Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Presentation is made of 27 cases of liver neoplasms in infancy and childhood that were admitted to the Pediatric Hospital of the National Medical Center of the IMSS, through a period of 15 years. Sixteen cases were diagnosed as hepatoblastoma, 2 as hepatocellular carcinoma, 4 as mesenchymal hamartoma, 2 as hemangioendothelioma of infantile type, 2 were embryonal rhabdomyosarcoma of the intrahepatic bile-ducts and one case of multiple bile-ducts hamartoma. The hepatoblastoma and the mesenchymal hamartoma are neoplasms that appear more frequently before the age of 2 years; the hepatocellular carcinoma, after 5 years of age and the infantile hemangioendothelioma generally appears before 6 months old. In all, benign and malignant cases, the clinical manifestations were non specific and did not help to establish the diagnoses. The mesenchymal hamartomas were characterized by the fast growth that led to think in neoplasms of malignant nature. The selective treatment is lobectomy with a better prognosis if the neoplasm is located in the left lobe. The right lobe was the most frequently affected by the benign tumors, as well as by the malignant, and the lung was the organ wherein metastases more often were found.
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PMID:[Liver neoplasms in children]. 627 66

Intravascular bronchiolo-alveolar tumor (IVBAT) is a rare and highly distinctive pulmonary tumor of disputed cellular nature. Both epithelial and endothelial differentiation of this neoplasm have been suggested. We have studied multiple nodules of IVBATs from three patients by light and electron microscopy and by immunohistochemical methods for Factor VIII-related antigen (FVIII RAG). Our light and ultrastructural studies are in essential agreement with the previous suggestion of the endothelial nature of the neoplasm and our demonstration of the presence of FVIII RAG in many of the tumor cells offers new evidence strongly supportive of their endothelial differentiation. We believe that IVBAT and a group of extrapulmonary tumors described as epithelioid hemangioendothelioma and endovascular papillary angioendothelioma are similar biologically indolent neoplasms of epithelioid and dendritic endothelial cells characterized by stromal sclerosis, intravascular spread, a low incidence of metastases and slow clinical evolution. Thus, we regard IVBAT as a low-grade sclerosing angiosarcoma of the lung.
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PMID:Intravascular bronchiolo-alveolar tumor (IVBAT): A low-grade sclerosing epithelioid angiosarcoma of lung. 628 46

A case of malignant epithelioid hemangioendothelioma of the liver mimicking veno-occlusive disease is reported. The histological, ultrastructural, and immunohistochemical features of the present case indicate striking similarities to epithelioid hemangioendothelioma (EHE) of the soft parts described by Weiss and Enzinger. Tumour metastasis to the lung gave a picture closely resembling intravascular bronchiolo-alveolar tumour (IVBAT) of the lung. EHE of the liver is considered to be a unique type of hepatic endothelial neoplasm behaving as a low grade malignant tumour with a veno-occlusive process which has rarely been described, and had previously been classified as other diseases or neoplasms.
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PMID:Malignant epithelioid hemangioendothelioma of the liver, spreading through the hepatic veins. 643 65

We describe the clinical and pathologic features of an hepatic angiosarcoma in a 5-year-old child. The neoplasm manifested as a multicentric vascular tumor and was initially treated by lobectomy. Histopathologically, the lesion showed the features of infantile hemangioendothelioma, in which foci of cytologic atypia ("type 2") were present. A recurrence with rapid growth appeared that suggested a malignant tumor, but no metastases developed. A literature review confirmed the rarity of angiosarcomas in children and underscored the difficulties of formulating a prognostic estimate about infantile hemangioendotheliomas based on histology alone. Mitotic activity and cytologic atypism are regular features of angiosarcoma in children and cannot be ignored. This report also contrasts certain features of the biology of malignant vascular tumors of the liver in childhood with the counterpart in adults.
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PMID:Hepatic angiosarcoma in childhood. A case report and review of the literature. 654 21

Arterioportal shunting is observed angiographically in a wide variety of pathologic conditions. The route of flow has classically been considered to be via the hepatic sinusoids (transsinusoidal). This route occurs in cases of cirrhosis or the Budd-Chiari syndrome, and results in retrograde hepatofugal flow in portal branches. More recently, a transvasal route has been recognized angiographically, in which portal flow often remains hepatopetal. The transvasal route occurs in cases of hepatocellular carcinoma, metastases, shock, hepatic arterial obstruction, and many other conditions. Histologic confirmation of this route has been sought for many years, with other partial success. Nevertheless, angiographic evidence, as presented here, is sufficiently compelling to justify description of this pathway and its significance. Arterioportal flow may also occur via a post-traumatic fistula (disruption of adjacent portions of hepatic artery and portal vein), and via benign tumor vessels in hemangioma or hemangioendothelioma.
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PMID:Arterioportal communications: observations and hypotheses concerning transsinusoidal and transvasal types. 706 71

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

Renal hemangioendothelioma (HE) is an extremely rare malignant tumor. The sixth case is presented and all six analysed. The tumor affects middle-aged persons of either sex with past history of chronic renal disease and very recent onset of renal pain and hematuria. Anemia is common and a renal mass or tenderness is present. Pyelography shows poor function hydronephrosis, filling defect or collecting system distortion. A radical nephrectomy and radiotherapy is indicated and histology establishes the diagnosis. Renal HE appears to take a hurricane course and local recurrence and widespread vascular metastases occur in a matter of days or weeks after traditional cancer therapy. Industrial carcinogenesis is likely while chronic renal disease may predispose.
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PMID:Renal hemangioendothelioma. 716 Oct 1

Eighteen cases of hemangioendothelioma (angiosarcoma) of the bone were found in a review of the files of the Department of Pathology of the Institut Curie. The ages of the patients, 11 males and seven females, ranged from 12--69 years (median 32 years). Pain was the usual symptom (11/16). Fifty percent (9/18) of the tumors occurred in the long tubular bones. Radiographic findings showed osteolytic, often multifocal, lesions without evidence of reactive bone formation. Grossly, the tumor masses were always found to be confined to the cavity of the bone. In all cases microscopic findings fulfilled the diagnostic criteria proposed by Stout. Solid and cellular small structures closely resembling embryonal vessels were observed in several instances, and were also helpful in identifying the endothelial vascular nature of the tumors. The histologic grade was difficult to determine in the majority of cases because there were several areas with different grades of differentiation. Of 15 patients with follow-up data, seven were alive and well with no evidence of disease 2--14 years after diagnosis (median nine years). Another patient was alive with metastatic disease after five years. The survival of the seven patients who died ranged from 7--53 months (median 21 months). This study confirms that hemangioendothelioma of the bone is a distinct pathologic entity. In contrast, it appears very difficult to determine the common clinical behavior of these tumors because they can show an extremely variable malignant potential.
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PMID:Hemangioendothelioma (angiosarcoma) of bone: a distinct pathologic entity with an unpredictable course? 719 34

Hemangiopericytoma (HP) is extremely rare in our country. During the last 30 years only two cases were found in the material of our institute. The first case was a tumor of the neck. In spite of little histological evidence of malignancy, local recurrence together with bone metastases occurred four years after removal of the primary tumor. The second case might be a transition of a HP into a hemangioendothelioma. The primary tumor at the lower arm showed the histological pattern of a HP but contained some capillaries with suspicious endothelial proliferations. A nodule in the axilla revealed the pattern of hemangioendothelioma with some areas which resembled a HP. The visceral metastases contained tumor tissue with the typical appearance of hemangioendothelioma. Evaluation of dignity of hemangiopericytomas is extremely difficult. Differentiation of malignant cases of HP from other richly vascularized soft tissue sarcomas and especially from stromal sarcoma of uterus may present problems.
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PMID:[Hemangiopericytoma (author's transl)]. 726 28

Twenty-nine cases of hemangioendothelioma (H.E.) of the bone have been studied. In addition to the clinical and radiologic features of the tumor, attention was principally paid to the relationships between a tentative histologic grading of malignancy and the clinical course and final outcome of the cases. Three histologic grades are identified: Grade I H.E., Grade II H.E., and Grade III H.E. (or hemangiosarcoma). Grades I and II H.E. are frequently multicentric in the same lower limb. Grade I H.E. has a constantly good prognosis. It may remain stationary for several years even without treatment and it may be cured even by curettage or radiation. Grade II H.E. often has a good prognosis. In one of our cases the initial biopsy was interpreted as being a low grade tumor but the subsequent histology of the local recurrence indicated a Grade III malignancy and the patient died with metastases. Another case was graded II and the patient died with metastases. There are three explanations for this discrepancy: (1) some tumors are malignant in spite of a seemingly low grade histology; (2) low grade and fully malignant areas are present in the same tumor; (3) malignancy may progress in some low grade tumors in the course of time. On the basis of our experience we lean towards the last two possibilities, and therefore recommend histological study of large and multiple sections. Grade III H.E. has a very bad prognosis. The study of H.E. of the bone presents several problems. More information from a larger series of cases is needed to define the value of the histological grading in determining a prognosis, therefore indicating treatment.
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PMID:Hemangioendothelioma of bone: a study of 29 cases. 739 43


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