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Query: UMLS:C0027627 (metastases)
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Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.
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PMID:Primary vascular tumors of lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and delineation of two new entities. 831 16

Epithelioid hemangioendothelioma is a very rare tumour of vascular origin, the most peculiar feature of which is that it is composed of endothelial cells closely resembling epithelial cells. Confirmation of its endothelial origin is achieved by positive staining for Factor VIII-related antigen or by the demonstration of Weibel-Palade bodies by electron microscopy. We report two cases of primary epithelioid hemangioendothelioma of the liver that occurred in our center in the last ten years. The first patient is a 21 year old woman who presented with a right upper quadrant pain and an echographic finding of multiple hepatic lesions suggestive of metastases. The second patient is a 32 year old woman operated on for a cerebral glioma, in whom an abdominal CT scan, performed in view of radiotherapy, revealed multiple hepatic lesions, originally interpreted as being metastatic. In both cases, diagnosis was made based on the histologic examination of biopsies have been adopted in each case: the first patient has undergone successful liver transplantation. In the second patient an attentive surveillance appeared to be the best option, due to the presence of a previous cerebral glioma.
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PMID:Epithelioid hemangioendothelioma of the liver: report of two cases. 174 18

Epithelioid hemangioendothelioma is an unusual type of endothelial-derived vascular tumor located mainly in the soft tissues of the limbs. The present report describes a 48-year-old woman with an epithelioid hemangioendothelioma located in the liver with very unusual metastases to the myocardium. While the disease itself was controlled by adriamycin the patient died as a consequence of portal hypertension due to the abundant fibrotic component distinctive of this type of tumor. An epithelioid appearance can sometimes lead to a mistaken diagnosis of metastatic carcinoma, as happened initially in our case. The endothelial origin of the tumor must be confirmed by electron microscope identification of Weibel-Palade bodies or by immunohistochemical staining of factor VIII-related antigen. To the authors knowledge, this is the first case of myocardial metastases from an epithelioid hemangioendothelioma of the liver to be reported in English in the medical literature.
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PMID:Epithelioid hemangioendothelioma of the liver with myocardial metastases. 273 68

Epithelioid hemangioendothelioma is a rare, ubiquitous, vascular tumor, difficult to diagnosis but sometimes identified after detection of metastases, and with a prognosis intermediary between angioma and angiosarcoma. The tumors have been described as developing in the large venous trunks and recently lesions have been reported in liver. Little documented data exist with respect to radiologic appearances. A case is reported with a histologically confirmed hepatic localization in association with a lesion in superior vena cava.
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PMID:[Epithelioid hemangioendothelioma of the liver and superior vena cava. Radiological results apropos of a case in an adult]. 356 32

Epithelioid hemangioendothelioma (EH) is a vascular neoplasm that occurs predominantly in soft tissue and is not infrequently misdiagnosed as an epithelial neoplasm or angiosarcoma. Only a few cases of hepatic EH have been described, and a relationship to oral contraceptive (OC) use in patients with the hepatic lesions has not generally been recognized. We present a series of five patients with malignant epithelioid hemangioendothelioma of the liver. Confirmation of the endothelial origin of these tumors was provided by positive immunoperoxidase staining for Factor-VIII-related antigen in the four cases studied by that technique, and by the demonstration of Weibel-Palade bodies in two tumors examined by electron microscopy. All five patients were young women (mean age 33 years) and all five gave a history of OC use of 4-7 years' duration. The clinical course varied from indolent but progressive to rapid death. One patient who underwent resection of the primary tumor has survived 3 years without evidence of disease, and one patient with metastatic disease who was treated with radiation and chemotherapy has survived for 8 years with disease. Three patients with extrahepatic spread have died of the tumor. Early diagnosis of this distinctive tumor might offer the hope of salvage by resection or liver transplantation.
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PMID:Malignant epithelioid hemangioendothelioma of the liver in young women. Relationship to oral contraceptive use. 390 92

Epithelioid hemangioendothelioma is a unique tumor of adult life which is characterized by an "epithelioid" or "histiocytoid" endothelial cell. Forty-one cases of this rare tumor have been recognized at the Armed Forces Institute of Pathology. They may occur in either superficial or deep soft tissue, and in 26 cases appeared to arise from a vessel, usually a medium-sized or large vein. They are composed of rounded or slightly spindled eosinophilic endothelial cells with rounded nuclei and prominent cytoplasmic vacuolization. The latter feature probably represents primitive lumen formation by a single cell. The cells grown in small nests or cords and only focally line well-formed vascular channels. The pattern of solid growth and the epithelioid appearance of the endothelium frequently leads to the mistaken diagnosis of metastatic carcinoma. The tumor can be distinguished from a carcinoma by the lack of pleomorphism and mitotic activity in most instances and by the presence of focal vascular channels. Ultrastructural study in four cases confirmed the endothelial nature of the tumor in demonstrating cells surrounded by basal lamina, dotted with surface pinocytotic vesicles, and occasionally containing Weibel-Palade bodies. Follow-up information in 31 cases indicated that 20 patients were alive and well following therapy; three developed local recurrences and six metastases. It is suggested the term epithelioid hemangioendothelioma be used to designate these biologically "borderline" neoplasms. The significance of the epithelioid endothelial cell is not entirely clear. Since it may be observed in both benign and malignant vascular lesions, its presence alone does not define a clinicopathologic entity.
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PMID:Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. 709 31

Epithelioid hemangioendothelioma is an uncommon intermediate-grade vascular tumor which most often presents in soft-tissue and is known to occasionally metastasize. A primary cardiac occurrence of this tumor was resected from the left atrium of a 71 year-old woman. Four months later, she underwent gluteal exploration and resection of three nodules from her right buttock. These nodules, which resembled the previously resected tumor but showed features of malignant transformation, were identified as metastatic epithelioid hemangioendotheliomas. To the best of our knowledge, only one case of primary cardiac epithelioid hemangioendothelioma has been previously reported in the literature. We believe ours is the second such case and the first to demonstrate distant metastases. This rare case of primary cardiac epithelioid hemangioendothelioma with distant metastases is illustrative of many of the known features and behaviors of the tumor. The case is reviewed and discussed alongside a review of recent topical literature.
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PMID:Epithelioid hemangioendothelioma of the heart with distant metastases. A case report and literature review. 830 Jul 22

In 1973, a 10 year old boy presented with numerous bilateral lung nodules, diagnosed as histiocytosis X by open lung biopsy. The patient was treated with prednisone until 1984. In 1993, he developed severe pain in the neck. A biopsy of the spine revealed the same tumour morphology as was seen in the lung in 1973. Immunohistological examination of the former and present biopsy led to the definitive diagnosis of epithelioid haemangioendothelioma of the lung with metastases to spine and liver. Epithelioid haemangioendothelioma of the lung is a rare soft tissue tumour of vascular origin, readily mistaken for carcinoma or, as in this case, histiocytosis. The tumour has an intermediate malignant potential. Although metastases of epithelioid haemangioendothelioma of the lung are well-known, metastatic spread to bones, as in our case, has not previously been mentioned in the literature.
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PMID:Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls. 857 93

Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round, to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10, more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is better regarded as a fully malignant, rather than borderline, vascular neoplasm, albeit the prognosis is better than in conventional angiosarcoma.
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PMID:Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. 913 Sep 82

Epithelioid hemangioendothelioma of the lung, therefore labeled as IntraVascular BronchioAlveolar Tumor (IVBAT), is a tumor of endothelial origin; its presentation with multiple pulmonary nodules radiographically suggests metastatic disease. We describe here an epithelioid hemangioendothelioma with an exclusively myxoid pattern. The tumor was composed of nodules extending in a polypoid fashion from the alveolar septa. The cells within the tumor were epithelial-like and contained cytoplasmic lacunae positive for anti-FVIII, CD31 and CD34 antibodies. According to usual criteria, it was considered as a tumor of low grade malignancy, without vascular or bronchiolar extension or cytonuclear atypia and mitosis; the prognosis, difficult to determine, depends on locoregional tumor progression.
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PMID:[Pulmonary epithelioid hemangioendothelioma (or IVBAT). Report of a case of exclusively myxoid form]. 922 Oct 2


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