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1/36. 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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2/36. 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 = 246.56234441637
keywords = intermediate
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3/36. Epithelioid hemangioendothelioma of the parotid salivary gland.

    Epithelioid hemangioendothelioma is a borderline neoplasm characterized by proliferation of endothelial cells with epithelioid morphology. The tumor, in terms of histology and behavior, occupies an intermediate position between hemangioma and conventional angiosarcoma. It is encountered in a wide variety of sites, such as soft tissues and skin, visceral organs, and bone.This article describes a slowly growing, painful tumor that developed in the parotid gland of a 48-year-old white woman and was treated with a superficial parotidectomy. Microscopically, the lesion consisted of short strands, cords, or small clusters of epithelioid vacuolated cells that exhibited strong immunoreactivity for endothelial cell markers (CD 31, CD 34, and factor viii-related antigen).Previous reports have presented epithelioid hemangioendotheliomas in the head and neck region but, to our knowledge, this is the first reported case in the parotid salivary gland.
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ranking = 248.10618857533
keywords = intermediate, gland
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4/36. Epithelioid hemangioma of bone.

    Epithelioid hemangioma of bone is a rare type of angiomatous tumor. We report a documented case of epithelioid hemangioma occurring in the distal femur of a 35-year-old man. The clinical, radiographic, MR imaging and histologic findings of the tumor are described. Radiographs showed a well-defined expanding, osteolytic lesion in the diaphysis of the femur. MR imaging showed the lesion to have low signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images. Grossly the lesion was multiloculated with a dark brown, jelly-like content. To characterize the imaging appearances of epithelioid hemangioma, we reviewed the available literature on the subject.
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keywords = intermediate
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5/36. Epithelioid haemangioendothelioma of the brain: a case report.

    We report a rare epithelioid haemangioendothelioma in the frontal lobe. The CT and MRI findings are characteristic and correspond to histological features intermediate between those a cavernous haemangioma and an aggressive vascular tumour.
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6/36. Epithelioid hemangioendothelioma with osteoclast-like giant cells.

    Epithelioid hemangioendothelioma is an unusual entity of intermediate malignant vascular tumors and rarely admixes with multinucleated osteoclast-like giant cells. We describe such a case in a 50-year-old woman with an 1-year follow-up period. When the patient was 39 years old, a 0.7 x 1-cm firm mass was found in the left neck. She developed ascites three years later. At that time, the specimen from the neck mass was excised and found to be composed of cords of epithelioid cells in a myxoid hyaline stroma. Scattered intracytoplasmic vacuoles were seen. Immunohistochemical studies confirmed the diagnosis of epithelioid hemangioendothelioma. Abdominal ultrasonography and computed tomographic scan revealed multiple low density areas in the liver. Eleven years later, a metastatic lesion was found in the thoracic spine at the level of Th7-8. The histologic findings were basically similar to those of the left neck mass. However, because of the presence of Kp-1-positive multinucleated osteoclast-like giant cells throughout the tumor, the thoracic lesion was diagnosed as an epithelioid hemangiothelioma with osteoclast-like giant cells. This unique form of epithelioid hemangioendothelioma is extremely rare and should be distinguished from other benign and malignant tumors with osteoclast-like giant cells.
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ranking = 246.56234441637
keywords = intermediate
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7/36. Primary epithelioid hemangioendothelioma originating in the lower petroclival region: case report.

    BACKGROUND: We report the first case of primary epithelioid hemangioendothelioma (EH) originating in the lower petroclival region. CASE DESCRIPTION: A 55-year-old female presented with a 45-year history of subclinical atrophy on the right side of her tongue and a 15-year history of hoarseness. neuroimaging revealed an expansile, homogeneously enhanced intraosseous mass with bony shell and honeycomb configuration in the petroclival region. A right far lateral transcondylar approach was utilized for subtotal removal of the tumor, which demonstrated high vascularity in the petroclival bone. Histopathological and immunohistochemical examination confirmed the diagnosis of EH. Because of the intermediate malignancy, adjuvant gamma knife radiotherapy was performed for the residual mass 5 months after surgery. CONCLUSION: EH rarely occurs in the skull base region. The appearance of surrounding bony structure and rich vascularity are important findings for the differential diagnosis. A far lateral transcondylar approach provides sufficient exposure of the jugular tubercle and lower clivus. Additional radiotherapy may be recommended for residual tumors with a high MIB-1 labeling index.
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ranking = 246.56234441637
keywords = intermediate
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8/36. Epithelioid and retiform hemangioendothelioma of the skull bone--report of four cases.

    hemangioendothelioma (HE) is a borderline or intermediate type of vascular neoplasm. We report clinical and histopathological characteristics of four cases of HE arising from the skull bones because of its extreme rarity in this location. The age of the patients ranged from 6-45 years. Three patients presented with a painless swelling over the head and one case had sphenoid wing mass with dimness of vision and proptosis. Radiographic images showed a well-demarcated, osteolytic lesion in the skull bone in all the four, one case in addition had sclerotic edges and another had specks of calcification. Grossly, the tumour was very vascular with hemorrhagic areas. Histologically, three cases showed features of an epithelioid variant of HE, with short strands and solid nests of rounded to slightly spindled, eosinophilic endothelial cells, some of them having small intracellular vacuoles. The stroma was myxoid--hyalinised with focal mixed inflammatory infiltrate. One case had features of a 'retiform' histological variant composed of numerous elongated vessels lined by a single layer of hobnail endothelial cells, focal lymphocytic infiltrate and papillae with hyaline collagenous cores. The tumour cells in all the four were immuno-labelled by antibody to factor viii-associated protein. The tumour cells lacked cytological atypia and mitosis was sparse. These features were important in prognostication as low-grade tumours can be cured by complete wide-resection.
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ranking = 246.56234441637
keywords = intermediate
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9/36. Malignant intracranial epithelioid hemangioendothelioma presumably originating from the lung: case report.

    OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.
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keywords = lobe
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10/36. Epithelioid haemangioendothelioma of the parotid salivary gland: a case report.

    Haemangioendothelioma is a vascular tumour characterised by the proliferation of endothelial cells with an epithelioid appearance. The behaviour of this neoplasm is intermediate between haemangioma and angiosarcoma. It may be localised in a wide range of sites, with a preference for soft and bone tissue. It is only rarely localised in the head and neck and even more rarely in the salivary glands. We describe a case of haemangioendothelioma in a 28-year-old man that originated in the retroneural region of the parotid gland, compressing the gland tissue and posteriorly infiltrating the muscular plane. A total parotidectomy surgical operation including the removal of lymph nodes in the region was performed followed by radiotherapy. An immunohistochemical investigation, carried out using the oxidase-antiperoxidase method, indicated that the neoplastic elements of the marker of the endothelial cells CD34 were positive for vimentine and for muscle-specific actin, showing a moderate proliferative action of the cellular elements with MIB-1 positivity estimated at around 6%. The peculiarity of the case we describe resides in the rarity of the haemangioendothelioma localisation in the parotid gland.
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ranking = 248.62080329498
keywords = intermediate, gland
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