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 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

Three cases of primary pulmonary rhabdomyosarcoma in adults are presented. The patients were all men between the ages of 57 and 78 yr (mean 67.5). All patients presented with symptoms referable to their tumors, including cough, shortness of breath, pleuritic chest pain, and weight loss. In one patient, a history of tobacco and alcohol abuse was obtained. Anatomically, two tumors were located in the left upper lobe and one in the left lower lobe. Grossly, the tumors ranged in size from 6 to 11.5 cm and were tan-gray, firm masses with areas of necrosis and hemorrhage. Histologically, the tumors were characterized by a spindle cell proliferation admixed with areas showing a pleomorphic cell population with numerous rhabdomyoblasts and areas of hemorrhage and necrosis. Immunohistochemically, all three tumors showed strong positivity with desmin and myoglobin antibodies and negative staining with antibodies against keratin, epithelial membrane antigen, and S-100 protein. All patients had a fatal outcome. Two patients died a few days after admission with respiratory distress; the third one died 2 years after diagnosis with widely metastatic disease. Autopsy findings in all cases disclosed disseminated metastases to multiple abdominal and thoracic organs. Primary pulmonary rhabdomyosarcoma should be considered in the differential diagnosis of poorly differentiated pulmonary neoplasms in adults and should be distinguished from other primary and metastatic sarcomas.
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PMID:Primary pulmonary rhabdomyosarcomas: a clinicopathologic and immunohistochemical study of three cases. 853 1

A 25-year-old patient with a rapidly growing sarcomatoid carcinoma of the urinary bladder is reported. The diagnosis was made on the basis of extended atypical proliferations of spindle or pleomorphic cells in the area of pelvic floor and the radix of the penis. The tumor showed invasion of the blood and a high Ki-67 growth fraction up to 40%. Immunohistochemically, the reactions with antibodies against cytokeratin, EMA, and vimentin were positive, while negative results were obtained in reactions with antibodies against desmin, actin, PSA, S 100, human epithelial antigen (Ber-EP4), and cytokeratin 13. The differential diagnosis against myosarcomas, pseudosarcomatous lesions, and inflammatory pseudotumours is discussed. After radical surgery a pelvic recurrence and pulmonary metastases developed, which led to the patient's death 3 months later. This case shows that sarcomatoid carcinomas of the urinary bladder can be found even in young people.
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PMID:[Sarcomatoid carcinoma of the bladder in a 25-year-old man]. 857 May 63

Four round cell tumors, situated at the lip of dogs older than 4 years of age, which could not be further classified, were examined light and electron microscopically, immunocytochemically and in part functionally and cytochemically. Immunocytochemically they were positive for vimentin, but negative for cytokeratin, desmin, neurofilament, synaptophysin, S-100 protein, neuron specific enolase, lysozyme, IgG and a pan-T-cell marker. Cell lines were established from two malignant neoplasms. In vitro, neoplastic cells had morphological, functional and cytochemical properties of myelomonocytic cells. A tumor cell-specific polyclonal rabbit antiserum reacted immunocytochemically positive with primary and recurrent tumors and metastases of the original and the three other round cell tumors. Immunoblotting demonstrated a main band with approximately 65-75 kDa. All four tumors were present in aged dogs and metastasized. They most likely represent a distinct group of malignant tumors among the canine round cell tumors.
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PMID:Round cell sarcomas of possible myelomonocytic origin localized at the lip of aged dogs. 857 97

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

We present 22 (0.9%) cases of rhabdoid tumour of the kidney diagnosed amongst 2392 renal tumours in children. The patients ages ranged from 3 weeks to 94 months (median 7 months) and the female:male ration was 1.2:1. Clinically, they presented with an abdominal mass but four (18%) children also had hypercalcaemia and one (4.5%) developed a brain tumour (primitive neuroectodermal tumour). None of the children presented with stage I disease, five (23%) had stage II, ten (46%) stage III, and five (23%) stage IV disease. Two (9%) patients had bilateral tumours. Histologically, the vast majority (20/22) of the tumours exhibited a classical pattern but other histological patterns were also noted. Immunohistochemical studies performed in 12 cases showed vimentin positivity in all cases, CAM 5.2 in eight, epithelial membrane antigen in six, neuron specific enolase in four, S-100 protein in eight, and desmin in one case. In only 12 of the 22 tumours was there agreement between the reporting pathologist and the panel on a diagnosis of rhabdoid tumour of the kidney. Eight tumours originally diagnosed as rhabdoid tumour of the kidney were found to be other renal tumours and in another ten cases the initial diagnosis was changed by the panel to rhabdoid tumour. Metastases developed in 18 (82%) of the children--in eight they were present at the time of diagnosis and in 10 they developed from 2 weeks to 9 months after initial diagnosis. Metastases were found in the lung, abdomen, lymph nodes, liver, bone and brain. Of the 19 children with adequate follow-up, only two (10.5%) with stage II disease are alive, while 17 (89.5%) died 2 weeks to 20 months after the diagnosis.
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PMID:Rhabdoid tumour of the kidney: a clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file. 873 42

Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local invasiveness, and occurrence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/NO/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, alpha-sarcomeric actin, muscle-specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.
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PMID:Solid alveolar rhabdomyosarcoma of the hand in adolescence: a clinical, histologic, immunologic, and ultrastructural study. 874 83

Dedifferentiated chondrosarcomas are primary bone tumors characterized by the presence of both low-grade cartilaginous and high-grade sarcomatous components. The high-grade component usually shows histologic features of either malignant fibrous histiocytoma or fibrosarcoma. We are aware of only 10 published cases in which the high-grade component showed rhabdomyosarcomatous differentiation. To further clarify the clinical, radiographic, and pathologic features of this unusual variant, we report three additional cases of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation. The patients included two men and one woman; their mean age was 63 years. Tumors originated in the pelvis (ilium), scapula, and tibia. Two patients presented with radiographic findings typical of dedifferentiated chondrosarcoma, including a geographic, lytic lesion with areas of mineralization suggestive of cartilage in close association with a permeative component. The third patient presented with a primarily lytic, destructive lesion of the right iliac wing. Histologically, the tumors contained lobules of well-differentiated chondrosarcoma associated with a high-grade sarcoma with prominent rhabdomyoblasts. Immunohistochemical stains for actin and desmin were positive in all three tumors, and electron microscopy revealed evidence of skeletal muscle differentiation. All three patients died with metastatic disease, 1, 6, and 12 months postoperatively. This histologic variant of dedifferentiated chondrosarcoma is rare, but it shows radiographic and clinical features similar to "conventional" dedifferentiated chondrosarcoma, including a very poor prognosis.
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PMID:Dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation. 877 82

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

Superficial leiomyosarcomas are rare tumours. The lesions confined to the dermis, contrary to those involving the subcutis, have been reported to carry a favourable prognosis. A retrospective study of 41 consecutive cases of surgically treated intradermal and subcutaneous leiomyosarcomas was undertaken in order to determine the prognostic factors that may influence the survival of these patients. Seven tumours were predominantly intradermal and 34 involved the subcutaneous tissue. Fifty-four percent of the tumours were located in the lower extremities. All cases stained positively for smooth muscle antigen and 66% for desmin. The tumours were classified with regard to tumour grade I (low grade, 3%), II (intermediate, 12%), IIIA (high grade, 46%) and IIIB (high grade, 39%). In all patients, follow-up information was available. Mean follow-up time was 5 years. The patients with intradermal tumours were all alive without signs of recurrence, whereas 14 of those with leiomyosarcomas involving the subcutis have died with pulmonary metastases. Our study confirms that "pure" intradermal leiomyosarcomas independent of tumour grade behave in a benign fashion, probably due to small tumour size. Tumour size > or = 5 cm, deep localization with fascia involvement, and high malignancy grade (IIIB) were found to deteriorate survival based on a univariate analysis. However, in a multivariate analysis only tumour size was found to be an independent prognostic factor.
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PMID:Intradermal and subcutaneous leiomyosarcoma: a clinicopathological and immunohistochemical study of 41 cases. 891 54


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