1/32. Hepatic adenomatosis: rapid sequence MR imaging following gadolinium enhancement: a case report.Hepatic adenomas are primary liver tumors usually associated with underlying metabolic disease or with anabolic steroid or oral contraceptive use. Hepatic adenomatosis (HA) is defined as the presence of more than four adenomas. Only 13 cases of HA have been reported in patients without glycogen storage disease or steroid use. We report a case of HA imaged by postcontrast T1-weighted images obtained during a breath-holding series. The lesions were most conspicuous 3-4 min after contrast administration; 4 of the 5 tumors were not identified on T2-weighted images. Unlike previous reports of HA in which the lesions remained hyperintense during sequential postcontrast imaging, the smaller lesions in this case demonstrated contrast washout, thereby distinguishing them from hemangiomata.- - - - - - - - - - ranking = 1keywords = glycogen storage disease, glycogen storage, storage disease, glycogen, storage (Clic here for more details about this article) |
2/32. Long-term outcome of liver transplantation in patients with glycogen storage disease type Ia.liver transplantation may be indicated in patients with GSD type Ia when dietary treatment fails or when hepatic adenomas develop, because they carry a risk of liver cancer or severe intratumoral haemorrhage. Published reports on the results of liver transplantation in patients with GSD Ia include 10 patients and provide little information on long-term outcome. In particular, it is not known whether liver transplantation prevents renal failure due to focal segmental glomerulosclerosis. We report here on 3 patients with GSD Ia in whom liver transplantation was performed at 15, 17 and 23 years of age because of multiple hepatic adenomas in all 3 patients with a fear of malignant transformation, and of poor metabolic balance and severe growth retardation in the youngest one. Renal function was normal in all patients. During the 6-8 years following transplantation, the quality of life has initially greatly improved, with none of the previous dietary restraints and a spectacular increase in height. However, long-term complications included chronic hepatitis c in one patient, gouty attacks in another and focal segmental glomerulosclerosis with progressive renal insufficiency in the third. These results: (1) confirm that liver transplantation restores a normal metabolic balance in patients with GSD Ia, allows catch-up growth and improves the quality of life; (2) suggest that liver transplantation may be considered in teenagers with unresectable multiple adenomas because of a lack of clear-cut criteria to detect malignant transformation early; and (3) suggest that liver transplantation does not prevent focal segmental glomerulosclerosis associated with GSD Ia.- - - - - - - - - - ranking = 10.720304127501keywords = glycogen storage disease type, glycogen storage disease, storage disease type, glycogen storage, storage disease, glycogen, disease type, storage (Clic here for more details about this article) |
3/32. Management issues regarding hepatic adenomatosis.Hepatic adenomatosis is a rare disease defined by multiple hepatic adenomas. There is controversy with regard to the optimal treatment for this disease because the potential for intraperitoneal hemorrhage or malignant transformation of the tumors is difficult to estimate. Furthermore, the technical difficulties of complete resection of all adenomas present unique operative challenges. We report experience with two patients and reviewed all reported cases from 1977. We define hepatic adenomatosis as five or more hepatic adenomas not associated with a medical history of glycogen storage disease, anabolic steroid use, or oral contraceptive use. The incidence of hepatic adenomatosis was preponderate among women [20 of 32 patients (63%)]. Thirteen of 32 patients (41%) had intratumoral bleeding, including four patients with intraperitoneal hemorrhage. Ten of 13 patients (77%) with intratumoral bleeding complained of abdominal pain; overall, 19 of 32 patients (59%) had abdominal pain. Twelve patients underwent hepatic resection, 6 patients underwent liver transplantation with no reported mortality, and 14 patients had no surgical treatment. Hepatocellular carcinoma was histologically confirmed in 2 of 32 patients (6%). Larger symptomatic adenomas exposed to liver surface have a bleeding propensity and should be surgically resected. Routine biopsy of other small nodules suspected as adenomas is recommended for definitive diagnosis.- - - - - - - - - - ranking = 1keywords = glycogen storage disease, glycogen storage, storage disease, glycogen, storage (Clic here for more details about this article) |
4/32. Microcystic adenoma of the pancreas (glycogen-rich cystadenoma) with stromal amyloid deposits.AIMS: We report a case of a pancreatic glycogen-rich microcystic serous adenoma with stromal amyloid deposits, focusing on the significance of isolated amyloid deposits in tumours. methods AND RESULTS: The architectural pattern was characterized by thin-walled cysts lined by a single layer of flat or cuboidal epithelial cells intensely stained by the PAS-reaction only before diastase digestion, suggesting the presence of glycogen. Tumour stroma was composed of broad fibrocollagenous tissue with lamellar hyalinized areas which were positively stained by congo red and showed green birefringence and dichroism with polarized light. For amyloid protein characterization, immunohistochemical studies were performed with anti-beta amyloid protein and anti-amyloid precursor pre-A4695. The former antibody diffusely stained tumour stroma, while the latter stained only scattered stroma cells. CONCLUSIONS: This is the first documented case of amyloid deposition in pancreatic serous adenoma. We indicate that the source of amyloid is an APP-like precursor secreted by stromal myofibroblasts.- - - - - - - - - - ranking = 0.30967310123167keywords = glycogen (Clic here for more details about this article) |
5/32. Hepatic adenomas treated with percutaneous ethanol injection in a patient with glycogen storage disease type Ia.We report a 20-year-old man with glycogen storage disease type Ia (GSD Ia) who presented multiple hepatocellular adenomas (HCAs) in 1993 and in whom percutaneous ethanol injection (PEI) was conducted as treatment for some enlarging tumors beneath the liver surface. In a 6-year follow-up period, we observed gradual enlargement of some of the HCAs, and the rapid growth of a newly developed tumor. In August 1996, one slow-growth HCA was 52 mm in diameter and was located beneath the surface of the liver. We conducted PEI therapy to prevent its spontaneous rupture. During the following year, another tumor developed beneath the liver surface, but showed extremely rapid growth, reaching 51 mm in diameter, from being undetectable, within 12 months. PEI therapy was again conducted for this newly developed tumor. Although additional PEI therapy was required for each tumor, because of suspected recurrence, no findings of discrete recurrence have been detected by computed tomography and magnetic resonance imaging for more than 2 years, up to the time of this study. We consider PEI to be a useful and effective therapeutic modality for individual HCAs in patients with GSD Ia.- - - - - - - - - - ranking = 13.400380159377keywords = glycogen storage disease type, glycogen storage disease, storage disease type, glycogen storage, storage disease, glycogen, disease type, storage (Clic here for more details about this article) |
6/32. Multiple adenomas and hepatocellular carcinoma in a renal transplant patient with glycogen storage disease type 1a (von Gierke disease).We report on a 42-year-old female patient with glycogen storage disease type 1a (von Gierke disease, GSD 1a) who developed hepatic adenomas and finally a hepatocellular carcinoma 10 years after renal transplantation. The tumor was resected; however, the patient died 6 months later as a result of fulminant carcinoma recurrence. In patients who have GSD 1a with terminal renal failure, combined liver and kidney transplantation may be considered at an early stage of the disease.- - - - - - - - - - ranking = 13.400380159377keywords = glycogen storage disease type, glycogen storage disease, storage disease type, glycogen storage, storage disease, glycogen, disease type, storage (Clic here for more details about this article) |
7/32. Thyroid clear cell adenoma with marked dilatation of membranous structures: electron-microscopic study.The electron-microscopic features of a thyroid follicular adenoma with clear cell change is described. The tumor was 7 mm in size and located in the upper portion of the left lobe of the thyroid of a 29-year-old Japanese female. Its cut surface was pale brown in color and well demarcated. The lesion consisted of solid nests with pinpoint lumina. The cytoplasm of the tumor cells was completely clear, but slightly vesicular without oncocytic change. Electron-microscopic examination revealed that the cytoplasm of the tumor cells was filled with numerous empty vacuoles. The vacuoles consisted of dilated membranous structures, indicating that they had been formed from rough endoplasmic reticulum and golgi apparatus. There was no accumulation of glycogen, mucin, or lipid. The dilated membranous structures in the cytoplasm may be related to the increased thyroglobulin synthesis by the tumor.- - - - - - - - - - ranking = 0.051612183538612keywords = glycogen (Clic here for more details about this article) |
8/32. Hepatic adenomatosis in glycogen storage disease type Ia: report of a case with unusual histology.Hepatic adenomatosis is a well-known complication of glycogen storage disease type Ia (von Gierke disease). Although most of these tumors have an appearance similar to sporadic hepatocellular adenomas, unusual histologic features have been reported, including Mallory hyaline, varying degrees of fibrosis, and aggregates of neutrophils. We report the fourth case of Mallory hyaline in the adenomas of glycogen storage disease type Ia in a 28-year-old woman undergoing segmental hepatectomy for enlarging liver nodules. Other prominent findings included steatohepatitis and nonspecific granulomatous inflammation--2 findings that are commonly seen in sporadic adenomas but not, to our knowledge, previously reported in glycogen storage disease type Ia.- - - - - - - - - - ranking = 18.760532223127keywords = glycogen storage disease type, glycogen storage disease, storage disease type, glycogen storage, storage disease, glycogen, disease type, storage (Clic here for more details about this article) |
9/32. Lower urinary tract function in patients with pituitary adenoma compressing hypothalamus.BACKGROUND: The micturition reflex is under the tonic influence of suprapontine structures including the anteromedial frontal cortex, basal ganglia, and hypothalamus. However, there have been few reports about the role of the hypothalamus on the lower urinary tract (LUT) function in humans. OBJECTIVE: To investigate LUT function in patients with pituitary adenomas. methods: Urodynamic studies were carried out in three patients with LUT symptoms who had pituitary adenomas extending upwards to the hypothalamus. RESULTS: All three male patients (age 28 to 62 years) developed LUT symptoms (urinary urgency and frequency (3); urinary incontinence (3); voiding difficulty and retention (2)) along with weight loss, psychiatric symptoms, unsteady gait, and/or visual disturbances. One had the syndrome of inappropriate secretion of antidiuretic hormone, but none had diabetes insipidus. Two had resection of the tumour and subsequent radiation therapy, but LUT dysfunction persisted. The third patient had partial resection of the tumour to ameliorate hydrocephalus. Urodynamic studies showed detrusor overactivity during the storage phase in all patients; during the voiding phase there was underactive detrusor in two and non-relaxing sphincter in one. CONCLUSIONS: Hypothalamic lesions can cause severe LUT dysfunction in both the storage and voiding phases of micturition. This may reflect the crucial role of the hypothalamus in regulating micturition in humans.- - - - - - - - - - ranking = 0.0069833501654798keywords = storage (Clic here for more details about this article) |
10/32. Budd-Chiari-like presentation of hepatic adenoma.Hepatic adenoma is a benign tumor characterized by its hypervascularity. Hepatic adenoma tends to occur more frequently in women and is related to the use of contraceptive hormones, androgenic/anabolic steroids, pregnancy, glycogen storage diseases and hemochromatosis. Hepatic venous obstruction, or budd-chiari syndrome, is a condition of hepatic vein occlusion that has many causes. A 35-year-old woman presented shortly after pregnancy with a huge cystic lesion in the liver. The lesion compressed the hepatic vein and created an early stage of budd-chiari syndrome. Tumor resection was carried out successfully. The final diagnosis of this case was multiple hepatic adenomas.- - - - - - - - - - ranking = 1keywords = glycogen storage disease, glycogen storage, storage disease, glycogen, storage (Clic here for more details about this article) |
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