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1/21. 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.
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ranking = 1
keywords = hemangioma
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2/21. 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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ranking = 5
keywords = hemangioma
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3/21. 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 = 1
keywords = hemangioma
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4/21. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in the oral mucosa. A case report and review of the literature.

    angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign uncommon entity whose aetiology and pathogenesis is under debate. Clinically, it is characterised by cutaneous papules or nodules. Cases of this entity reported in the oral mucosa are very rare. We describe such a case, discuss the problems of histological differential diagnosis between ALHE and other diseases of the region and review the literature.
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ranking = 4
keywords = hemangioma
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5/21. 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 = 1
keywords = hemangioma
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6/21. 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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ranking = 7
keywords = hemangioma
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7/21. Fine needle aspiration cytology of intraoral epithelioid hemangioma. A report of two cases.

    BACKGROUND: Epithelioid hemangioma (EH) is an uncommon, benign, vascular lesion of unknown etiology. The clinical differential diagnosis is broad and includes both epithelial and vascular neoplasms. In contrast to the histopathology of this lesion, the cytopathology, as obtained by fine needle aspiration biopsy (FNAB), has been described only once before. CASES: Two cases of EH of the oral cavity were evaluated by FNAB. The first case included histologic follow-up, while the second included immunohistochemical analysis of the aspirate material (cell block). The smear characteristics included cohesive epithelioid cells with moderate cytoplasm, ovoid nuclei and small nucleoli that formed occasional abortive vascular channels as well as spindle cell groups and a few larger cells with pleomorphic nuclei and irregular nuclear contours against a largely bloody background. Immunohistochemical staining for CD34 and factor viii-related antigen confirmed the endothelial nature of these proliferations. CONCLUSION: The cytomorphology, an endothelial immunophenotype, and the appropriate clinical presentation should permit diagnostic consideration of EH in the differential diagnosis of an endothelial lesion.
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ranking = 5
keywords = hemangioma
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8/21. Reactive angioendotheliomatosis of the intestine.

    We present a case of reactive angioendotheliomatosis (RAE) of the colon, featuring intravascular proliferation of endothelial cells with histologic resemblance to glomeruloid hemangioma. A 19-year-old Japanese male with an anal fistula was diagnosed endoscopically with Crohn's disease. Six months later, he was hospitalized for fever and abdominal pain. Emergency resection of ileocecum and splenic flexure of the colon was undertaken to control massive intestinal hemorrhage, and in all parts of the resected colon, foci of many small vessels with intravascular proliferation of endothelial cells were noted throughout the layers. Moreover, solid proliferation of endothelial cells was seen in the submucosa at the base of open ulcers. Two small granulomas, compatible with Crohn's disease, were also evident in the muscle layer of the terminal ileum. No other hemangiomas or hemangioma-like structures were observed with CT scans, and the vascular lesions were histologically diagnosed as RAE. The pathogenesis of this disorder is unknown, and most cases occur in skin with systemic disease. The present case might thus be a first case of RAE of the intestine without cutaneous involvement. Whether there is a relation with coexistent enteritis suggestive of Crohn's disease needs to be clarified.
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ranking = 3
keywords = hemangioma
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9/21. Intramuscular angiomatous tumors associated with periosteal changes and pain.

    This case report discusses two cases in which patients present with the uncommon triad of extremity pain, focal periosteal reaction on imaging studies, and intramuscular hemangioma on pathologic examination. Both patients presented in this article were older than those previously reported in the literature. Neither patient reported complete resolution of pain following excision, although there was marked improvement.
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ranking = 1
keywords = hemangioma
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10/21. hemangioendothelioma with an epithelioid phenotype arising in hemangioma of the fibula.

    The classification of certain vascular bone tumors that show an epithelioid cytologic appearance remains confusing, with overlap in features of epithelioid hemangioma, hemangioendothelioma and epithelioid hemangioendothelioma. Radiographs of a 27-year-old woman who presented with ankle pain showed an expanded lytic-sclerotic lesion in the distal left fibula. magnetic resonance imaging showed an intramedullary lesion with a small lateral intracortical component. The lesion was hypo- to isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images. Initial incisional biopsy was inconclusive. Open biopsy showed hemangioendothelioma with epithelioid morphology, and the lesion was completely resected with reconstruction using a peroneal fibular rotation graft. Examination of the resected specimen showed focal hemangioendothelioma with an epithelioid phenotype arising in a hemangioma. This case illustrates the difficulty and pitfalls of making the correct diagnosis on the basis of a small biopsy specimen.
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ranking = 6
keywords = hemangioma
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