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1/24. 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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2/24. Aggressive epithelioid hemangioendothelioma of the lower extremity: a case report and review of the literature.

    A case of epithelioid hemangioendothelioma (EHE) of the left lower extremity in a 50-year-old male is discussed. The neoplasm presented as a tan-purple, painful mass involving the fourth digit of the left foot. Following an excisional biopsy and partial amputation of the fourth digit, the lesion recurred with a multifocal distribution. A physical exam following the recurrence demonstrated multiple palpable lymph nodes in the left inguinal region. A complete amputation of the fourth digit was performed in conjunction with local excisions of the remaining lesions. Additionally, an inguinal lymphadenectomy was performed for staging purposes. Histologically, the tumor was predominantly composed of epithelioid endothelial cells forming nests, cords, and sheets and occasionally forming irregular, primitive vascular structures. Less commonly, nests of tumor cells assumed a spindled morphology. Patches of necrosis and hemorrhage were apparent throughout the neoplasm. There was minimal atypia and mitoses were not identified. Immunohistochemical stains for CD34, CD31, and ulex europaeus antigens were positive in tumor cells. This case illustrates the potential aggressive behavior of this rare neoplasm and argues against the designation "tumor of low-grade malignancy" with which it is commonly attributed.
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3/24. 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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4/24. 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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5/24. 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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6/24. Hepatic epithelioid hemangioendothelioma.

    Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin with unpredictable malignant potential. We describe two patients with primary EHE of the liver and review 34 cases previously published in japan and compare them with those in Western countries. The clinical aspects of EHE and relevant treatment results in Japanese patients were similar to those found in Western countries, the exception being the matter of difference in incidence between genders. Although this tumor type has been reported to be more frequent among women, there was no significant difference in incidence between men and women. Hepatic EHE generally behaves as a low-grade malignant tumor with a slow progression phenotype; however, this disease seems to be resistant to chemotherapy and to be lethal in some cases. Surgical resection or liver transplantation is recommended after rapid diagnosis by radiologic examination and histologic findings, including positive staining of tumor cells for factor viii-related antigen.
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7/24. Epithelioid haemangioendothelioma of the sphenoid bone.

    The authors report a case of cranial EH occurred in the left sphenoid bone that was totally excised without transfusion. A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye. A cranial X-ray showed a mixed osteolytic and sclerotic expansile mass lesion in the left sphenoid bone. Neuroradiologic imaging study revealed a left temporosphenoidal extra-axial expansile mass lesion with heterogeneous enhancement after contrast enhancement associated with destruction and erosion of the temporal and sphenoid bones. The cerebral parenchyma and orbital contents were compressed without any sign of infiltration. Bone scan showed hot uptake in the left orbital region. Angiogram demonstrated marked neoplastic angiogenesis from the middle meningeal artery and other branches of left external carotid artery, for which preoperative embolisation was tried in vain. Cranio-orbito-zygomatic approach was undergone in two stages because of the patient's religious belief (patient was Jehovah's witness) and profuse bleeding during the first surgery. Two weeks after initial operation, second surgery was carried out with total excision of the residual mass in the left orbital ridge. Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor viii-related antigen. The authors report an unusual case of EH involving sphenoid and temporal bone in a young woman. Preoperative embolisation is thought to be absolutely essential before removal. Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.
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8/24. Expression of glucocorticoid receptor and 11beta hydroxysteroid dehydrogenase in a case of pulmonary epithelioid haemangioendothelioma.

    This report describes a case of pulmonary epithelioid haemangioendothelioma in which the tumour cells expressed the glucocorticoid receptor and 11beta-hydroxysteroid dehydrogenase. The patient, a 15 year old girl, who had no other complaints or past illnesses, was found to have an abnormal shadow on a chest roentgenogram obtained at a school medical examination. Multiple nodular shadows in the bilateral lungs were also confirmed by computerised axial tomography scan. A diagnosis of pulmonary epithelioid haemangioendothelioma was made on the basis of lung biopsy specimens. The tumour cells were immunohistochemically positive for factor viii related antigen, CD31, and CD34, but not surfactant apoprotein A. In addition, almost all of the tumour cells showed simultaneous expression of the glucocorticoid receptor and 11beta hydroxysteroid dehydrogenase, suggesting that steroid treatment would be effective.
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9/24. Epithelioid haemangioendothelioma of the rib--a case report.

    Epithelioid haemangioendothelioma is a rare primary malignant tumour of the bone that accounts for less than 1% of all primary bone malignancies. The case discussed here is of a 35 years old male who presented with gradually increasing left infrascapular mass attached to 10th rib. X-ray showed an expansile lytic lesion in 10th rib. On FNA the diagnosis of fibrous dysplasia or fibrous-histiocytic lesions was suggested. The lesion was excised along with adjacent rib. Histopathological examination showed features of epithelioid haemangioendothelioma. immunohistochemistry revealed focal factor viii related antigen positivity.
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10/24. 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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