Cases reported "Neoplasm Metastasis"

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1/66. bone marrow metastases in disseminated alveolar rhabdomyosarcoma: case report with ultrastructural study and review.

    A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis.
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ranking = 1
keywords = rhabdomyosarcoma, alveolar
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2/66. Molecular features of a human rhabdomyosarcoma cell line with spontaneous metastatic progression.

    A novel human cell line was established from a primary botryoid rhabdomyosarcoma. reverse transcription polymerase chain reaction investigations of this cell line, called RUCH-2, demonstrated expression of the regulatory factors PAX3, Myf3 and Myf5. After 3.5 months in culture, cells underwent a crisis after which Myf3 and Myf5 could no longer be detected, whereas PAX3 expression remained constant over the entire period. karyotype analysis revealed breakpoints in regions similar to previously described alterations in primary rhabdomyosarcoma tumour samples. Interestingly, cells progressed to a metastatic phenotype, as observed by enhanced invasiveness in vitro and tumour growth in nude mice in vivo. On the molecular level, microarray analysis before and after progression identified extensive changes in the composition of the extracellular matrix. As expected, down-regulation of tissue inhibitors of metalloproteinases and up-regulation of matrix metalloproteinases were observed. Extensive down-regulation of several death receptors of the tumour necrosis factor family suggests that these cells might have an altered response to appropriate apoptotic stimuli. The RUCH-2 cell line represents a cellular model to study multistep tumorigenesis in human rhabdomyosarcoma, allowing molecular comparison of tumorigenic versus metastatic cancer cells.
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ranking = 0.9962246833661
keywords = rhabdomyosarcoma
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3/66. Primary rhabdomyosarcoma of the cerebrum.

    A primary cerebral rhabdomyosarcoma is described in a 48-year-old Negro man. At autopsy two metastatic nodules were present in the liver. A review of the literature indicates that this is a rare and highly malignant tumor with no 5-year survival. The multipotentiality of the mesenchymal tissue in the leptomeninges is considered the histogenetic origin of intracranial rhabdomyosarcomas.
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ranking = 0.85390687145666
keywords = rhabdomyosarcoma
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4/66. hypokalemia, metabolic alkalosis, and hypertension: Cushing's syndrome in a patient with metastatic prostate adenocarcinoma.

    Ectopic adrenocorticotropic hormone (ACTH) and/or corticotropin-releasing hormone (CRH) are associated with a growing list of tumors. We report a 69-year-old white man with a history of high-grade prostate carcinoma and widely metastatic adenocarcinoma who presented with metabolic alkalosis, hypokalemia, and hypertension secondary to ectopic ACTH and CRH secretion. Laboratory values were consistent with hypokalemia and metabolic alkalosis. Markedly elevated serum cortisol (135 microg/dL), ACTH (1,387 pg/dL), CRH (69 pg/dL), and urine free cortisol (16,276 microg/24 h) levels were found. Chest computed tomographic (CT) scan showed small noncalcified parenchymal densities; however, bronchoscopy and bronchoalveolar lavage washings were unremarkable for a neoplastic process. Abdominal CT scan and magnetic resonance imaging showed multiple small liver lesions and multiple thoracic and lumbar intensities consistent with diffuse metastatic disease. Histological analysis of a biopsy specimen from the thoracic spine showed an undifferentiated adenocarcinoma consistent with a prostate primary tumor. The severe metabolic alkalosis secondary to glucocorticoid-induced excessive mineralocorticoid activity was treated with potassium supplements, spironolactone, and ketoconazole. In this case report, we describe an unusual tumor associated with ectopic ACTH and CRH production and the pharmacodynamic relationship of plasma cortisol levels and urinary cortisol excretion with ketoconazole treatment.
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ranking = 0.00075506332677953
keywords = alveolar
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5/66. Intrascrotal rhabdomyosarcoma.

    rhabdomyosarcoma is the most frequent spermatic cord tumor of infants, children, and young adults, but also occurs as a primary tumor in the testis, epididumis, and testicular tunics. In the last fifteen years, 7 patients with intrascrotal rhabdomyosarcoma were treated at our institution, and an additional 155 cases were found on review of the English literature. On the basis of these 162 cases, incidence and survival statistics were calculated with particular attention to employed forms of therapy. An over-all survival of greater than 73 per cent should be obtainable with proper utilization of surgery, radiation therapy, and combination chemotherapy.
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ranking = 0.71158905954722
keywords = rhabdomyosarcoma
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6/66. osteosarcoma of the jaw. The Chaim Sheba Medical Center experience.

    OBJECTIVE: The purpose of this article is to present 14 cases of osteosarcoma of the jaw treated at our medical center from 1989 to 1998. These cases are discussed in the light of a comprehensive review of 774 cases reported in the English literature over the past 3 decades. Differences between osteosarcoma of the jaws and osteosarcoma of the long bones are examined. SUBJECTS AND methods: The patients ranged in age from 8 to 78 years, the mean age being 33 years. Each patient had a histopathologically established diagnosis of osteosarcoma of the jaw. Records were reviewed for epidemiologic data, treatment modalities, and survival. RESULTS: Of the 14 patients, 6 (42%) had tumors in the mandible and 8 (58%) had tumors in the maxilla. Of the mandibular tumors, 5 occurred in the body of the mandible; all maxillary tumors originated in the alveolar ridge and involved the maxillary sinus. The chief complaint was an intraoral or extraoral painless swelling. Histopathologic types included chondroblastic, osteoblastic, fibroblastic, and malignant fibrous histiocytoma-like. Pathologic grade was determined to be high (3 or 4) in 13 cases and low (1) in only 1 mandibular case. All patients underwent surgical resection and immediate reconstruction. Adjuvant therapy included postoperative radiation (5 patients), postoperative chemotherapy (2 patients), and preoperative chemotherapy and postoperative radiation (1 patient). CONCLUSIONS: The results of the present study support the literature indicating that osteosarcoma of the jaw differs from osteosarcoma of the long bones in its biological behavior even though they have the same histologic appearance. Because of differences in tumor characteristics, the introduction of chemotherapy did not dramatically alter the prognosis of osteosarcoma of the jaw. early diagnosis and radical surgery are the keys to high survival rates.
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ranking = 0.00075506332677953
keywords = alveolar
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7/66. A highly aggressive primitive mesenchymal tumor with a translocation (1;19)(q12;q13.2).

    Soft tissue sarcomas constitute a heterogeneous group of malignant tumors of mesenchymal origin, the classification of which may present a diagnostic challenge. We present here the cytological, histopathological, immunohistochemical, and cytogenetic findings of an unusual case of a highly aggressive sarcoma. Based on the morphology and the immunohistochemical profile, this primitive tumor and its metastases could not be conclusively classified as any of the defined subtypes of sarcomas, although the findings were suggestive of a variant of rhabdomyosarcoma. Cytogenetic characterization using G-banding, SKY, FISH, and CGH revealed almost identical chromosomal compositions of the primary tumor and the metastasis. The hypertetraploid karyotype was characterized by numerical imbalances as well as by an unbalanced translocation t(1;19)(q12;q13.2), which has not been previously reported.
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ranking = 0.14231781190944
keywords = rhabdomyosarcoma
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8/66. Case report of embryonal rhabdomyosarcoma of the testis with an 8-year survival.

    The low incidence of embryonal rhabdomyosarcoma prevents any one person or institution from presenting any meaningful data in the form of effective treatment based on clinical experience. We realize that this is an antidotal case but the stakes are high when treating any malignancy of the young. Therefore, we advocate early and aggressive therapy using all modalities of treatments, and cite this case which initially seemed hopeless but to date has all the hallmarks of a cure.
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ranking = 0.71158905954722
keywords = rhabdomyosarcoma
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9/66. Malignant mesenchymoma of the heart.

    An autopsy case of malignant cardiac mesenchymoma, which arose in the atrial septum was presented. This tumor contained fibrosarcomatous, rhabdomyosarcomatous, chondrosarcomatous and osseous elements. A small metastatic lesion was found in the lung. Histogenesis of this tumor was also considered with a survey of literature.
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ranking = 0.14231781190944
keywords = rhabdomyosarcoma
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10/66. Testicular germ cell tumor with rhabdomyosarcoma successfully treated by disease-adapted chemotherapy including high-dose chemotherapy: case report and review of the literature.

    Treatment and prognosis have not been well characterized in germ cell tumors (GCT) with a malignant nongerm cell component. patients with a mediastinal tumor, neural or rhabdomyosarcomatous differentiation and distant metastases have the poorest prognosis. We report a rare case of mixed GCT composed of seminoma, teratoma and rhabdomyosarcoma with the rhabdomyosarcomatous component metastasized into the liver and bone marrow (BM) causing hypercalcemia. The patient was treated with differentiation-tailored chemotherapy (CHT) including a disease-adapted high-dose (HD) CHT regimen with purified autologous PBSCT (APBSCT) and pamidronate. To date, remission has lasted for 4 years. Tumor-adapted CHT including HD-CHT with APBSCT can induce long term remissions in high-risk patients with transformed GCT. A review of the literature is given.
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ranking = 0.9962246833661
keywords = rhabdomyosarcoma
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