Cases reported "Hemangioendothelioma"

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1/18. hemangioendothelioma of the spleen: imaging findings at color Doppler, US, and CT.

    The ultrasonographic, color Doppler, and computed tomography findings of an unusual vascular primary tumor of the spleen are reported. A brief clinical and histopathological analysis of this entity is discussed and the differential diagnosis of other primary lesions of the spleen is attempted.
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2/18. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.

    AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.
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3/18. Radionuclide studies in hemangioendotheliomatosis: case report.

    Multiple abnormal radionuclide studies of brain, lungs, kidneys, liver, and spleen were observed in a patient with hemangioendotheliomatosis.
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4/18. thorium induced hepatic hemangioendothelioma. Roentgen-angiographic findings in two additional cases with clinical "inform and consent" problems.

    1. Two additional cases of presumed, thorium-induced hemangioendothelioma of the liver are reported. 2. The angiographic pattern of large venous lakes conforms closely to the pathologic descriitions of this malignancy. 3. To our knowledge, angiography of the thorotrast (and vinyl chloride) liver, before the clinical evolution of malignancy, has not been recorded and may provide information of prognostic and therapeutic value. 4. Incidental identification of thorium deposits in the tissues of clinically healthy patients creates perplexing "inform and consent" problems. Presumably, these must be solved on an individual basis. 5. Because the use of thorotrast has been discontinued since the early 1950s, all physicians reviewing chest and abdominal roentgenograms should be aware of the characteristic appearance of human thorotrast deposits, especially in the liver, spleen and celiac lymph nodes. 6. The number of living thorotrast "carriers" and previous deaths from thorotrast related malignancy seems highly uncertain at this time.
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5/18. hemangioendothelioma of liver and spleen: trauma-induced consumptive coagulopathy.

    The case of a 9-year-old boy with hemangioendothelioma of the liver and spleen who presented with consumptive coagulopathy one month after sustaining a blunt trauma to his abdomen is reported. A contrast enhanced computed tomography scan of the abdomen showed a ruptured spleen with multiple lesions in the liver that were enhancing with contrast. On exploration, the child was found to have splenic rupture with multiple vascular lesions of the liver. A splenectomy with liver biopsy was done. The histopathologic examination found that both the liver and spleen had a similar tumor morphology characteristic of an epithelioid and spindle cell hemangioendothelioma. The child ultimately died of relentless consumptive coagulopathy.
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6/18. hemangioendothelioma of the spleen.

    Hemangioendotheliomas of the spleen are rare and are considered to be of intermediate/borderline malignancy. We report such a case in a patient who presented with chronic anemia but who otherwise was asymptomatic. The tumor involved half the organ and was solitary and nonencapsulated. Microscopically, it was composed of vascular and stromal elements. Both types of elements showed moderate atypia and rare mitoses. The lining cells stained positively with antibodies to factor viii-related antigen and ulex europaeus lectin. The stromal component showed evidence of myofibroblastic differentiation. One year after splenectomy, all hematologic parameters slowly improved and returned to normal. The clinicopathologic differences between hemangioma, angiosarcoma, and hemangioendothelioma are discussed, and cases that have recently been reported in the literature are reviewed.
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7/18. Primary large cell lymphoma of the splenic sinuses: a variant of angiotropic B-cell lymphoma (neoplastic angioendotheliomatosis)?

    A case of large cell lymphoma of B-cell lineage originating in the splenic sinuses is described. In addition to widening the spectrum of primary malignant lymphomas of the spleen, this case raises the possibility that variants of angiotropic large cell lymphomas may exist that do not involve blood vessels but do involve the spleen and lymph node sinuses.
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8/18. Clinics in diagnostic imaging (89). Infantile hepatosplenic haemangioendotheliomas.

    The ultrasonographical and computed tomography findings of a six-month-old female infant with haemangioendotheliomas of the liver and spleen are described. The splenic lesion had an unusual ultrasonographical appearance of a well-defined isoechoic mass with a hypoechoic rim. diagnosis was confirmed by histological examination of the hepatic biopsy specimen. Hepatosplenic lesions in the first year of life may be due to a variety of pathological processes. It is important to include haemangioendotheliomas in the differential diagnosis of hepatosplenic masses in an infant.
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9/18. Myoid angioendothelioma of the spleen.

    Although vascular neoplasms of the spleen are rare, they are the most common nonhemopoietic proliferation of the organ, and include hemangiomas, lymphangiomas, hamartomas, littoral cell angiomas, hemangioendotheliomas, and angiosarcomas, as well as the recently described myoid angioendothelioma (MA). MA is an uncommon, benign tumor of the spleen, which is morphologically characterized by a composite of vascular spaces and stromal cells with myoid features. In 1999, in the only report of this unusual neoplasm, Kraus and Dehner described the features of 3 cases. We present another case of MA of the spleen occurring in a 51-year-old man that demonstrated the characteristic morphologic and immunohistochemical features of this neoplasm. In addition to the features described by Kraus and Dehner, our case also displayed the previously unreported findings of focal spindling of the stromal cells and scattered S100-positive cells in the stroma. The case was further unique in having a central stellate scar. Careful attention to histology, possibly with the aid of immunohistochemistry, should distinguish other splenic neoplasms from MA. Although MA is a morphologically distinct lesion, its histologic spectrum, biological behavior, and relationship to other vascular tumors are yet to be fully discovered. It is hoped that the recognition of further cases, and the use of newer molecular technologies, will help better define the nosological position and implications of the diagnosis of this unusual tumor.
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10/18. Epithelioid and spindle-cell hemangioendothelioma of the spleen. Report of a distinctive splenic vascular neoplasm of childhood.

    A case of a distinctive vascular neoplasm of the spleen in a 3-year-old boy is described. The tumor was characterized histologically by a biphasic growth pattern, with discrete nodular areas composed of atypical round, epithelioid cells with large nuclei and prominent nucleoli, and areas showing an intricate proliferation of vascular channels lined by elongated spindle cells. Immunohistochemical studies showed cytoplasmic staining of the tumor cells with factor viii-related antigen, ulex europaeus lectin, and vimentin antibodies. Stains for keratin, actin, desmin, lysozyme, and S-100 protein were negative in the tumor cells. Electron microscopy revealed a fairly cohesive population of cells that contained mature and immature cell junctions, basal lamina material, and surface pinocytotic activity consistent with vascular endothelial cells. Five-year follow-up has shown the patient to be alive and free of disease. This case appears to represent a previously unreported primary vascular neoplasm of the spleen showing combined features of epithelioid and spindle-cell hemangioendothelioma. The lesion should be distinguished from other benign and malignant vascular proliferations of the spleen such as Kaposi's sarcoma, angiosarcoma, and the recently described littoral-cell angioma.
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