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)

We present 25 cases of a primary pulmonary sarcoma bearing histological, immunohistochemical, and ultrastructural features indistinguishable from those of monophasic synovial sarcoma of soft tissue. The patients were 11 men and 14 women between the ages of 16 and 77 years. Clinically, the most common symptoms were chest pain, cough, shortness of breath, and hemoptysis. The lesions involved all lung segments. Grossly, they varied in size from 0.6 to 20 cm and were described as soft to rubbery tumors with areas of necrosis and hemorrhage, some with cystic changes. Two lesions involved the bronchial wall and in one case the tumor was described as encircling the bronchial tree. Histologically, all of the lesions were characterized by an atypical spindle cell proliferation with a solid growth pattern. Areas of myxoid, neural, hemangiopericytic, and epithelial-like growth pattern were observed. Mitoses, necrosis, and hemorrhage were seen in all lesions in varying proportions. Immunohistochemical studies for epithelial membrane antigen (EMA) and keratin showed strong focal positivity in 25 of 25 and 23 of 25 lesions, respectively. Immunohistochemical study for vimentin showed diffuse strong positivity in all lesions. Other immunostains, including desmin, smooth muscle actin, and S-100 protein, were negative. Electron microscopy in three cases showed spindle cells with elongated nuclei containing abundant cytoplasmic rough endoplasmic reticulum and well developed desmosome type intercellular junctions. Follow-up information ranging from 2 to 20 years was obtained in 18 patients. Six patients died of their tumors, whereas four patients died of unrelated causes without evidence of recurrence or metastases. Eight patients were alive with disease (recurrence and/or metastases) from 1 to 7 years after diagnosis. Four patients were alive and well without evidence of recurrence or metastases from 2 to 20 years (mean follow-up, 12.5 years). The present group of lesions appears to constitute a distinctive and as yet previously undescribed primary sarcoma of the lung, which probably represents the visceral counterpart of monophasic synovial sarcoma of soft tissue in a pulmonary location. Because of their distinctive biology these lesions should be distinguished from a variety of primary and metastatic malignancies of the lung.
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PMID:Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. 775 Sep 31

The sialic acid nucleoside conjugate KI-8110 has been shown to inhibit the formation of hepatic metastases from human colorectal cancer cell lines in a nude mouse intrasplenic injection model. The compound does not inhibit sialyltransferases from either human colorectal tumor cells or human liver. Transport of CMP-sialic acid into endoplasmic reticulum and Golgi vesicles is inhibited and can account for the reduction in surface sialic acid found on treated cell lines. Only a 50% inhibition of CMP-sialic acid transport could be achieved suggesting the presence of more than one transport protein with differing specificities.
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PMID:Inhibition of CMP-sialic acid transport in human liver and colorectal cancer cell lines by a sialic acid nucleoside conjugate (KI-8110). 842 99

The fetoacinar pancreatic (FAP) protein is a specific component of the human exocrine pancreas associated with the differentiation and proliferation of acinar cells. FAP expression is enhanced in cases of pancreatic exocrine cancer and it is found in relatively high concentrations in pathological pancreatic juices. However, tumor cell lines do not secrete FAP into the culture medium. In this paper we analyze the intracellular localization of FAP in cell lines and compare some biological properties of the tumoral FAP with the normal adult and fetal forms. Immunocytological experiments performed using Mab J28 which characterizes FAP, gave a staining pattern suggestive of FAP localization in the ER. Subcellular fractionation corroborated this localization and established that FAP is tightly associated with the microsomal membranes. The absence of reactivity of the tumoral FAP with wheat germ agglutinin lectin and its strong reactivity with concanavalin A is consistent with the idea that FAP in tumor cells does not reach the Golgi apparatus and it is consequently retained in the endoplasmic reticulum (ER). FAP contained in hepatic metastasis derived from pancreatic adenocarcinoma appeared to be similar, if not identical, to that expressed by cell lines. This supports the hypothesis that FAP retention in the ER of malignant cells is a physiological phenomenon and not the result of a modification of cell lines due to the culture conditions. FAP expressed by cancer cell lines and metastases appeared by sodium dodecyl sulfate polyacrylamide gel electrophoresis a homogeneous protein with a M(r) of 120,000. Instead, the secreted mature protein consists of a main component of M(r) 110,000 and shows pronounced polymorphism (dispersion from M(r) 110,000 to 80,000). Increased size of the ER-retained protein is likely due to elongation of the peptide chain. Defective processing in the ER as a result of amino acid mutation could therefore explain the behavior of this protein in tumors.
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PMID:Retention of the fetoacinar pancreatic (FAP) protein to the endoplasmic reticulum of tumor cells. 846 90

Six cases of chordomas presenting as primary posterior mediastinal tumors are described. Three patients were female, and three were male between the ages of 8 and 65 years (mean, 40.6 years). In all cases, the tumors presented radiographically as relatively well-circumscribed, encapsulated soft tissue masses that did not seem to be related to the thoracic or dorsal spine. Only in one case, focal infiltration of bone at the level of T6-T7 was observed at the time of surgery. Histologically, the lesions showed a spectrum of features that ranged from sheets and cords of large cells with abundant vacuolated cytoplasm to small, stellate cells embedded within an abundant mucoid matrix. In one case, the cell population showed more pronounced nuclear atypia with loss of cytoplasmic vacuolization, frequent mitotic figures, necrosis, and solid areas characterized by a perivascular distribution of atypical spindle cells set against a myxoid stroma. Another case showed features of chondroid chordoma, with an immature chondroid-appearing matrix surrounding the atypical tumor cells. Immunohistochemical studies in all cases showed positive staining of the tumor cells with CAM 5.2 and broad-spectrum keratin, epithelial membrane antigen (EMA) and vimentin, and, to a lesser extent, with S-100 protein. Stains for muscle actin, carcinoembryonic antigen (CEA), and desmin were negative. Ultrastructural examination in two cases showed a spectrum of features that varied from large cells with abundant cytoplasm containing scattered ribosomes, glycogen granules, Golgi apparatti, abundant intermediate filaments, and small lumen formation with immature microvilli to smaller cells with elongated cytoplasmic processes, fewer intermediate filaments, rare desmosome type intercellular junctions, and complexes of mitochondria/rough endoplasmic reticulum. On clinical follow-up, two patients died with metastases to the lungs, chest wall, and liver from 1 to 3 years after diagnosis, and two patients are alive and well without evidence of disease after 3 and 16 years. Chordoma should be entertained in the differential diagnosis of posterior mediastinal tumors. Application of immunohistochemical stains or electron microscopy will be of aid in separating them from other conditions that may histologically closely resemble these lesions.
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PMID:Chordomas of the mediastinum: clinicopathologic, immunohistochemical, and ultrastructural study of six cases presenting as posterior mediastinal masses. 852 9

A case of clear cell dermatofibroma is presented. Clinically, a 41-year-old woman exhibited a hard brown nodule on her instep that was assumed to be a dermatofibroma. Histologically, more than 90% of the lesion was composed of clear cells. Epidermal hyperplasia and a storiform arrangement of spindle cells and sclerotic collagen in some foci at the periphery of the lesion indicated the fibrohistiocytic origin. Moreover, prominent vascularity and some bizarre giant cells in the lower part of the lesion were reminiscent of multinucleate cell angiohistiocytoma. Of a broad panel of antibodies, the lesion was positive only for Factor XIIIa (and vimentin). Ultrastructurally, clear-cell changes corresponded to a mostly translucent cytoplasm, focally with some endoplasmic reticulum and prominent lysosomal structures. A review of 1,496 dermatofibromas seen during the last 15 years at our institute revealed 12 cases (1%) with similar clear-cell changes in a minor part of the infiltrate (less than 10%). The differential diagnosis includes metastases of renal-cell carcinoma, which exhibit more atypia and mitoses and are positive for epithelial cell markers; clear-cell sarcoma, a lesion of tendons or aponeurosis with some moderate cytoplasmic melanin deposition and immunoreactivity with HMB-45; and various non-X histiocytic disorders, such as the predominantly vacuolated type of juvenile (or adult) xanthogranulomas or papular xanthoma, with a mixed infiltrate of various types of mononuclear and multinucleate histiocytes positive with a variety of macrophage markers.
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PMID:Clear cell dermatofibroma. Case report of an unusual fibrohistiocytic lesion. 904 95

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

Clinical examinations including X-ray analysis, gross pathological, histopathological and ultrastructural investigations were performed on a case of ovarian granulosa cell tumour in a 5-year-old German boxer bitch. Radiographic examination of the abdomen revealed a round, sharply demarcated formation about 4-5 cm in size. The tumour of the left ovary was of a malignant granulosa cell type, with numerous metastases 5-20 mm in size, present in the myocardium (left ventricle), lungs (in all lobes) and prescapular lymph node. Ultrastructural characterization of the tumour demonstrated closely packed cells, with rough endoplasmic reticulum, indicating markedly enhanced protein synthesis probably associated with rapid cell proliferation.
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PMID:A case of metastasizing ovarian granulosa cell tumour in the myocardium of a bitch. 890 42

Glycoproteins of the carcinoembryonic antigen family (CEA) and epithelial membrane antigen (EMA) are established markers for glandular and mucosal tissues. However, their precise ultrastructural distribution in sweat glands has not been determined as yet. Therefore, normal human skin, 19 cases of various sweat gland neoplasms, Paget's disease, and cutaneous metastases of visceral carcinomas were stained with well-defined antibodies using a postembedding immunogold technique. In some cases, a new method of re-embedding paraffin material for immunoelectron microscopy was applied. In normal sweat glands, immunoreactivity of the endoplasmic reticulum and vesicles indicated biosynthesis and processing of CEA and EMA. Along the luminal surfaces both CEA and EMA represented an integral part of microvilli. However, a differential expression of CEA and EMA was demonstrated in apocrine epithelia, mucous cells of eccrine glands, and sweat ducts. In fetal glands, CEA was associated with formation of secretory and ductal lumina. The overall cellular distribution of CEA and EMA was highly preserved in benign sweat gland neoplasms whereas malignant neoplasms were characterized by loss of protein targeting and cellular polarity. In conclusion, these immunoelectron microscopical findings suggest a role of CEA and EMA for cell differentiation and secretory mechanisms of sweat gland epithelia.
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PMID:Ultrastructural localization of carcinoembryonic antigen (CEA) glycoproteins and epithelial membrane antigen (EMA) in normal and neoplastic sweat glands. 900 82

This series presents six cases of a rare variant of dermatofibroma, characterized by marked clear cell change. All lesions occurred on the lower extremities of middle-aged adults (four women, two men), mostly with the clinical diagnosis of fibrohistiocytic lesion. Histological examination revealed well circumscribed, faintly stained dermal to subcutaneous lesions which were due to the overwhelming presence of clear cells (> 90%), some with prominent PAS-positive cytoplasmic granulation. Overlying epidermal hyperplasia as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lympho-histiocytic infiltrate at the periphery of the lesion indicated the fibrohistiocytic origin. Individual histopathological peculiarities included: bizarre giant cells in two cases, perifollicular arrangement and haemangiopericytoma-like features with iron deposition in one case each. Immunohistochemically three of four lesions showed moderate reactivity for factor XIIIa and two of four with an anti-metallothionen marker E9, but were otherwise negative with a broad panel of markers. Electronmicroscopy in two cases revealed large pools of glycogen beside focal, prominent endoplasmic reticulum and lysosomes in some granular cells, but only optically translucent cells in cases of clear cells. Recognition of clear cell dermatofibroma is important as the differential diagnosis includes some entities with more serious outcome/considerations such as metastases of renal cell carcinoma, xanthogranulomatous reactions, balloon cell naevus/melanoma and clear cell sarcoma.
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PMID:Clear cell dermatofibroma. 902 59

A case of myofibrosarcoma of the breast is described. A 55-year-old woman presented with a small mammary nodule. A large recurrent lesions appeared a month later, and the patient died 11 months following initial presentation from diffuse pleuropulmonary metastases. Histologically, the primary tumor consisted mainly of spindled cells, arranged in fascicles and surrounded by varying quantities of dense hyaline collagen. The recurrent lesion had a more pleomorphic organization. In both lesions, there was positive immunostaining for vimentin, smooth-muscle actin, and fibronectin, and negative results for desmin, laminin, and type IV collagen. Electron microscopy revealed abundant rough endoplasmic reticulum, myofilaments with focal densities, and the fibronexus junctions and fibronectin fibrils characteristic of myofibroblasts. Given these cellular features and behavior, the tumor was interpreted as a malignant neoplasm showing myofibroblastic differentiation, i.e., a myofibrosarcoma. This case enlarges the group of myofibrosarcoma of breast, also with the demonstration of fibronexus and fibronectin fibrils. The paper emphasizes the criteria required for myofibroblastic differentiation and reviews lesions of the breast reported in the literature as myofibroblastic.
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PMID:Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. 913 Sep 98


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