Hemangioendotelioma epitelioide de pene / Epithelioid hemangioendothelioma of penis

Describimos un HE de pene en un varón de 64 años. Se trata de un tumor vascular de potencial maligno intermedio entre un hemangioma y un angiosarcoma de muy escasa aparición en esta localización. Su tratamiento comprende la extirpación amplia y la vigilancia una vez descartada la presencia de metástasis o multifocalidad. La histopatología es esencial en el diagnóstico

A case of EH of the penis in a sixtyfour-year-old patient is described. This is a rare –in this location- vascular tumor of borderline malignant potential between hemangioma and angiosarcoma. Local excision of the tumor with a cut-off of normal tissue and careful follow-up of the patient is the accepted procedure. Immunohistochemistry is essential for the diagnosis

Epithelioid hemangioendothelioma of soft tissue: report of a case with ultrastructural observations.

The ultrastructure of an epithelioid hemangioendothelioma arising in the soft tissues of the neck of an 18-year-old female is reported. Comparison with similar tumors in other sites from the authors' electron microscopy files indicates that diffuse nonspecific intermediate filaments, pinocytotic vesicles, intracytoplasmic lumens, and pericytic cells are frequent but variable features of this neoplasm.

Cytopathologic features of hepatic epithelioid hemangioendothelioma.

We present the cytological features of hepatic epithelioid hemangioendothelioma (EH), which is considered to be a vascular proliferation of intermediate malignant potential. The case report concerns a 52-yr-old previously healthy man discovered to have multiple hepatic masses upon evaluation for abnormal liver function tests. Fine-needle aspiration demonstrated a neoplasm composed of interanastomosing epithelioid cells that contained intracytoplasmic lumens. Histologic sections, immunohistochemistry, and ultrastructural evaluation were confirmatory. Although hepatic EH is a rare tumor, its characteristic cytological, histological, and ultrastructural features permit a straightforward diagnosis. It is important to distinguish this entity from adenocarcinoma and angiosarcoma because long-term disease-free survival is possible, especially in the setting of orthotopic liver transplantation.

Epithelioid hemangioendothelioma of the lung.

Light and electron microscopic features of an epithelioid hemangioendothelioma of the lung in a 43-year-old woman are reported. The tumor cells stained for factor VIII-related antigen. A mass excised from the thigh 10 years earlier showed identical appearances.

Epithelioid hemangioendothelioma of the lung: pleural effusion cytology, ultrastructure, and brief literature review.

Epithelioid hemangioendothelioma (EHE) of the lung is a low-grade malignant tumor of vascular origin initially described under the name intravascular bronchioloalveolar tumor (IVBAT). We present a case of a 44-yr-old Caucasian female with severe radiating back pain, shortness of breath, recurrent malignant pleural effusions, and a negative malignancy workup. Cytopathologic examination of the four pleural fluid specimens revealed large undifferentiated plasmacytoid malignant cells with abundant pink and finely granular cytoplasm, round nuclei, and prominent nucleoli. The differential diagnosis based on the cytologic findings included hepatocellular, adrenal, and renal carcinomas, melanoma, mesothelioma, and neuroendocrine tumors. Electron microscopy performed on a pleural fluid specimen and subsequent histologic examination of pleural and lung biopsies established the diagnosis of EHE.

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.

Epithelioid hemangioendothelioma of the anterior mediastinum. Clinicopathologic, immunohistochemical, and ultrastructural analysis of 12 cases.

Twelve cases are reported of primary epithelioid hemangioendothelioma of the anterior mediastinum. Patient ages ranged from 19 to 62 years (mean, 49.4); three were women and nine were men. Seven patients presented with symptoms due to compression of surrounding structures; the remainder were asymptomatic and the lesions were discovered on routine chest x-ray films. The tumors measured from 4.5 to 13.5 cm in greatest diameter; they were encapsulated and well-circumscribed in seven cases and locally infiltrative in five. Histologically, a spectrum of features was observed ranging from those classically described for low-grade epithelioid hemangioendothelioma at other locations--including cells with abundant eosinophilic cytoplasm showing prominent vacuolization and intracellular lumen formation, few mitotic figures, and myxoid changes in the stroma--to more pronounced cytologic atypia, increased mitotic activity, and areas of necrosis. Metaplastic bone formation and osteoclast-type giant cells were observed in five cases, and four tumors displayed focally a prominent intravascular papillary endothelial component. Immunohistochemical studies showed positive staining of the neoplastic cells with factor VIII-related antigen and vimentin and focal staining in most cases with Ulex europaeus lectin. Stains for broad-spectrum keratin, CAM-5.2, S-100 protein, CD34, actin, and desmin were negative. Electron microscopic examination in three cases showed features distinctive for epithelioid vascular endothelial cells, including abundant cytoplasmic intermediate filaments, basal lamina material, marked pinocytotic activity, and primitive intracytoplasmic lumen formation. All cases were treated by surgical excision. Follow-up information was available in nine patients; seven patients were alive and well without evidence of disease 2-21 years after diagnosis (mean follow-up, 8 years); one patient had a recurrence after 1 year and died due to complications of surgery, and another patient died after 1 year of undetermined causes. Despite their often ominous clinical, radiological, and pathological features, epithelioid hemangioendotheliomas arising in the anterior mediastinum appear to behave as low-grade malignant neoplasms that may be adequately controlled in most instances by surgery alone.

Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations.

This report is a case of epithelioid hemangioendothelioma presenting as multiple lytic lesions of the ilium with radiographic findings of diffuse, bilateral lung involvement and biopsy-proven scalp involvement. Histologically, the tumor within bone and skin exhibited cords and nests of plump, epithelioid-appearing cells exhibiting rudimentary vascular differentiation within a myxohyaline stroma. Aggressive histologic features were not present. Immunohistochemical reactivity for Factor VIII-related antigen, Q-bend 10 (CD34), and cytokeratin were demonstrated. Ultrastructural studies revealed abundant intermediate cytoplasmic filaments, pinocytotic vacuoles, and Weibel-Palade bodies. The concurrent bone, skin, and lung involvement, low-grade histologic type, and female sex of the patient aroused speculation about the role of hormones in the development and possible treatment of the tumor, but estrogen and progesterone receptors were not detected. Despite intense combination chemotherapy, the patient died of widely metastatic disease. This report demonstrates the aggressive potential of histologically low-grade epithelioid hemangioendothelioma and the need for a thorough evaluation for metastases.