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)

Six cases of spindle cell hemangioendothelioma (SCH) are presented with immunohistochemical and flow cytometric analyses. One case was associated with Maffucci's syndrome. All lesions were solitary or multifocal in the extremities, and prepresentation duration ranged from years to decades. One case recurred but none had metastases. Histologically, in four of the six cases the main lesions appeared to arise within vessels, predominantly muscular vessels. All lesions consisted of cavernous hemangioma-like areas and solid cellular areas resembling Kaposi's sarcoma. Cellular atypia was minimal. At the periphery of the lesions, a cluster of large thick or thin walled, and probably malformed, vessels were observed. Immunohistochemically, factor-VIII related antigen, CD34, vimentin, and lectin binding Ulex europaeus agglutinin 1 stained endothelial cells lining vascular channels, and vacuolated, or epithelioid cells. Spindle cells in the solid areas were negative for these endothelial markers except for vimentin, but showed divergent positive immunoreactions of HHF35, alpha-smooth muscle actin, desmin, and collagen type IV. Five cases were diploid and one was aneuploid. There was no significant correlation among DNA ploidy, S-phase fraction, and local recurrence in SCH although the number of cases examined was small. These results suggest SCH may be a benign lesion, probably a reactive process, rather than a low-grade angiosarcoma.
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PMID:Spindle cell hemangioendothelioma: an immunohistochemical and flow cytometric study of six cases. 749 4

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.
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PMID:Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations. 750 96

The existence of angiosarcoma of the thyroid gland and its relation to angiomatoid carcinoma have been debated. The authors reviewed eight angiomatoid thyroid neoplasms. Controls consisted of six sarcomatoid thyroid carcinomas without angiomatoid features and an angiosarcoma metastatic to the thyroid gland. All eight angiomatoid neoplasms consisted of epithelioid cells with prominent eosinophilic cytoplasm lining vascularlike spaces. All eight expressed vimentin. Four tumors were predominantly angiosarcomalike neoplasms, based on staining for factor VIII-related antigen (three of four), CD31 (four of four), CD34 (one of four), and Ulex europaeus I lectin (four of four); they lacked epithelial markers other than cytokeratin (two of four). Four tumors were designated as angiomatoid carcinomas, based on staining for multiple epithelial markers: cytokeratin (four of four), epithelial membrane antigen (three of four), thyroglobulin (three of four). Three angiomatoid carcinomas also expressed or labeled with one or more vascular markers: CD34 (one of four), CD31 (two of four), Ulex europaeus I lectin (one of four), factor VIII-related antigen (one of four). The metastatic angiosarcoma to the thyroid gland labeled for factor VIII-related antigen, vimentin, CD31, and with Ulex europaeus I lectin. It did not express CD34. The six sarcomatoid carcinomas without angiomatoid features stained for cytokeratin (four of six), epithelial membrane antigen (one of six), and vimentin (six of six). None labeled for thyroglobulin, factor VIII-related antigen, CD31, CD34, or with Ulex europaeus I lectin. Angiomatoid carcinomas of the thyroid gland exhibit both epithelial and endothelial features. "Angiosarcoma" may represent the extreme in this spectrum of endothelial differentiation. All tumors behaved in a similar clinical fashion characterized by persistent local disease, widespread metastases and poor prognosis.
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PMID:Angiomatoid carcinoma and 'angiosarcoma' of the thyroid gland. A spectrum of endothelial differentiation. 808 56

Nine vulvar and three vaginal angiomyofibroblastomas from patients 23 to 71 years of age (mean, 46 yr) were analyzed. The tumors were well circumscribed and ranged from 0.9 to 11 cm (average, 4.7 cm) in maximal dimension. On microscopic examination, they had hypercellular and hypocellular areas. The neoplastic cells were spindle-shaped, plasmacytoid, or epithelioid; a variable number were binucleated or multinucleated cells. A focal storiform pattern was present in one tumor, and, in one tumor, the neoplastic cells formed a collar around a central area of dense collagen. There was no significant nuclear atypia, and there was less than one mitotic figure per 10 high-power fields. The tumors contained small- to medium-sized blood vessels, which were characteristically thin walled and, occasionally, ectatic and branching. The stroma was edematous, separated collagen fibers and contained a variable number of inflammatory cells, especially lymphocytes and mast cells. Three vulvar tumors contained a variable amount of fat. Ultrastructural study of three tumors showed intracytoplasmic, dilated, rough endoplasmic reticulum, moderate numbers of pinocytotic vesicles, and numerous filaments without dense bodies; rare intercellular rudimentary junctions were identified. Eleven of 11 tumors were immunoreactive for vimentin, 11 of 12 for desmin, three of 11 for muscle actin, one of 12 for smooth muscle actin, and four of 12 for CD34. There was no staining for factor XIIIa, keratin, S100 protein, Leu-7, glial fibrillary acidic protein, or CD68. Follow-up revealed no recurrences or metastases. Angiomyofibroblastoma is a distinctive benign tumor that arises most commonly in the vulva and vagina and has a diverse histologic and immunohistochemical profile.
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PMID:Angiomyofibroblastoma of the vulva and vagina. 868 29

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor characterized by local invasion and recurrence. Fewer than 50 cases have been reported in the pediatric population. We reviewed our experience in the treatment of children with DFSP to define clinical and pathological characteristics. Seven pediatric patients were included in the study (mean age, 11.7 yr). Clinically, the tumors were described as firm nodules fixed to the skin but mobile over the deep fascia, with slow, progressive growth. Diagnosis was made by excisional biopsy in 6 patients and punch biopsy in 1 patient. Six of 7 patients had positive margins after the diagnostic procedure. Pathologically, diagnosis was based on histology, with confirmation by CD34 staining. Definitive surgical therapy consisted of wide local excision (1-3 cm margins) in 5 patients and Moh's micrographic resection in 2 patients. There have been no local recurrences or distant metastases, with a mean follow-up of 15.1 months. Pathological and clinical diagnostic criteria for the pediatric population are reviewed, and treatment options are discussed.
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PMID:Clinical and pathological features of pediatric dermatofibrosarcoma protuberans. 874 47

Six gastric schwannomas found among 150 mesenchymal tumours of the gastrointestinal tract were analysed clinically, histologically and immunohistochemically. These tumours occurred in the wall of stomach in middle-aged patients, five women and one man, and measured 2-9 cm in diameter. Follow-up from 3 to 24 years showed no recurrences or metastases. Histologically, all tumours were composed of spindle cells with vague nuclear palisading and variably myxoid stroma. One case showed a multinodular pattern of growth, reminiscent of plexiform neurofibroma. In all cases, there was a peripheral cuff-like B-lymphocyte infiltration with germinal centers in two cases. None of the tumours showed mitotic activity. Immunohistochemically, the tumour cells were positive for S-100 protein and focally for GFAP and CD57 (Leu 7). They were negative for desmin and actin, unlike true leiomyomas, and negative for CD34, unlike most gastrointestinal stromal tumours that were examined for comparison. Electronmicroscopy of three cases showed complex cell processes surrounded by prominent basement membranes, while myofilaments were not present. These cases show that schwannomas can be identified as rare, benign gastrointestinal tumours which probably arise from the gastrointestinal autonomic nervous system.
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PMID:Gastric schwannoma--a clinicopathological analysis of six cases. 884 66

A 49-year-old woman who suffered from caecal cancer in 1988 underwent chemohyperthermic peritoneal perfusion for peritoneal and ovarian metastases in 1990, and high dose chemotherapy (HDC) with peripheral blood stem cell transplantation (PBSCT) for lung metastases in 1995. Heated saline containing anticancer drugs such as cisplatin, mitomycin C, etoposide (ETP), and pirarubicin, was intraperitoneally perfused at 43 degrees C for 60 minutes. The CD34 positive cells were mobilized by intravenous 500 micrograms G-CSF administration on five consecutive days. These cells were transplanted three days after the last day in the course of HDC, which included intravenous administration of 475 mg carboplatin, 2,020 mg cyclophosphamide, and 540 mg etoposide. The patient has survived with no sign of the disease.
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PMID:[Chemohyperthermic peritoneal perfusion and high-dose chemotherapy followed by peripheral blood stem cell transplantation in advanced colon cancer--a case report]. 885 10

We report a case of an intrathoracic, extrapleural, infantile rhabdomyofibrosarcoma in a 4-year-old boy. Histologically, the primary lesion showed extensive hyalinization and stroma sclerosis and was composed of relatively uniform spindle-shaped, at least focally rather polygonal tumour cells with scattered intracytoplasmatic globoid inclusions. Although chemo- and radiotherapy was given postoperatively, local recurrences and metastases in the lung and thymus have developed; the patient died of tumour disease 3 years later. Recurrences and metastases showed features of tumour progression with higher cellularity and increased mitotic activity. Immunohistochemically, the tumour cells stained strongly positive for vimentin, desmin, and muscle-specific actin, and at least focally for MyoD1; the tumour did not stain for alpha-smooth muscle actin, neural and epithelial markers, or CD34 and CD31. The differential diagnosis of these aggressive tumours in the spectrum of spindle-cell lesions in early childhood is discussed.
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PMID:[Infantile rhabdomyofibrosarcoma. An aggressive tumor in the spectrum of spindle cell tumors in childhood]. 892 96

Unknown primary tumors (UPT) are characterized by early and widespread metastasis. There is a strong indication that angiogenesis measured as microvessel density (MVD) correlates with the incidence of metastases in several solid tumors. The objective of this study was to compare MVD in liver metastases of UPT with MVD in known primaries and in liver metastases of colon and breast tumors and to investigate the prognostic significance of MVD in UPT. The clinical data and the MVD in liver metastases of 39 consecutive patients with UPT adenocarcinomas were studied. For comparison, MVD in the primary tumor and in liver metastases from known primary adenocarcinomas of the colon (n = 24) and the breast (n = 6) were measured. Most of the pathological material was obtained by needle biopsy. MVD was determined on formalin-fixed, paraffin-embedded histological sections of liver metastases, using the CD34 and von Willebrand Factor (vWF) antibodies and immunocytochemistry. The association of MVD with age, gender, number of metastases and tumor differentiation was assessed in the UPT population. The prognostic value of clinical variables and of MVD on survival was estimated by univariate and multivariate regression analysis. There was no difference between the MVD in liver metastases of UPT and known primaries. The MVD counts in the primary tumors of colon and breast were, however, significantly higher than in the metastases. MVD counts correlated well between anti-CD34 and anti-vWF. Within the UPT population there was no association between MVD and age, gender, number of metastases and tumor differentiation. The MVD was the only prognostic factor for survival in univariate analysis. High MVD was correlated with short survival. In the multivariate analysis, the number of metastases, tumor differentiation, therapy and MVD were all prognostic indicators for survival.
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PMID:Microvessel density in unknown primary tumors. 903 74

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


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