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1/29. Congenital hemihypertrophy and epithelioid haemangioendothelioma in a 10-year-old boy: a case report.

    Epithelioid haemangioendothelioma has not been previously described in a patient with congenital hemihypertrophy and diabetes mellitus. Hepatic nodules were incidentally discovered on a routine US examination searching for known associated abnormalities. Pulmonary nodules were present on chest X-ray and CT of the lungs. The diagnosis was confirmed by open biopsy of a hepatic nodule. Despite significant disease progression the patient remains symptom free.
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keywords = hepatic
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2/29. Epithelioid hemangioendothelioma, multiple focal nodular hyperplasias, and cavernous hemangiomas of the liver.

    Malignant vascular neoplasms of the liver are uncommon. We report the case of a young woman who developed an epithelioid hemangioendothelioma of the liver associated with multiple focal nodular hyperplasias and hepatic cavernous hemangiomas. Such an unusual association is probably not fortuitous and could support the theory that focal nodular hyperplasia is a reaction to an abnormal vascular supply rather than a true neoplasm.
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keywords = hepatic
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3/29. Epithelioid hemangioendothelioma with marked liver deformity and secondary budd-chiari syndrome: pathological and radiological correlation.

    A case of malignant epithelioid hemangioendothelioma of the liver in a 48-year-old woman with severe portal hypertension and marked deformity of the liver is presented. This woman had a history of mild liver dysfunction since the age of 30 years, and abdominal distention, esophageal varices, splenomegaly and ascites since October 1996. Imaging examinations revealed liver deformity with severe atrophy of the left lobe and the anterior segment of the right lobe. Celiac arteriography showed narrowing and upward deviation of the proper hepatic artery, and occlusion of the left and right anterior hepatic arteries. Since March 1997, hepatic venography showed stenosis in the right hepatic vein truncus. budd-chiari syndrome was clinically diagnosed. She died in June 1997. The autopsy disclosed massive tumor embolism in the left and right anterior portal branches, few in the hepatic artery, and occlusion of the left and right anterior hepatic arteries. The extensive tumor embolism resulted in portal hypertension, and atrophy of the left lobe. The anterior segment of the right lobe was probably caused by the occlusion of both the hepatic arteries and the portal veins. The posterior segment of the right lobe, without massive tumor embolism in its portal branch, appeared hypertrophic.
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ranking = 7
keywords = hepatic
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4/29. Primary hepatic epithelioid hemangioendothelioma: case report.

    Hepatic epithelioid hemangioendothelioma (HEH) is a very rare vascular tumor of the liver. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It poses special difficulties for clinicians in its diagnosis and treatment because of its non-specific clinical manifestations and findings on imaging, and it is easy to be misdiagnosed pathologically. Its clinical course and prognosis are variable but supposed to be intermediate between hemangioma and angiosarcoma. The primary treatments of choice are radical resection or liver transplantation. We report a 62-year-old man with right upper quadrant abdominal pain of several days' duration, who was initially misdiagnosed as having a liver abscess. Finally, HEH was diagnosed on the basis of positive immunohistochemical staining for factor viii-related antigen in tumor cells. This case could serve to highlight the pitfalls in diagnosing this rare tumor. Increasing the index of suspicion and familiarity with the radiological and histological characteristics of this tumor would facilitate the accurate diagnosis and thus avoid unnecessary interventions.
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ranking = 5
keywords = hepatic
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5/29. Hepatic epithelioid hemangioendothelioma: MR imaging findings.

    We describe the sonographic, computed tomography and magnetic resonance imaging findings of two patients with histologically proven hepatic hemangioendothelioma. Both patients presented with multiple liver nodules. color Doppler ultrasound demonstrated moderate vascularity at the periphery of the nodules, as well as central neovascularity. On enchanced computed tomography images, the lesions showed peripheral enhancement only. On magnetic resonance images, tumor signal was low on T1-weighted and moderately high on T2-weighted images. The distinction between normal liver and tumor was difficult on all sequences. Administration of superparamagnetic iron oxide particles delineated well the tumor by suppressing the signal of the normal liver and better depicted areas of remaining healthy parenchyma.
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keywords = hepatic
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6/29. Chemoembolization followed by orthotopic liver transplant for epithelioid hemangioendothelioma.

    Hepatic epithelioid hemangioendothelioma (HEHE) is a rare liver tumor with an indolent course relative to other hepatic malignancies. Over the past two decades, primary treatment for these lesions has been defined as resection for localized disease, or transplantation for diffuse and multifocal tumors. No published report to date has described effective pre- or post-operative adjuvant treatment for this disease. In this report, we present the first case of HEHE effectively managed with chemoembolization followed by transplantation, documenting objective tumor response to embolization. Furthermore, diagnosis for this lesion can easily be mistaken, directing management in erroneous directions. This case illustrates diagnostic pitfalls affiliated with the work-up of this tumor.
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keywords = hepatic
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7/29. hemangioendothelioma of the liver associated with a solitary extrahepatic lesion.

    Epithelioid hemangioendothelioma of the liver is very uncommon and is quite difficult to distinguish from a cholangiocarcinoma or a metastatic liver disease by imaging diagnostics. However, the histological features are characteristic for this soft-tissue tumor. Although liver tumor is usually multifocal and a liver resection is not possible in most cases, we report a case of a multifocal epithelioid hemangioendothelioma of the liver that was completely resected. The tumor was associated with a solitary extrahepatic lesion, which was first discovered six months after the liver resection and which was also resected.
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ranking = 5
keywords = hepatic
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8/29. A case of hypertrophic osteoarthropathy associated with epithelioid hemangioendothelioma.

    Epithelioid hemangioendothelioma is a rare vascular tumor, which occurs in the lung, liver, bone, and soft tissue. Hypertrophic osteoarthropathy is a syndrome characterized by subperiosteal new bone formation, joint effusion and clubbing, and may be associated with cyanotic heart disease, chronic pulmonary disease, liver disease, and other miscellaneous diseases. The activation of endothelium and platelets has been suggested to be involved in the development of hypertrophic osteoarthropathy. We report a rare case of hypertrophic osteoarthropathy, which developed in association with hepatic epithelioid hemangioendothelioma with pulmonary metastasis. We also discuss the role of vascular endothelial growth factor in its pathogenesis.
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ranking = 1
keywords = hepatic
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9/29. Three-chamber priapism in a patient with primary epithelioid hemangioendothelioma of penis.

    A 58-year-old man presented with a 6-month history of painful progressive penile firmness, initially diagnosed as Peyronie's disease. Penile fibrosis involved the entire corpora cavernosa and spongiosum, making it consistent with three-chamber priapism. Cavernosal biopsies revealed epithelioid hemangioendothelioma, and the metastatic workup found hepatic and pulmonary lesions. The patient was treated with paclitaxel, but eventually died of cancer progression. Early infiltrative vascular malignancies of the penis may be indistinguishable from Peyronie's disease. A review of published reports revealed that penile masses associated with progressive growth, obstructive urinary symptoms, dysuria, or painful erections might warrant further evaluation with biopsies.
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ranking = 1
keywords = hepatic
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10/29. thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma.

    Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver with an unpredictable malignant potential. Its growth can lead to hepatic failure, extrahepatic metastasis and death. Surgical resection or liver transplantation is the treatment of choice if metastasis is not identified. Several antineoplastic agents have been proposed for cases of nonresectable HEH. We report the case of a 52-year-old patient with HEH metastatic to the lungs who was successfully treated with oral thalidomide therapy.
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ranking = 6
keywords = hepatic
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