1/9. Ruptured hepatic adenoma in liver adenomatosis: a case report of emergency surgical management.In hepatic adenomatosis, multiple liver cell adenomas (usually > or = 10) generally affects patients with no prior history of oral contraceptive use, androgenic steroid use, or glycogen storage disease. We report a rare case of a 44 year-old female who underwent emergency surgery for hemoperitoneum due to spontaneous rupture of a liver cell adenoma in hepatic adenomatosis, after prolonged use of two different contraceptives (Gestodene and Ethinylestradiol).- - - - - - - - - - ranking = 1keywords = storage disease, storage (Clic here for more details about this article) |
2/9. Spontaneous regression of hepatic adenoma in a patient with glycogen storage disease type I after hemodialysis: ultrasonographic and CT findings.A 23-year-old woman was admitted to our hospital with recurrent gouty arthritis. Laboratory findings showed hypoglycemia, lactic acidosis, hyperlipidemia, and hyperuricemia, with normal values of serum alfa-fetoprotein (AFP) and protein induced by vitamin k absence (PIVKA-II). A diagnosis of glycogen storage disease type I (GSD-type I) was made on the basis of the laboratory data, liver biopsy findings, and partially deficient thrombocyte glucose-6-phosphatase (G-6-Pase) activity. ultrasonography and computed tomography revealed multiple focal hepatic masses. Biopsied specimens of the lesion demonstrated a hepatic adenoma, which changed in appearance in the relatively short period between echography and computed tomography. This interesting phenomenon may highlight the importance for careful follow-up of hepatic adenomas, because of the potential of rupture, hemorrhage, or malignant transformation. During follow-up, the present patient received hemodialysis due to renal failure, and the adenoma regressed spontaneously after 8 years. Included are diagnostic images, demonstrating the association of hepatic adenoma and GSD-type I.- - - - - - - - - - ranking = 5keywords = storage disease, storage (Clic here for more details about this article) |
3/9. Liver cell adenoma in a 26-year-old man.This is a report of a case of liver cell adenoma (LCA) in a 26-year-old man with no prior history of liver disease or glycogen storage disease and no record of hormonal therapy. He was found to have an asymptomatic hepatic mass during a routine medical examination. The physical findings were unremarkable, and the results of routine laboratory studies were all within normal limits. Selective hepatic arteriography showed a hypervascular mass within the right lobe of the liver. Despite the radiological examination, the nature of the mass was unknown, and preoperative biopsy was unadvisable because of the risk of bleeding. Because of the difficulty of determining the malignancy of the hepatic tumor preoperatively, elective laparotomy for diagnosis and hepatectomy as treatment appeared to be the best available approach. Pathological examination of the surgical specimen resulted in a diagnosis of LCA. A review of the literature revealed that LCA unassociated with the use of oral contraceptives is rare.- - - - - - - - - - ranking = 1keywords = storage disease, storage (Clic here for more details about this article) |
4/9. Glycogenosis storage type I diseases and evolutive adenomatosis: an indication for liver transplantation.We report on two cases of type I glycogen storage disease (GSD) complicated by malignant tumors. A 23-year-old man had GSD Ia with adenomatosis. He underwent transplantation for rapidly growing and radiologically changing adenomata. At histological examination, one adenoma had become a hepatocellular carcinoma. A 22-year-old, HBV-infected woman had GSD type Ib with adenomatosis. At follow-up, several tumors showed changing morphological characteristics. Pre-transplant laparotomy confirmed the presence of a metastatic cholangiocarcinoma. liver transplantation should be considered in GSD type I patients with adenomatosis, especially when tumor characteristics change. Regular detailed Doppler ultrasound and magnetic nuclear resonance screening during childhood and adolescence are, therefore, mandatory in order for the timing of transplantation to be optimized.- - - - - - - - - - ranking = 1.2781981506798keywords = storage disease, storage (Clic here for more details about this article) |
5/9. Treatment of multiple hepatic adenomatosis using transarterial chemoembolization: a case report.Liver adenomatosis is a rare entity in which multiple liver cell adenomas (more than 10) occur in patients with no prior history of steroid use or glycogen storage disease. This report describes angiographic findings and treatment strategies of a case of liver adenomatosis.- - - - - - - - - - ranking = 1keywords = storage disease, storage (Clic here for more details about this article) |
6/9. Liver cell adenoma: a case report with clonal analysis and literature review.We report a case of liver cell adenoma (LCA) in a 33-year-old female patient with special respect to its clonality status, pathogenic factors and differential diagnosis. The case was examined by histopathology, immunohistochemistry and a clonality assay based on X-chromosomal inactivation mosaicism in female somatic tissues and polymorphism at androgen receptor focus. The clinicopathological features of the reported cases from china and other countries were compared. The lesion was spherical, sizing 2 cm in its maximal dimension. Histologically, it was composed of cells arranged in cords, most of which were two-cell-thick and separated by sinusoids. Focal fatty change and excessive glycogen storage were observed. The tumor cells were round or polygonal in shape, resembling the surrounding parenchymal cells. mitosis was not found. No portal tract, central vein or ductule was found within the lesion. The tumor tissue showed a positive reaction for cytokeratin (CK) 18, but not for CK19, vimentin, estrogen and progesterone receptors. Monoclonality was demonstrated for the lesion, confirming the diagnosis of an LCA. Clonality analysis is helpful for its distinction from focal nodular hyperplasia.- - - - - - - - - - ranking = 0.069549537669944keywords = storage (Clic here for more details about this article) |
7/9. A case of hepatocellular adenomatosis with a follow-up of 11 years.Hepatocellular adenomatosis is characterized by the presence of numerous (arbitrarily > 10) adenomas within an otherwise normal liver without a history of glycogen storage disease or steroid hormone therapy. Although the disease is rare, its importance lies in its tendency to produce symptoms such as abdominal pain and its potential for abdominal hemorrhages. However, the prognosis of hepatocellular adenomatosis remains uncertain. Here we describe the case of a 40-yr-old female with hepatocellular adenomatosis without evidence of serious complications, who was observed over a period of 11 yr.- - - - - - - - - - ranking = 1keywords = storage disease, storage (Clic here for more details about this article) |
8/9. Impact of magnetic resonance imaging on the diagnosis of abdominal complications of paroxysmal nocturnal hemoglobinuria.Magnetic resonance (MR) imaging is a method of choice for assessing vascular patency and parenchymal iron overload. During the course of paroxysmal nocturnal hemoglobinuria (PNH), it is clinically relevant to differentiate abdominal vein thrombosis from hemolytic attacks. Furthermore, the study of the parenchymal MR signal intensity adds informations about the iron storage in kidneys, liver, and spleen. Twelve PNH patients had 14 MR examinations of the abdomen with spin-echo T1- and T2-weighted images and flow-sensitive gradient echo images. Vessels patency and parenchymal signal abnormalities--either focal or diffuse--were assessed. MR imaging showed acute complications including hepatic vein obstruction in five patients, portal vein thrombosis in two patients, splenic infarct in one patient. In one patient treated with androgens, hepatocellular adenomas were shown. Parenchymal iron overload was present in the renal cortex of eleven patients with previous hemolytic attacks. On the first MR study of the remaining patient with an acute abdominal pain showing PNH, no iron overload was present in the renal cortex. Follow-up MR imaging showed the onset of renal cortex iron overload related to multiple hemolytic attacks. Despite the fact that all our patients were transfused, normal signal intensity of both liver and spleen was observed in three of them. MR imaging is particularly helpful for the diagnosis of abdominal complications of PNH.- - - - - - - - - - ranking = 0.069549537669944keywords = storage (Clic here for more details about this article) |
9/9. Hepatocellular adenoma in glycogen storage disease type iv.The development of hepatocellular adenoma has been recognized in association with glycogen storage disease type I and, less often, with glycogen storage disease type iii, but, to our knowledge, it has not been reported in glycogen storage disease type iv. We had the opportunity to study an 11-month-old male infant who underwent orthotopic liver transplantation for cirrhosis that developed in the setting of glycogen storage disease type iv. A clinically unsuspected hepatocellular adenoma was present in the explanted liver. glycogen storage disease type iv should be considered as a potential precursor to the development of hepatocellular adenoma. Recognition of this association is important, both in terms of the differential diagnosis of tumors that occur in this setting and also to anticipate potential complications of this benign neoplasm.- - - - - - - - - - ranking = 9keywords = storage disease, storage (Clic here for more details about this article) |