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1/8. 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.
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2/8. 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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3/8. 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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4/8. Epithelioid hemangioendothelioma of the lung diagnosed by transesophageal endoscopic ultrasound-guided fine needle aspiration: a case report.

    BACKGROUND: Epithelioid hemangioendothelioma is a rare vascular tumor of the lung and is also known as intravascular sclerosing bronchoalveolar tumor. Although it has relatively low malignant potential, extensive pulmonary involvement and systemic metastasis have been described. The cytologic features of these tumors are not very well defined, with only few case reports describing the cytologic findings of epithelioid hemangioendothelioma of the lung on fine needle aspiration. CASE: endoscopic ultrasound-guided fine needle aspiration of a hilar mass was performed on a 25-year-old female. The cytology showed loosely cohesive sheets and clusters of epithelioid cells. The cellular features included large, irregular nuclei with nucleoli and a moderate amount of vacuolated cytoplasm. Rare cells had a suggestion of cytoplasmic lumen formation. Histologic examination of tissue fragments on the cell block revealed a tumor composed of rounded to spindled epithelioid cells in a background of light blue stroma. The endothelial differentiation was evidenced by cytoplasmic vacuoles and lumens, some of which contained erythrocytes. The endothelial nature of these cells was confirmed by positive staining with factor viii and CD34. CONCLUSION: The cytomorphologic features of epithelioid hemangioendothelioma described in the literature and observed in our case are distinctive and can help with the interpretation of cytologic smears and prevent misdiagnosis.
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5/8. High resolution CT findings of pulmonary epithelioid hemangioendothelioma: unusual manifestations in 2 cases.

    Pulmonary epithelioid hemangioendotheliomas (PEH), also known as intravascular sclerosing bronchoalveolar tumor, is a rare vascular tumor of the lung common among young women. Primitive lumena lined by single cells is the characteristic pathologic feature. The endothelial nature of these cells is confirmed by positive staining with factor viii and CD34. PEH usually presents as single or multiple pulmonary nodules. The present report describes high resolution CT (HRCT) findings of 2 cases with unusual manifestations of PEH. One case was a 54-year-old woman with multiple pulmonary nodules with irregular thickening of both the bronchovascular bundles and perilobular structures, representing intensive lymphangitic spread on HRCT. The other was an 18-year-old woman who had multiple minute peripheral nodules in the lungs bilaterally. These HRCT findings demonstrated the presence of tumor nodules in the lymphatic spaces, which is quite an unusual histologic presentation for this tumor. Both cases also showed hepatic lesions on abdominal CT; the former showing hypoattenuating masses with coarse calcifications and the latter showing multiple tiny calcifications in the hepatic parenchyma. Recognition of these features in the appropriate clinical setting may allow the clinician and the pathologist to consider this rare tumor.
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6/8. Cerebral metastasis of an atrial myxoma mimicking an epithelioid hemangioendothelioma.

    A 60-year-old woman presenting with a 15-month history of Jacksonian seizures was found on computed tomography (CT) scan to have an enhancing subcortical lesion high in the left anterior parietal lobe. The excised tumor had light microscopic features similar to those of an epithelioid hemangioendothelioma, and immunohistochemical stains confirmed its vascular nature. At surgery, the patient had no clinical or radiological (including CT body scan) evidence of any other lesion. However, 22 months after symptoms first appeared, she presented with dyspnea and bilateral pleural effusions, and a left atrial myxoma was detected echocardiographically. The histologic features of the resected myxoma were identical to those of the previously excised cerebral lesion, and it became apparent that the intracranial tumor was a metastasis of the atrial myxoma. This case illustrates that before a diagnosis of visceral epithelioid hemangioendothelioma is rendered, the possibility of metastatic occult cardiac myxoma should be considered. echocardiography is warranted for such patients.
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7/8. Epithelioid haemangioma of the heart.

    The authors report a case of epithelioid haemangioma (EH) of the right atrium, the first description of this tumour originating in the heart. The lesion was found incidentally during a cardiac echocardiogram and diagnosed pre-operatively as cardiac myxoma. The tumour must be differentiated from the exceptionally rare epithelioid haemangioendothelioma (EHE) of the heart and from a cardiac myxoma. A correct pathological diagnosis is clinically important since EH is a benign tumour, whereas EHE and cardiac myxoma can recur and metastasize. The uneventful follow-up of this patient confirms the benign nature of EH.
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8/8. Subtotal liver calcification due to epithelioid hemangioendothelioma.

    A hepatic epithelioid hemangioendothelioma leading to almost complete calcification of the right and left liver lobes in a 75-year-old female is reported. Over a period of 16 years the liver progressively calcified, but its function remained normal by compensatory hypertrophy of the caudate lobe. Multiple partially calcified metastases were present in the lungs, peripancreatic region and along the left principal bronchus. Extreme liver calcification has only rarely been reported. The case presented here reflected slow tumor growth and subsequent long disease course. The vascular nature of the tumor was confirmed by immunohistochemical and ultrastructural analysis.
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