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1/160. Epithelioid hemangioendothelioma with solitary bone location associated to multiple lung and liver lesions. A case report.

    Progressive development of a lobulated osteolytic lesion of the left calcaneus was observed in a 28-year-old woman. The bone tumor was associated with multiple lung nodules and later with a few small nodular liver lesions. A histological and immunochemical diagnosis of epithelioid hemangioendothelioma was obtained by bone tumor resection and thoracoscopic lung biopsy. Imaging aspects are reported with emphasis on its multifocality. ( info)

2/160. Intraosseous epithelioid hemangioendothelioma of the mandible: a case report with an immunohistochemical study.

    Epithelioid hemangioma is the prototype of a group of vascular tumors characterized by epithelioid endothelial cells. hemangioendothelioma of bone is a rare lesion that constitutes less than 0.5% of primary malignant skeletal tumors. We report and discuss a case of epithelioid hemangioendothelioma arising intraosseously in the anterior portion of the mandible in a 76-year-old woman. The case was treated successfully by wide resection. Radiographically, the tumor mass showed osteolysis and expansion. Histologically, the tumor showed invasive and destructive growth, although it lacked frequent mitotic figures and severe atypia. On immunohistochemical study, tumor cells exhibited characteristics of mesenchymal and endothelial origin, i.e., strong to moderate immune reactivity against vimentin, factor viii-related antigen (F8RA), ulex europaeus agglutinin type 1 lectin (UEA-1), and CD 34, but not against keratin, epithelial membrane antigen (EMA) or S-100 protein (S100). The proliferating cell nuclear antigen (PCNA)-positive cell index was 27.5%. These pathological findings suggested a borderline malignant potential for this tumor. Thus, clinically, wide resection with or without dissection of regional lymph nodes is recommended. ( info)

3/160. Epithelioid haemangioendothelioma.

    Epithelioid haemangioendothelioma is a rare pulmonary neoplasm with less than 40 cases described world wide. We describe the only case to have presented with hypertrophic pulmonary osteoarthropathy who has been treated with azathioprine and has remained alive and well with no deterioration in pulmonary function since being diagnosed 16 years ago. The progression of the chest radiograph and spiral CT appearances of this rare neoplasm are described, and current views regarding the cellular origin of the neoplasm, its cytological appearance, clinical presentation and prognosis are discussed. ( info)

4/160. 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. ( info)

5/160. Epithelioid haemangioendothelioma of the lung: a high malignancy case.

    Pulmonary epithelioid haemangioendothelioma (PEH) is a rare lung tumour of vascular origin and low-grade malignancy that affects various organs: liver, central nervous system, lung, etc. A rare malignant case of PEH is described. The cryptic clinical appearance and the nonspecific results of radiography and haematological tests caused great difficulty in arriving at a definite diagnosis. Histological analysis and immunohistochemical detection of CD31 (a membrane receptor and a sensitive and specific marker for vascular lesions), on open lung biopsy, enabled the correct diagnosis to be made. ( info)

6/160. 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. ( info)

7/160. 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. ( info)

8/160. Epithelioid hemangioendothelioma of the greater omentum: report of a case.

    We report herein the case of an 11-year-old girl found to have an epithelioid hemangioendothelioma of the greater omentum. The child presented with a 4-month history of vague lower abdominal pain and gradually increasing generalized distension. Her symptoms and signs could not be ascribed to any specific organ system. ultrasonography and computerized axial tomography revealed a mass lesion anterior to the bowel loops and a laparotomy revealed a huge lobulated mass, arising from the greater omentum, which was completely excised. She has remained well postoperatively without any adjunctive chemotherapy or radiotherapy. ( info)

9/160. Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy.

    Epithelioid haemangioendotheliomas are rare tumours of endothelial origin. They can develop in any tissue but occur principally in the lung and liver. Their usual course is a slow progression, so that they can be treated by surgery. In aggressive forms, no treatment has proved efficient to date. This study, describes a case of bilateral pleural epithelioid haemangioendothelioma that extended to the peritoneum. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. After six courses of carboplatine plus etoposide, a complete response was obtained. The complete remission is still lasting at 18 months after the diagnosis and the patient is healthy. ( info)

10/160. Ulcerated cutaneous epithelioid hemangioendothelioma.

    Epithelioid hemangioendothelioma described first by Weiss and Enzinger in 1982 is an uncommon vascular tumor usually involving soft tissue, less frequently the lung and the liver and exceptionally the skin. We herein report a 52-year-old woman who presented an isolated moderately painful persistant ulceration of the concha of her left ear. Histopathological findings showed strands and nests of epithelioid endothelial cells typical of cutaneous epithelioid hemangioendothelioma. Immunohistochemical stainings confirmed the vascular nature of the tumor. Surgical excision by ear amputation was performed. In a review of the literature, to our knowledge, this clinical presentation as ulceration has never previously been reported. ( info)
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