1/33. Progressive development of diffuse liver hemangiomatosis.Diffuse liver hemangiomatosis is extremely rare. The etiology and natural history of the disease are unknown. It is also unclear whether tumor growth is induced or modulated by drug therapy. Tumor recurrence after ablative therapy has not been described in patients with diffuse liver hemangiomatosis. Diffuse hemangiomatosis of the left hepatic lobe was suspected in a 35-year-old woman by ultrasonography, CT and hepatic arteriography, and confirmed by laparotomy and biopsies. The patient denied any drug or estrogen use. The tumor was removed by left hepatectomy. Two and six years later, the patient was again hospitalized with progressive tumor growth into the right hepatic lobe. Although diffuse liver hemangiomatosis is a rare disease, its diagnosis should be considered in patients with progressive tumor growth in one or both hepatic lobes. The absence of drug intake or estrogen use does not exclude the diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/33. 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 = rare disease (Clic here for more details about this article) |
3/33. Malignant melanoma with liver and spleen metastases: case report.CONTEXT: The diagnosis of primary melanoma is easily confirmed after histological analysis of the lesion, whereas it is rarely diagnosed when the patient even has distant metastases. DESIGN: Case report CASE REPORT: Malignant melanoma is responsible for about 1% of all deaths caused by cancer in the USA and only 3% of all malignant skin diseases. Malignant melanoma is a rare disease, although it corresponds to 65% of all deaths caused by skin cancer. The liver and spleen are rarely the first sites of melanoma metastases. This paper reports on the clinical picture of a patient with fatal malignant melanoma and hepatic and spleen metastases. As this was an un-usual presentation, the melanoma diagnosis could only be made after pathological analysis of the skin and hepatic lesions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/33. Multiple inflammatory pseudotumors mimicking liver metastasis from colon cancer: report of a case.A 54-year-old man underwent an operation for colon cancer histologically diagnosed as moderately differentiated adenocarcinoma with clinical staging of Dukes C. He was prescribed carmofur for adjuvant chemotherapy. A follow-up computed tomography scan done 6 months later revealed two new low-density areas in the liver. A diagnosis of metastatic adenocarcinoma from the previous colon cancer was presumed, based on the patient's history and radiological findings, and resection of the affected area of liver was performed. Histological examination of these tumors revealed that they were inflammatory pseudotumors (IPT). The patient had an excellent postoperative course and has shown no further signs of recurrence in the 3 years since his last operation. IPT of the liver is a rare disease, for which no methods of diagnosis and treatment have been established, since it is difficult to distinguish IPT from hepatocellular carcinoma or metastatic carcinoma. We describe this case with a review of the 101 cases of IPT documented in the Japanese literature, in the hope that it will contribute to the diagnosis and treatment of this unusual disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/33. Benign regenerative nodules with copper accumulation in a case of chronic budd-chiari syndrome: CT and MR findings.budd-chiari syndrome is a rare disease characterized by hepatic outflow obstruction due to different causes. Hepatic nodules in patients with budd-chiari syndrome are rare and can be due to hepatocellular carcinoma and benign regenerative nodules. Imaging descriptions of regenerative nodules in cirrhotic livers are numerous, but the imaging findings of benign regenerative nodules associated with budd-chiari syndrome have only recently been reported. We present the computed tomographic and magnetic resonance features of benign regenerative hepatic nodules with copper accumulation in a patient with chronic budd-chiari syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/33. Diffuse hepatic hemangiomatosis in an adult.Diffuse hepatic hemangiomatosis without extrahepatic lesions is extremely rare in adults. A case of diffuse hepatic hemangiomatosis involving right lobe in a 50-year-old woman was presented. The hemangiomatosis was demonstrated by ultrasonography, computerized tomography (CT) and magnetic resonance image (MRI), and was confirmed histopathologically. Although diffuse hepatic hemangiomatosis is a rare disease in adults, its diagnosis should be considered in patients with diffuse tumor growth in one or both hepatic lobes and distinguished from malignant tumors. The present case is the first documented case of diffuse hepatic hemangiomatosis in an adult in korea.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/33. A case of fibrous histiocytoma of the liver.Malignant fibrous histiocytoma (MFH) is a rare disease. We describe a 68-year-old man admitted to the hospital because of malaise. On admission, hematologic and serum chemical examinations showed no abnormalities. A tumor measuring 6.0 x 6.0 x 5.5 cm was found in segment S6 of the right lobe of the liver. A computed tomographic scan of the abdomen revealed a mass surrounded by a capsule-like region with a nonuniform shadow at its margin. The mass contained a nonuniform low density area. A magnetic resonance imaging scan showed low intensity on T1-weighted images and high intensity on T2-weighted images. An angiogram of the abdomen revealed a tumor with a darkly stained margin during the venous phase. Partial resection of the liver, including S6 and part of S7, was performed. On histopathological examination, this case was characterized by a storiform pattern. The inside of the tumor showed a storiform-pleomorphic pattern with inflammatory cell infiltration and partial mucinous degeneration. On immunohistochemical studies, the tumor cells stained positively for CD6. The diagnosis was MFH.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/33. Clinical features and imaging diagnosis of biliary cystadenocarcinoma of the liver.Biliary cystadenocarcinoma of the liver is a relatively rare disease. Herein, we reported a case of biliary cystadenocarcinoma with a review of the literature. A 71-year-old female was admitted with the chief complaint of epigastralgia. The imaging studies revealed a biliary cystadenocarcinoma in the left hepatic lobe with suspicion of direct invasion to the left and middle hepatic veins and inferior vena cava. However, there was no direct invasion of the tumor to these veins in operation findings, and an extended left hepatic resection was performed without resection of inferior vena cava. The tumor was histologically diagnosed as biliary cystadenocarcinoma of the liver. diagnosis of biliary cystadenocarcinoma is usually difficult preoperatively, however, a diagnosis was possible with the use of imaging studies. It was suggested that this tumor originated from a benign cystadenoma because of the existence of a transitional zone between normal cells and atypical cells in the cystic wall. Systematic hepatectomy was recommended as the initial treatment in consideration of the features of cystadenocarcinoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/33. Primary hepatic low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type: a case report and review of the literature.Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis a presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin d1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
10/33. Metastatic choriocarcinoma presenting as a bleeding duodenal ulcer.We report a case of a young man with a rare disease, a choriocarcinoma of the gastrointestinal tract presenting as a common entity, a bleeding duodenal ulcer. Pathological findings and strongly positive immunostains of tissue specimens for beta human chorionic gonadotrophin confirmed the entire tumor to be a choriocarcinoma ruling out the possibility of an adenocarcinoma with focal components of choriocarcinoma or a beta human chorionic gonadotrophin producing adenocarcinoma. The pattern of tumor invasion in this case is more suggestive of metastatic than primary involvement of the gastrointestinal tract. The diagnosis of primary gastrointestinal choriocarcinoma is difficult because of the need to meticulously rule out the occurrence of a primary in other organs, which at times regresses spontaneously, a diagnosis made more difficult in this case in which no autopsy was performed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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