1/64. Fibropolycystic disease of the hepatobiliary system and kidneys.This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.- - - - - - - - - - ranking = 1keywords = ductal (Clic here for more details about this article) |
2/64. von hippel-lindau disease with multiple malignant renal tumors: the importance of genetic testing.We present an adult man who, while being evaluated for gross hematuria, was found to have polycystic kidneys and multiple bilateral renal cell carcinomas. Further evaluation and the presence of a significant family history of cancer suggested the diagnosis of von hippel-lindau disease. Through the aid of genetic testing, this unusual diagnosis was confirmed and led to the identification of other family members with the von Hippel-Lindau gene. patients with von hippel-lindau disease have an increased incidence of malignant carcinomas and the syndrome can mimic the presentation of other cystic diseases of the kidney. early diagnosis and genetic screening of family members is essential to improve the prognosis and survival of those affected.- - - - - - - - - - ranking = 6.9913736942514keywords = carcinoma (Clic here for more details about this article) |
3/64. Renal cell carcinoma in cases of adult polycystic kidney disease: changing diagnostic and therapeutic implications.Renal cell carcinoma in adult polycystic kidney disease is of rare occurrence and poses a diagnostic and therapeutic challenge. We have treated three such patients in our department. One was male and two were females ranging in age from 20 to 60 years. All were diagnosed preoperatively with ultrasonography or contrast-enhanced CT. Radical nephrectomy was performed in all patients. During the follow-up no patient had recurrence in the contralateral kidney. One patient had local recurrence in the renal fossa and was treated with local radiotherapy and immunotherapy. In the past due to difficulty in diagnosis, invasive investigations like angiography were recommended and prophylactic contralateral nephrectomy was often undertaken. With the availability of reliable noninvasive investigations these patients can be diagnosed accurately and the contralateral kidney saved in most cases.- - - - - - - - - - ranking = 17.478434235629keywords = carcinoma (Clic here for more details about this article) |
4/64. Clinics in diagnostic imaging (46). Renal cell carcinoma in acquired cystic kidney disease.Acquired cystic disease of the kidney and renal cell carcinoma are associated with chronic renal failure. In recent years, there has been increased incidental detection of renal tumours through the liberal use of ultrasonography (US) and computed tomography (CT). A 40-year-old man suffering from chronic renal failure and who was being treated with haemodialysis for six years, was found to have a complex cystic lesion on US and CT. nephrectomy was performed and the lesion was confirmed to be renal cell carcinoma associated with acquired cystic kidney disease. The current role of imaging in the management of affected patients are discussed.- - - - - - - - - - ranking = 20.974121082754keywords = carcinoma (Clic here for more details about this article) |
5/64. iodine-131 ablation therapy for a patient receiving peritoneal dialysis.The authors describe a patient with follicular thyroid carcinoma who was receiving continuous ambulatory peritoneal dialysis to manage end-stage renal disease. To deliver radioiodine therapy to ablate thyroid remnants safely and under optimal conditions, the behavior of 37 MBq (1 mCi) I-131 was followed daily for 3 days. blood activity and total body count decreased with a half-life of 100 hours (4.17 days). The daily iodide removal rate, estimated as a percentage of the total administrated activity, was low: 5.3% to 8.6% in peritoneal dialysate and 1.3% to 2.2% in urine. The thyroid uptake, measured using a probe, was 2.4% to 2.1% from day 1 to day 3 and 1.9% later at day 8. The volume of thyroid remnants was determined by ultrasonography to be 0.6 g. The patient received a reduced ablative I-131 dose of 814 MBq (22 mCi). radiation emitted from the patient after I-131 therapy, monitored using a radiation monitor probe located at a distance of 1 meter, decreased with an effective half-life of 70 hours (2.9 days). The integration of the curve from t = 0 showed a level always less than 25 microSv/hour as early as 24 hours after treatment. Because the iodine removal rate is continuous but low in a case of peritoneal dialysis, smaller therapeutic doses must be administered to deliver maximal radiation to residual thyroid tissue while minimizing excessive radiation exposure to patients, their families, and medical staff.- - - - - - - - - - ranking = 3.4956868471257keywords = carcinoma (Clic here for more details about this article) |
6/64. Pancreatic ductal adenocarcinoma associated with Potter type III cystic disease.Although polycystic liver disease (PLD) is known to be associated with autosomal dominant polycystic kidney disease, a finding of PLD with pancreatic ductal adenocarcinoma is extremely rare. We have experienced one such case of a ductal adenocarcinoma of the pancreas in a patient with Potter type III cystic disease of the liver and kidney. A 63-year-old man was admitted to our hospital because of obstructive jaundice. Six months previously, on admission to a local hospital for treatment of diabetes mellitus, he had been found to have polycystic disease of the liver and kidney. Ultrasound examination revealed dilatation of the intrahepatic bile duct and the common bile duct. blood tests showed an elevated total bilirubin level. Abdominal computed tomography scans and magnetic resonance imaging demonstrated polycystic lesions in the liver and the bilateral kidneys. Percutaneous transhepatic cholangio-drainage was performed, and fluorography of the biliary tree revealed obstruction of the lower common bile duct, causing jaundice. This appears to be a case of independent association of pancreatic ductal adenocarcinoma with polycystic disease of the liver and kidney. The patient's sister, who also had polycystic disease of the liver and kidney, had died of squamous cell carcinoma of the tongue. Although familial associations of carcinomas with polycystic liver disease may be extremely rare, they provide a perspective for the etiology of polycystic liver disease.- - - - - - - - - - ranking = 38.461181624131keywords = carcinoma, ductal (Clic here for more details about this article) |
7/64. tuberous sclerosis with cystic renal disease and multifocal renal cell carcinoma in a baby girl.An infant, in whom the prenatal diagnosis of tuberous sclerosis complex was made, presented with extreme bilateral nephromegaly owing to diffuse cystic changes. histology of the resected non-functioning left kidney revealed, in addition to the characteristic cysts, two foci of renal cell carcinoma not visible on US or MRI. This infant is exceptional given the extensive cystic transformation of both kidneys and the presence of malignant lesions at this young age.- - - - - - - - - - ranking = 17.478434235629keywords = carcinoma (Clic here for more details about this article) |
8/64. Multilocular renal cysts with renal cell carcinoma: report of four cases.According to Bosniak's classification, renal cysts with moderate calcification, irregular margins, and thickened enhanced septa should raise a suspicion of malignancy. Diagnosis of multilocular renal cysts depends on their histological features. Since 1951, it has generally been considered that a multilocular renal cyst can change from a benign lesion to one which may be combined with malignant change. According to a report from japan, multilocular renal cysts with renal cell carcinoma are uncommon, and the reported incidence was about 9%. A radical nephrectomy was performed for malignant change. Herein, we present 4 cases of multilocular cyst (Bosniak's class IV) with renal cell carcinoma and review the related literature for their pathogenesis and management.- - - - - - - - - - ranking = 20.974121082754keywords = carcinoma (Clic here for more details about this article) |
9/64. Collecting duct carcinoma exhibiting diastase-resistant PAS-positive globular cytoplasmic inclusions and rhabdoid features arising in adult polycystic kidney disease: a case report.A 76-year-old man complained of lumbago, and a subsequent detailed examination revealed a mass in the left kidney. Macroscopically, multiple cysts were recognized in the bilateral kidney; the tumor proliferated predominantly in the renal medulla and showed a whitish color with focal necrosis and hemorrhage. A histologic examination of the surgically resected tumor showed various growth patterns, such as solid, tubular, and papillary, by tumor cells. The diagnosis of collecting duct carcinoma (CDC) was made. Furthermore, tumor cells contained globular inclusions resembling hyaline globules and large eosinophilic inclusions, resulting in rhabdoid features, in the cytoplasm. Histochemically, globular inclusions were positive for periodic acid-Schiff (PAS) with diastase pretreatment. Immunohistochemically, large esosinophilic inclusions were reactive for vimentin. Although these findings are rare in CDCs, they should be recognized in the pathologic spectrum of CDCs. Int J Surg Pathol 12(2):171-177, 2004- - - - - - - - - - ranking = 17.478434235629keywords = carcinoma (Clic here for more details about this article) |
10/64. Renal sarcoma associated with adult polycystic kidney disease. A case report and literature review.The development of renal cell carcinoma in adult Polycystic kidney Disease (APKD) has been reported in the literature; one of the features of renal malignancy in APKD is the difficulty to make a diagnosis, and the majority of cases reported are incidental findings at surgery or autopsy. We report a rare case of renal sarcoma in a patient with APKD. sarcoma associated with APKD does not seem to have particular biological characteristics when compared with primary renal sarcoma; however the polycystic kidney represents an aggravating circumstance, because of the difficulty in making an early diagnosis of a disease with a poor prognosis.- - - - - - - - - - ranking = 3.4956868471257keywords = carcinoma (Clic here for more details about this article) |
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