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 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cerebral metastases have not yet been reported in the literature despite extensive reports on the pathology of alveolar rhabdomyosarcoma. The morphological diagnosis of rhabdomyosarcoma was supported by the immunohistochemical demonstration of desmin, myosin, and myoglobin in the tumor cells.
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PMID:Alveolar rhabdomyosarcoma in a young female patient metastasizing to the brain. 405 Mar 50

We present three cases of primary bone sarcoma with rhabdomyosarcomatous component which in one case appeared in a pure form, i.e., as rhabdomyosarcoma; in the other two cases it presented as one of multiple components of the tumor. The first patient was a 70-year-old man with a tumor of the left femur involving surrounding soft tissue of the thigh. Histologically, this was a case of so-called dedifferentiated chondrosarcoma with pleomorphic rhabdomyosarcoma representing high grade malignant component. The second patient, a 31-year-old man, developed a tumor in the left tibia which was a rare, fibrosarcoma-like type of rhabdomyosarcoma. The third patient was a 43-year-old woman with a tumor in the left tenth rib which was shown to be osteo- and chondrosarcoma with a minor rhabdomyosarcomatous component and was classified as malignant mesenchymoma. All patients were treated by surgery and chemotherapy. The first two of them died of metastatic disease 3 months, and 3 years and 4 months following surgery, respectively. Light microscopical diagnosis of rhabdomyosarcoma of hematoxylin-eosin sections was confirmed immunohistochemically in each case using reactions to desmin, muscle specific actin and myoglobin, and ultrastructurally in two cases. Rhabdomyosarcomatous component in primary bone tumors appears to be rarely present but more cases may be diagnosed in the future, if immunohistochemical and ultrastructural examinations are to be employed.
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PMID:Primary bone sarcoma with rhabdomyosarcomatous component. 806 89

A 36-year-old female presented with a palpable mass in the right breast. She received a modified radical mastectomy after admission. Brain and multiple bony metastases were noted 10 months later. Microscopically, the tumor was composed of nodules of small spindle and large round cells showing abundant eosinophilic, vacuolated cytoplasm and prominent nucleoli. No glandular or ductular differentiation could be identified. By immunohistochemistry, the tumor cells were positive for vimentin and desmin in focal areas, but negative for cytokeratin, S-100, epithelial membrane antigen, actin, myoglobin, or alpha-1-antichymotrypsin. Electron microscopy showed focally cohesive large cells maintained together by poorly-defined intercellular junctions and surrounded segmentally by thin basal laminae. Intracytoplasmic filaments were identified in some tumor cells, diffusely arranged in the cytoplasm. These findings indicate that this was a case of epithelioid leiomyosarcoma of the breast. The literature concerning this rare neoplasm is reviewed.
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PMID:Epithelioid leiomyosarcoma of the breast: report of a case. 810 88

A subcutaneously transplantable tumour (SMT-Y) was established from a smooth muscle tumour arising from the uterus of a female F344 rat. SMT-Y was serially passaged by subcutaneous implantation into syngeneic female rats up to the 15th generation, but transplantation failed in males. The rat with the primary uterine tumour also had mononuclear cell leukaemia (MCL), and MCL cells grew concurrently in implanted rats. At passage five, MCL cells were eliminated from transplants by implanting the central part of an SMT-Y nodule, consisting only of neoplastic smooth muscle cells. SMT-Y at passages six to 15 was examined biologically and morphologically. The primary tumour and SMT-Y tumours consisted mainly of interlacing fascicles of elongated and fusiform neoplastic smooth muscle cells with abundant cytoplasm. Occasional cells showed nuclear atypia. Mitosis counts per 10 high-power microscopic fields in the primary tumour and SMT-Y ranged from 11 to 36. Neoplastic cells reacted positively for desmin, muscle actin and myosin, but not for myoglobin. Electron microscopy revealed cytoplasmic myofilaments with oval dense bodies. These findings suggested a smooth muscle origin of SMT-Y and it was regarded as a leiomyosarcoma by the criteria for human uterine smooth muscle tumours. Despite the malignant histological features, SMT-Y grew slowly into a large nodule, with an average diameter of 5 cm and average weight of 81 g, 24 weeks after transplantation. Neither invasive tumour growth nor metastases were observed in SMT-Y-bearing rats.
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PMID:Biological behaviour and morphological characteristics of a transplantable tumour derived from a uterine smooth muscle tumour in the F344 rat. 810 68

Four cases are presented of primary anterior mediastinal tumors in young adults that were characterized by solid, infiltrative lesions showing histologic and immunohistochemical features of rhabdomyoblastic differentiation. The patients were three men and one woman between 19 and 27 years of age (mean age, 23 years). All patients presented with symptoms referable to their tumors, including cough, chest pain, dyspnea, and left-sided pleural effusion. Grossly and radiographically, the lesions were characterized by their solid, infiltrative appearance. Histologically, two cases corresponded to the solid variant of alveolar rhabdomyosarcoma, one case was an embryonal rhabdomyosarcoma with a predominant spindle cell component, and the remaining case showed the features of a pleomorphic rhabdomyosarcoma. No glandular, epithelial, or other component could be identified in any of the tumors on extensive sampling. Immunohistochemical studies showed positive staining of the tumor cells with actin, desmin, and vimentin antibodies, with focal positivity for myoglobin in three cases and focal positive staining with S-100 protein in one case. Stains for low and high molecular weight keratin, carcinoembryonic antigen, alpha-fetoprotein, human chorionic gonadotropin, placental alkaline phosphatase, leukocyte-common antigen, and neuron-specific enolase were negative. All patients experienced rapid recurrence and metastases within the first 6 months after diagnosis. Three patients died within this period due to their tumors; the fourth patient has been lost to follow-up. Pure primary rhabdomyosarcomas of the anterior mediastinum are highly aggressive neoplasms that should be distinguished from germ cell, teratomatous, or carcinosarcomatous tumors with a focal rhabdomyoblastic component.
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PMID:Rhabdomyosarcomas of the anterior mediastinum: report of four cases unassociated with germ cell, teratomatous, or thymic carcinomatous components. 816 67

A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. A 13-year-old boy presented with a recurrent painless mass on the ventral side of the right forefoot. Microscopically, the tumor consisted of spindle-shaped and round tumor cells in a fascicular or storiform, focally hemangiopericytoma-like growth pattern. The cytoplasm of the spindle-shaped tumor cells was eosinophilic and fibrillary, in some areas resembling smooth muscle cells. Immunohistologically, all tumor cells were vimentin-positive, most of them also stained with antibodies to desmin and muscle specific actin (MSA). In addition, many tumor cells showed a co-expression of alpha-sarcomeric actin and myoglobin. All tumor cells were negative with alpha-smooth muscle actin. Two years after surgical treatment and chemotherapy the patient is well with no evidence of distant metastases. The clinicopathological features and differential diagnostic problems are discussed.
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PMID:Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma. 851 10

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

Five children with congenital-infantile fibrosarcoma are analyzed. The tumor was found at birth in four children: in one patient it was recognized at the age of 7 months. In three children the tumor affected the lower extremity. In one patient the inguinal region was the primary site, in another the abdominal wall. The morphology was that of a highly cellular spindle cell sarcoma with cells arranged in a fascicular pattern. Variations of this common pattern such as a cartwheel arrangement, and foci of small oval cells were observed. The immunohistochemistry revealed positivity of vimentin in four investigated tumors and muscle specific actin in three. Desmin, sarcomeric actin and myoglobin were all negative. There were scattered cells positive with KP1 (CD68), MAC 387, and in one case, with factor XIIIa antibodies which were considered to be reactive rather than tumor cells. The flow cytometry study showed DNA content in three tumors within diploid range; one tumor was hyperdiploid with the DNA index 1.2. Three children are disease-free from nine to 21 years after the diagnosis. One of them had the tumor preoperatively irradiated, and the subsequent histological examination revealed an almost complete tumor necrosis. In one patient there were six recurrences (treated by surgery only), and the child is well 25 months after the last recurrence. In one child the disease had an unusually aggressive course, and the patient died of widespread metastases to the lungs, lymph nodes and bones.
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PMID:Congenital-infantile fibrosarcoma: a clinicopathological study of five patients entered on the Prague children's tumor registry. 889 21

Childhood kidney tumors seldom metastasize into the cranial cavity unless it is a special histological variant. We report a 4-year-old boy with multiple intracranial metastases in the left parietotemporal and right cerebellar area from primary clear cell sarcoma of the kidney without evidence of bony metastases. Metastatic tumor revealed nests of uniformly polygonal cells with clear cytoplasm demarcated by delicate fibrovascular arcades. Tumor cells were positive for vimentin and negative for cytokeratin, S-100 protein, desmin, and myoglobin. Cellular proliferation rate measured by PCNA, and Ki-67 was not significantly different between primary tumor mass and metastatic brain lesion. Expression of p53 oncoprotein was not evident in both lesions. These findings suggested that the relapse and metastasis of clear cell sarcoma of the kidney was probably due to regrowth of micro-metastases which were present at an early stage of disease.
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PMID:Intracranial metastasis from clear cell sarcoma of the kidney--a case report. 936 10

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.
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PMID:Spindle cell rhabdomyosarcoma (so-called) in adults: report of two cases with emphasis on differential diagnosis. 953 74


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