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)

A case of multifocal hemangioendothelioma of the liver, adrenal gland, and placenta is reported. The histological appearance of the tumor is consistent with an infantile hemangioendothelioma, type 2. Multifocal development is the most obvious explanation for the disease but the possibility that this represents malignant placental neoplasm with metastases requires consideration.
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PMID:Multifocal hemangioendothelioma of the fetus and placenta. 922 58

We present a new case of retiform hemangioendothelioma (RH), an entity first described by Calonje et al. in 1994. The tumor was intradermal and located on the toe of an 11-year-old boy. Histologically, in addition to the distinctive retiform pattern of proliferating vessels in RH there are intraluminal papillae with hyaline cores similar to those seen in malignant endovascular papillary angioendothelioma (Dabska's tumor), but usually they are infrequent, focal and poorly developed. In our case, these papillary structures were well formed and distributed in a diffuse way. They were most conspicuous in superficial areas where the blood vessels were dilated. In deep areas, where the pattern of neoplastic vessels was retiform, the papillae filled their lumina totally, resembling solid cords. Our case shares the clinical and morphologic features of both retiform hemangioendothelioma and Dabska's tumor, supporting a relationship between these two kinds of neoplasms. The benign behavior of this case, with no recurrence or metastases over a 4-year follow-up, corresponds to the low malignancy of this kind of vascular neoplasm.
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PMID:Retiform hemangioendothelioma. A new case in a child with diffuse endovascular papillary endothelial proliferation. 927 63

Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 x 5 cm and clotted blood was found in the medullary area. The atypical tumor cells had a sinusoidal and solid appearance, and showed immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term 'hemangiosarcoma/angiosarcoma', 'hemangioendothelioma/endothelioma' or 'vascular sarcoma' of the kidney were reviewed and compared to the present case.
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PMID:Primary renal angiosarcoma: a case report and review of the literature. 941 38

In this third and last part of our review of cutaneous vascular proliferations we include malignant vascular neoplasms and a group of heterogeneous cutaneous neoplasms characterized by a significant vascular component. We also review some disorders that, in our opinion, have been erroneously considered as vascular neoplasms. We review the epidemiologic, histogenetic, clinical, and histopathologic aspects of Kaposi's sarcoma in its four distinctive variants (classic, African-endemic, immunosuppressive drug-associated, and AIDS-associated Kaposi's sarcoma). There is still controversy about whether Kaposi's sarcoma represents a reactive vascular proliferation or a true neoplastic proliferation. In any event, most authors believe that Kaposi's sarcoma does not produce metastatic disease, but rather develops in multifocal fashion. However, Kaposi's sarcoma may cause death, especially in immunosuppressed patients. Epithelioid hemangioendothelioma, Dabska's tumor, and retiform hemangioendothelioma are examples of low-grade angiosarcoma. In contrast, cutaneous angiosarcomas, including the clinical variants of angiosarcoma of face and scalp in elderly patients, angiosarcoma associated with lymphedema, and radiation-induced angiosarcoma are highly aggressive neoplasms with poor prognosis and most patients die within a short period after presentation. A group of benign and relatively frequent cutaneous neoplasms, including multinucleate cell angiohistiocytoma, angiofibroma, angioleiomyoma, angiolipoma, cutaneous angiolipoleiomyoma, and cutaneous angiomyxoma are here covered because of their significant vascular component. Finally, we review briefly a series of cutaneous disorders that have been erroneously considered as vascular neoplasms. Kimura's disease is an inflammatory reactive condition of unknown origin, "benign" angioendotheliomatosis is a reactive intravascular proliferation of endothelial cells that occurs in the skin as a response to a variety of stimuli, "malignant" angioendotheliomatosis is an intravascular lymphoma, and acral pseudolymphomatous angiokeratoma of children (APACHE) is better interpreted as a pseudolymphoma.
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PMID:Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. 948 70

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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PMID:Subtotal liver calcification due to epithelioid hemangioendothelioma. 958 38

Concomitant resistance (CR), the phenomenon by which tumor-bearing hosts are able to inhibit secondary implants of the same tumor at distant sites of the body, has been previously observed by us and others in different murine tumor models. Here, we verified the generation of CR in nude mice by tumors induced by SC inoculation of Calu-6, a human lung carcinoma cell line. Histological analysis of secondary tumors subject to CR did not reveal macrophage infiltration nor cytotoxic signs. Although serum from tumor-bearing mice inhibited in vitro [3H]thymidine uptake by Calu-6 cells, no significant differences in [3H]thymidine labeling index of tumors implanted in the right flank of mice with and without a primary tumor in the left flank were detected. In our model, the presence of a primary tumor hindered remote tumor angiogenesis, as well as serum from tumor-bearing mice inhibited in vitro proliferation of an endothelial cell line derived from a murine hemangioendothelioma. Conversely, an enhancement of the apoptotic index was observed in secondary tumor implants carried out in tumor-bearing mice. The results reported herein show that human tumor cells are capable of inducing CR, and that this phenomenon would be a consequence of an impaired neovascularization as well as an increased programmed cell death at sites distant from the primary tumor.
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PMID:Antiangiogenesis and apoptosis as mediators of concomitant tumor resistance induced by Calu-6, a human lung carcinoma cell line, in nude mice. 961 53

The clinicopathologic, immunohistochemical, and ultrastructural features of soft tissue angiosarcomas are not well defined. Eighty cases of angiosarcoma that involved the deep subcutis, skeletal muscle, retroperitoneum, mesentery, and mediastinum are reported. The lesions occurred in 50 male and 30 female patients who were 5-97 years of age; the peak incidence was in the seventh decade of life. A variety of associated conditions were documented in 20 of these cases, including a history of other neoplasms (some irradiated), synthetic vessel grafts, heritable conditions, and prior trauma or surgery. The angiosarcomas occurred in the extremities (n = 43 cases), trunk (n = 28), and the head and neck (n = 9) regions, with the thigh and the retroperitoneum being the most common sites. They often were characterized as enlarging, painful masses of several weeks' duration and were occasionally associated with acute hemorrhage, anemia, or a coagulopathy. The tumors measured 1-15 cm in diameter (median 5 cm) and frequently were hemorrhagic and multinodular. There was a wide morphologic spectrum within and between cases, including areas similar to cavernous and capillary hemangioma, Dabska tumor, spindle cell and epithelioid hemangioendothelioma, various spindle cell sarcomas, or carcinoma. Histologically, epithelioid angiosarcoma was the most frequently observed pattern; 70% of cases had epithelioid cells that were arranged in nests, clusters, papillae, and gaping vascular channels. Hemorrhage tended to obscure the diagnosis in several cases and often was associated with papillary endothelial hyperplasia-like areas. All 42 cases studied immunohistochemically stained at least focally for Factor VIII-related antigen, and nearly all stained strongly for vimentin, which accentuated the endothelial cells and vessel lumen formation. CD34 antigen was detected in 74% of cases, BNH9 in 72%, and cytokeratins in 35%. Epithelial membrane antigen, S-100 protein, and HMB45 were not detected. Fifty-five percent of the tumors had intracytoplasmic aggregates of laminin. Immunostains for alpha-smooth muscle actin demonstrated a prominent pericytic component in several tumors (24%). Ki67 immunostains with MIB1 indicated high proliferative activity (> or =10%) in 72% of cases. p53 immunoreactivity (>20% nuclear staining) was observed in 20% of cases. Ultrastructural studies performed on poorly differentiated areas of 12 cases showed groups of cells, which were frequently epithelioid, surrounded by basal lamina, and closely associated with pericytes, along with intercellular and intracellular lumina with or without red blood cells. Whorls of abundant intermediate filaments, occasional tonofilamentlike structures, and pinocytotic vesicles also were noted. In contrast to the findings of others, Weibel-Palade bodies were not seen. Follow-up in 49 cases (61%) showed that 53% of patients were dead of disease at a median interval of 11 months, whereas 31% had no evidence of disease at a median interval of 46 months. The remaining patients were either alive with disease (14%) or alive but disease status was unknown (2%). There were local recurrences in 20% of cases and distant metastases in 49%, most frequently to the lungs, followed by the lymph nodes, soft tissues, bone, liver, and other sites. These results indicate that angiosarcoma of soft tissue is a high-grade sarcoma. Older patient age, tumor location in the retroperitoneum, and larger tumor size as well as detection of MIB1 in > or =10% of the tumor cell population were all associated with a poorer prognosis.
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PMID:Angiosarcoma of soft tissue: a study of 80 cases. 963 Jan 75

The epithelioid hemangioendothelioma of the lung is generally detected incidentally by a routine chest radiograph, usually in young asymptomatic woman. This tumor pursues a clinical course intermediate between that of hemangioma and angiosarcoma. Survival over 20 years have already been described in the literature. We report the case of a pulmonary epithelioid hemangioendothelioma diagnosed 16 years ago, with hepatic metastases and a radiologic follow-up that highlights the slow evolution of the pulmonary and hepatic lesions.
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PMID:[Clinical case of the month. Multiple pulmonary nodules. Epithelioid hemangioendothelioma]. 981 Feb 4

The term haemangioendothelioma has been used in the past for a number of vascular lesions, which vary not only by their morphological features, but more importantly, also by their biological behavior. In the recent WHO-classification of mesenchymal tumours haemangioendotheliomas have been defined as vascular tumours of "intermediate" or "borderline" malignancy, and spindle cell haemangioendothelioma (SHE), epithelioid haemangioendothelioma (EHE), and rare malignant endovascular papillary angioendothelioma (Dabska's tumour) were included in this category. To this list might be added the more recently delineated kaposiform (KHE), retiform (RHE), polymorphous (PHE), and composite haemangioendothelioma (CHE). Although very popular, the concept of "borderline" or "intermediate" malignancy encompasses a wide variety of clinical situations, prognosis, and biological behavior. Therefore uncritical use of the term haemangioendothelioma represents a potential source of confusion to patients and oncologists, and it should not be used without further clarification. SHE was originally described as low-grade angiosarcoma, however, the study of large series with expanded follow-up information clarified that these lesions are often multicentric in one anatomic region, whereas true recurrences are rather rare, and systemic metastases and tumour progression do not occur. Therefore redesignation of these lesions as spindle cell haemangioma has been proposed. EHE of skin and soft tissues represents a distinctive vascular neoplasm characterized by nests and cords of epithelioid endothelial tumour cells with characteristic cytoplasmic vacuoles, which are set in a myxohyaline matrix. The reported rates of systemic metastases (20-30%), and tumour related death of patients (13-17%) in EHE, and the occurrence of multicentric EHE argue against the classification of EHE as a low-grade or "borderline" malignant neoplasm; EHE should be better regarded as a clearly malignant vascular tumour (G2). Although it seems that KHE is associated with a high mortality rate, the deaths are almost always related to locally invasive effects or as result of bleeding and consumption coagulopathy. So far no metastasizing case of KHE has been reported, and it seems that the prognosis in KHE is mainly related to size, anatomical site and depth of the lesion. KHE should be classified as a locally aggressive, non-metastasizing vascular tumour. The remaining entities (RHE, Dabska's tumour, PHE, and CHE) are characterized by an infiltrative growth, a high rate of (often repeated) local recurrences, and a definitive risk of metastases. Therefore these lesions fulfil criteria for low-grade malignant vascular neoplasms.
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PMID:[Hemangioendotheliomas--evolution of a concept of a heterogeneous group of vascular neoplasms]. 1009 22

Hepatic transplantation has emerged as a potentially curative treatment of certain malignant hepatic neoplasms such as hepatocellular carcinoma, bile duct carcinoma, fibrolamellar hepatocellular carcinoma, metastases from neuroendocrine tumors, and epithelioid hemangioendothelioma. In the early years of hepatic transplantation, there was great enthusiasm to cure patients with unresectable hepatobiliary malignancy. This early enthusiasm was tempered by the unfavorable outcome of transplantation in advanced cases of malignancy and the organ-donor shortage. Presently, patients have to be selected with predictable likelihood for long-term survival. Pre-transplantation imaging is indispensable for detection, characterization, staging, and surgical road-mapping before the procedure. The present article focuses on the role of imaging modalities in these different aspects of preoperative assessment.
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PMID:Tumor diagnosis in the adult liver transplant candidate. 1036 78


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