of all four lesions labeled with HMB-45 and Melan-A, most intensely in the cellular subepithelial stroma, whereas smooth muscle actin and desmin demonstrated the opposite pattern, with greatest intensity in the muscle-predominant AML areas. Immunoreactivity for estrogen and progesterone receptors (ER, PR) and CD10 was present in all cases, with strongest and most diffuse staining in the subepithelial AML cells. The cyst lining cells were positive for pancytokeratin and soy bean agglutinin (the latter characteristic of distal nephron epithelium) but did not label for ER, PR, or melanocytic markers. Although these distinctive lesions may be confused with mixed epithelial-stromal tumor, their lack of female predominance or association with hormone therapy, their distinctive dysplastic blood vessels and disorganized exterior muscular layer, along with their immunohistochemical profile, support their classification as AML with epithelial cysts. Whereas the cysts appear to arise primarily from entrapped collecting ducts, the peculiar subepithelial condensation of AML stromal cells, with morphologic and immunohistochemical features suggesting differentiation toward endometrial stroma, may represent epithelial-induced mullerian differentiation not previously reported in AML.
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8/12. Pulmonary calcinosis.

    The clinical and pathological features are described of a case of pulmonary calcinosis complicating cystic disease of the renal medulla. A histopathological study of the lung revealed calcification in the alveolar walls and in the blood vessels, predominantly in the pulmonary veins and venules. The calcified deposits were also studied by electron microscopy, and appearances suggestive of active growth of the deposits were recognised. Chemical analysis of the lung revealed a calcium content some 55 times greater than that of a normal lung. There was a five-fold increase in magnesium content. Reference is made to the literature demonstrating that the chemical composition of metastic calcification differs according to whether it is visceral or non-visceral in type.
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9/12. Benign causes of acquired ureteropelvic junction obstruction: a uroradiologic spectrum.

    Five different benign causes of ureteropelvic junction obstruction in adults without prior obstructive history are presented: aortic aneurysm, renal cyst, xanthogranulomatous pyelonephritis, eosinophilic ureteritis, and a crossing blood vessel. Although uncommon, these etiologies warrant consideration when an adult patient presents with ureteropelvic junction obstruction without a prior history of obstruction.
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10/12. Angiographic and ultrasonic findings in infected simple cysts of the kidney.

    This paper describes angiographic and ultrasonic findings in three patients with proven infected renal cyst. The clinical picture was that of inflammatory renal disease. B-mode ultrasonography showed the characteristic features of renal cyst; however, this technique cannot differentiate between infected and simple cysts. Selective renal angiography demonstrated the following, enabling differentiation from simple renal cyst; hypervascular rim, irregular inflammatory vessels, indistinct interface between cyst and adjacent parenchyma, and prominent capsular branches. Since percutaneous puncture of an infected renal cyst or abscess carries risk of infectious complications, angiography appears justified only for those patients with fever and flank pain who have cystic lesions in the kidney confirmed by echography.
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