1/8. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/8. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/8. Spinal epidural epitheloid hemangioma--case report and review of the literature.Epitheloid hemangiomas are benign vascular lesions composed of epitheloid endothelial-cell-lined channels. We report an unusual case of a purely extradural non-osseous spinal epitheloid hemangioma presenting with quadriparesis. A 5-year-old boy presented with neck pain and quadriparesis following a trivial fall. MRI revealed a mixed intensity extradural lesion compressing the cervico-thoracic spinal cord posteriorly. At surgery the lesion consisted of vascular granulation tissue admixed with blood clots without abnormal vessels. Bone was normal. Histopathology revealed it to be an epitheloid hemangioma. The case and relevant literature is reviewed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/8. Disseminated epitheloid hemangioendothelioma mimicking symptoms of systemic vasculitis.BACKGROUND: Epitheloid hemangioendothelioma is a rare malignant tumor which can involve bones, liver, lungs, kidneys, deep soft tissue, muscles, dermis, and central nervous system. Multifocal disease occurs in 10% of the cases. The clinical presentation results from occlusion of small blood vessels due to the disease itself or as a paraneoplastic syndrome. CASE REPORT: We present a patient with symptoms suggesting systemic vasculitis (ESR and CRP elevated, weight loss, arthralgia, livedoid rash, and skin ulcerations) who finally was diagnosed having a disseminated epitheloid hemangioendothelioma when PET scan revealed hypermetabolic multifocal skeletal and soft tissue lesions. DISCUSSION: Diseases mimicking systemic vasculitis and the value of PET in this setting are discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/8. Epithelioid hemangioendothelioma of the spinal cord: Description of a case with cytogenetic analysis.A case of epithelioid hemangioendothelioma of the cauda equina is reported. The patient presented with rapidly worsening low back pain. magnetic resonance imaging revealed a sharply demarcated intradural lumbar lesion. A bluish-red lesion, attached to the filum terminale, was removed. The patient is alive without evidence of recurrence 18 months after surgery. The tumor was composed of variously sized vessels lined by epithelioid endothelial cells with clear cytoplasm and centrally located, moderately atypical nuclei. These cells were immunoreactive for CD31 and factor viii antibodies. cytogenetic analysis disclosed two clones: 44-45X, - Y [cp3]/46XY[11]. Epithelioid hemangioendothelioma may arise in several sites, the most common being soft tissues. It is a borderline tumor that may recur, may metastasize, and rarely causes death. The present case appears to be the first example of epithelioid hemangioendothelioma of the spinal cord.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/8. Primary intraoral epithelioid hemangioendothelioma presenting in childhood: review of the literature and case report.Epithelioid hemangioendothelioma (EH) is a recently described vascular neoplasm of borderline or intermediate malignant potential. This tumor arises from medium- to large-sized vessels, primarily involves the soft tissues of the extremities as well as the liver and lung, and rarely occurs in the head and neck region. Only eight well-documented cases of intraoral EH have been reported. We present an additional pediatric case of EH confined to the oral cavity and review the literature regarding EH presenting as an intraoral mass. EH is characterized histopathologically as an epithelioid lesion arranged in nests, strands, and trabecular patterns with infrequent vascular spaces. Occasional erythrocytes within intracytoplasmic lumina may be seen in tumor cells. Ultrastructural examination typically shows intracytoplasmic lumina with pseudopodial cellular membrane extensions. The cytoplasm usually contains intermediate filaments infrequently associated with weibel-palade bodies. Neoplastic cells are immunoreactive for factor VIIIR:Ag and ulex europaeus. Histopathologic features, which may be associated with aggressive clinical behavior, include significant cellular atypia, one or more mitoses per 10 high-power fields, an increased proportion of spindled cells, focal necrosis, and metaplastic bone formation. Because of the intermediate malignant potential of epithelioid hemangioendothelioma, complete tumor resection is recommended for intraoral lesions.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/8. Alveolar haemorrhage revealing epithelioid haemangioendothelioma.Epithelioid haemangioendothelioma of the pulmonary vessels is a rare neoplasm which usually has a minimal clinical expression. The present case report describes an exceptional case with severe inaugural alveolar haemorrhage which led to death by acute respiratory failure within a few weeks.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/8. Multiple cutaneous epithelioid hemangioendothelioma: a case with spindle cells.A case of multiple cutaneous epithelioid hemangioendothelioma (EH) in a Japanese male is reported. The patient was a 52-year-old Japanese bureaucrat. Clinically, about 10 purplish-red, elastic soft or hard papules and nodules had appeared on both lower extremities and the left buttock over the past 20 years. They were excised, but some of them recurred. Histologically, the papules and nodules were well-circumscribed tumors located in the superficial and deep dermis. They appeared to arise from medium-sized vessels and were composed of proliferations of characteristic epithelioid endothelial cells with intracytoplasmic vacuolization and spindle cells. No other extracutaneous tumors were found. We diagnosed the present case as multiple cutaneous EH with spindle cells. The presence of epithelioid endothelial cells and absence of pleomorphism and mitoses of nuclei of spindle cells made differential diagnosis from Kaposi's sarcoma possible. The protracted clinical course seems characteristic of EH in various organs including skin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |