1/13. Pancreatic lesions in von Hippel-Lindau syndrome: the coexistence of metastatic tumors from renal cell carcinoma and multiple cysts.Multiple cysts and benign cystadenomas of the pancreas have been documented occasionally in von Hippel-Lindau syndrome (HLS); however, the malignant involvement of the pancreas in HLS is very rare. We report a case of HLS in which metastatic tumors from renal cell carcinoma (RCC) coexisted with multiple cysts in the pancreas. A 22-year-old woman with a history of HLS had undergone a partial resection of the left kidney for RCC 3 years earlier, at which time a solid mass in the pancreatic tail and multiple pancreatic cysts were also incidentally detected by computed tomography. Over the following 3 years, the mass enlarged slightly, thus raising suspicions that it might be a primary neoplasm of the pancreas. She was referred to the Department of Surgery and Surgical Basic science to undergo surgery. In addition to the tumor in the pancreatic tail, however, further tumors in the pancreatic head were also disclosed by preoperative celiac arteriography and intraoperative palpation and ultrasonography. A distal pancreatectomy was performed, because the enucleation of all the tumors in the pancreatic head was technically impossible and because the patient declined a total pancreatectomy. A histologic examination of the mass in the pancreatic tail revealed metastatic RCC. This case emphasizes that metastatic disease should be included in the differential diagnosis when evaluating the pancreas in a patient with HLS.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/13. von hippel-lindau disease masquerading as autosomal dominant polycystic kidney disease.The diagnostic confusion in differentiating the various causes of renal cystic diseases in adults is well documented. This confusion can include misclassifications between autosomal dominant polycystic kidney disease (ADPKD) and von hippel-lindau disease (VHL). We describe such a case of VHL. A review of the literature and of the patients in our database regarding typical features of each disease, mean age of onset, and frequency of these features was undertaken to provide helpful differentiating features. Pancreatic cysts are one differentiating feature. In VHL, pancreatic cysts can occur in 70% of patients, often are multiple, and rarely may cause exocrine or endocrine insufficiency. Pancreatic islet cell tumors occur. In ADPKD, pancreatic cysts are found in only 9% of patients, usually are single and asymptomatic, generally occur in conjunction with cystic liver disease, and are not found in children or unaffected family members. Pancreatic malignancies do not occur with increased frequency in ADPKD. A different pattern, especially in patients without a strong family history of ADPKD, may be a clue to VHL masquerading as ADPKD. Genetic mutation screening of the VHL gene should be used in these patients.- - - - - - - - - - ranking = 0.2keywords = von (Clic here for more details about this article) |
3/13. common bile duct obstruction due to pancreatic involvement in the von Hippel-Lindau syndrome.Pancreatic involvement in the von Hippel-Lindau syndrome occurs frequently, but is nearly always asymptomatic. Until now, only two cases of bile duct obstruction as a complication of this syndrome have been reported. We present another report of cholestatic jaundice in a patient with the von Hippel-Lindau syndrome and multiple pancreatic cystic lesions, treated by endoscopic implantation of a biliary stent.- - - - - - - - - - ranking = 1.2keywords = von (Clic here for more details about this article) |
4/13. Pancreatic lesions in von Hippel-Lindau syndrome: report of a case.von Hippel-Lindau (VHL) syndrome is a rare autosomal inherited disorder. A germline mutation predisposes carriers to the development of multiple tumors, the most common of which are hemangioblastoma, renal cell carcinoma, pheochromocytoma, and islet cell and cystic tumors of the pancreas, the kidney, and epididymis. Visceral manifestations, such as cystadenoma of the pancreas, occur late in its course. We report the case of a 20-year old woman who presented with vomiting and dizziness, most probably caused by a palpable mass in the upper abdomen, diagnosed as pancreatic cysts. She had undergone surgery for cerebellar hemangioblastoma 4 years previously. In view of her young age, preservation of pancreatic function was crucial because of the expense of enzyme therapy and the difficulties with follow-up. Therefore, we performed cyst enucleation and distal pancreatectomy, which relieved her symptoms. She has been well with no sign of recurrence for 18 months. This case highlights that a well-prepared surgical procedure can cure a patient with pancreatic cysts caused by VHL, living in difficult socioeconomic conditions.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
5/13. Huge pancreas helps diagnosis in von hippel-lindau disease.We describe five patients diagnosed with von hippel-lindau disease who complained of abdominal distension, pain and discomfort for a long time. All patients underwent ultrasonography, CT scan and MRI, which showed huge pancreas filled with multiple cysts. Additionally, extrapancreatic findings such as cerebellar hemangioblastoma (3 patients), retinal hemangioblastoma (2), renal cell carcinoma (3), renal adenoma (1), renal cysts (4), and splenic cyst (1) helped to reach the right diagnosis. One patient who had no known associated pathology had a family history of von hippel-lindau disease. Pancreatic cysts detected on imaging may be a clue to the diagnosis of von hippel-lindau disease. In all patients with multiple pancreatic cysts, this disease should be included in the differential diagnosis.- - - - - - - - - - ranking = 1.4keywords = von (Clic here for more details about this article) |
6/13. Pancreatic involvement in von hippel-lindau disease: report of two cases and review of the literature.BACKGROUND: Von Hippel-Lindau (VHL) disease is an autosomal dominant multicancer syndrome caused by the germline mutation of a tumor suppressor gene. Affected individuals develop benign and malignant tumors of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive system. Although VHL disease is mainly diagnosed after the detection of central nervous system tumors, they may not always be the first presentation. CASE REPORT: We report the case of a patient presenting with pancreatic cysts for whom the final genetic diagnosis of VHL disease was formulated. During management, the use of endoscopic ultrasonography (EUS) proved to be valid in the characterization of the pancreatic lesions. family screening also revealed the genetic mutation in the patient's son and imaging investigations showed the presence of multiple tumors. The diagnosis allowed us to plan appropriate follow-up for both, thus improving their life expectancy. CONCLUSIONS: Gastroenterologists should be aware of the frequent pancreatic involvement in VHL disease and EUS can be useful in this setting.- - - - - - - - - - ranking = 0.8keywords = von (Clic here for more details about this article) |
7/13. Pancreatic lesions in the von Hippel-Lindau syndrome.Common manifestations of the von Hippel-Lindau syndrome, an autosomally dominant inherited cancer-prone disorder, include retinal angiomatosis, hemangioblastoma of the central nervous system, renal cysts, renal cancer, pheochromocytoma, and epididymal cystadenoma. Multiple cysts and microcystic (serous) cystadenomas of the pancreas have also been reported occasionally in patients afflicted with this syndrome. In the large Freiburg study of the von Hippel-Lindau syndrome composed of 66 affected individuals, pancreatic lesions were systematically studied. Fifty-five living individuals were examined by abdominal ultrasound imaging. Abnormal findings were confirmed by computed tomographic scan and/or magnetic resonance imaging. For an additional 11 decreased patients autopsy data were available. Cystic lesions of the pancreas were found in 10 patients (15%). One of these patients presented with multiple pancreatic cysts as the only manifestation of the syndrome. In one patient, a malignant islet-cell tumor was found at autopsy. Because multiple pancreatic cysts did not cause major clinical symptoms and because follow-up examinations over an average period of 5 years did not show significant progression of the lesions, it is concluded that these patients usually do not require surgical treatment. Abdominal ultrasound screening is recommended for patients at risk as a tool to identify potential von Hippel-Lindau syndrome gene carriers with pancreatic manifestations. In all patients with multiple pancreatic cysts, the von Hippel-Lindau syndrome should be included in the differential diagnosis.- - - - - - - - - - ranking = 1.6keywords = von (Clic here for more details about this article) |
8/13. pancreatic pseudocyst in a patient with variant von Willebrand's disease.We report a case of pancreatic pseudocyst which occurred in a 32-year-old woman with variant von Willebrand's disease. A curvilinearly calcified shadow incidentally seen in the upper abdomen on radiographic examination was the first sign of the cyst. The abnormality of hemostatic mechanism found in this patient is speculated to be related to the cyst formation.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
9/13. Retinal angiomatosis and pancreatic cysts in von hippel-lindau disease: use of computed tomography scanning for diagnosis and surveillance.Reports of the retinal and pancreatic manifestations of von hippel-lindau disease have been previously published. We present a case of von hippel-lindau disease in which computed tomography scanning was used in a new manner for diagnosis of retinal disease and for diagnosis and surveillance of pancreatic disease. We emphasize the importance of these new findings and recommend the use of yearly CT scanning of the abdomen. This replaces the previous recommendation of exploratory laparotomy in patients with pancreatic lesions. We further advocate that CT surveillance of the offspring of von hippel-lindau disease patients begin in the second decade, with the anticipation of curing potentially serious, blinding, or life-threatening lesions.- - - - - - - - - - ranking = 1.4keywords = von (Clic here for more details about this article) |
10/13. von Hippel Lindau syndrome presenting as obstructive jaundice with involvement of pancreas in two siblings.Von Hippel Lindau disease is rare autosomal dominant disorder. In our patient's family, 4 out of 7 siblings had manifestations of the disease; 3 of those affected had pancreatic lesions with obstructive jaundice in 2 siblings caused by pancreatic cysts is reported for the first time.- - - - - - - - - - ranking = 0.8keywords = von (Clic here for more details about this article) |
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