Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Life-threatening airway obstruction from large mediastinal masses in children poses a difficult diagnostic and therapeutic dilemma, requiring the close coordination of a pediatric surgeon, anesthesiologist, radiologist, and oncologist. To focus on this problem, the anesthetic and surgical management of 50 consecutive children with mediastinal masses treated between 1978 and 1984 were reviewed. Thirty children presented with respiratory symptoms; nine had life-threatening respiratory compromise with dyspnea, orthopnea, and stridor. Thirteen of these symptomatic children had marked compression of the trachea and/or mainstem bronchi on radiographic studies. The tracheal cross-sectional area which was measured by computed tomography was decreased by 35% to 93% of the normal tracheal dimensions in these children. Nonresectable malignant neoplasms including lymphoma, Hodgkin's disease, rhabdomyosarcoma, and neuroblastoma were the eventual diagnoses in 10 of these patients. The other 3 patients were less than 4 years old and had benign lesions. General anesthesia was judged to be prohibitively risky in 5 of 13 patients. The diagnosis was established by node or needle biopsy under local anesthesia, and general anesthesia was deferred until the compromised airway was alleviated by radiation and chemotherapy. General anesthesia with endotracheal intubation was administered to 8 patients, 5 of whom developed total airway obstruction. Using a variety of maneuvers, ventilation was reestablished in all 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Life-threatening airway obstruction as a complication to the management of mediastinal masses in children. 408 8

We will report the result obtained from sensitivity tests on various anti-cancer agents for malignant bone and soft-tissue tumors based on SDI (Succinic Dehydrogenase Inhibition Test) method with the use of enzymic activities as marker since 1976. Our study comprised 27 cases altogether 15 cases of osteosarcoma, one case each of Ewing's sarcoma, malignant fibrous histiocytoma and malignant lymphoma, 3 cases of metastatic bone tumor and one case each of angiosarcoma, fibrosarcoma, rhabdomyosarcoma, liposarcoma, 2 cases of metastatic lung tumor among soft-tissue sarcomas. In all cases, sensitivity tests were done on the tumor tissues according to SDI method at the same time as biopsy for the determination of the appropriate medications. Four to six weeks of pre-operative intra-arterial infusion was done followed by radical operation. The results obtained are as follows. Observing the long-term results between subjects that applied anti-cancer agents decided by sensitivity test and those without sensitivity test. The 5 years cumulative survival rate jumped from 30.5% to 50.5%, showing a clear improvement.
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PMID:[Clinical evaluation on the sensitivity test for anti-cancer agents in malignant bone and soft-tissue tumors]. 608 33

Unusual clinical, radiologic, or histologic findings were found in 11 of 17 cases of hypercalcemia associated with childhood tumors. Four children had undifferentiated lymphoblastic lymphoma with extensive bone involvement, but no visceral or neurologic involvement. At diagnosis, four adolescents with rhabdomyosarcoma had numerous metastases, particularly in the breasts and bone marrow. Three infants had renal tumor without bone metastases. Histologically, their tumors differed from classical nephroblastoma and resembled the malignant rhabdoid tumors of the kidney. These findings allow individualization of three distinct groups of tumors with unusual features which may suggest the presence of hypercalcemia. These tumors appear to have a poor prognosis since all patients but one died of their malignancy.
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PMID:Hypercalcemia preferentially occurs in unusual forms of childhood non-Hodgkin's lymphoma, rhabdomyosarcoma, and Wilms' tumor. A study of 11 cases. 609 59

We have extended our analysis of human tumors using antibodies specific for each of the five types of intermediate filaments to neuroblastoma, ganglioneuroblastoma, pheochromocytoma, ependymoblastoma, and alveolar soft part sarcoma. Tumor cells in the three cases of neuroblastoma, as well as in the single case of alveolar soft part sarcoma, did not react positively with sera directed against any of the five intermediate filament types. We suppose, therefore, that neuroblastoma at least may be derived from a cell type - possibly present in peripheral neurones - which in vivo has very few or no intermediate filaments. In ganglioneuroblastoma and in pheochromocytoma the tumor cells were positive when tested with antibodies directed against neurofilaments and negative with those directed against other intermediate filament types. The ependymoblastoma was positive when tested with antibodies directed against glial fibrillary acidic protein (GFA) and negative when tested with antibodies against other intermediate filament types. Use of antibodies to the different intermediate filament types appears to be a valid way in which to classify tumors, and so far the data presented here and elsewhere support the hypothesis that tumor cells retain the intermediate filament type typical of their cell of origin. Wider use of these sera would seem particularly useful in cases such as neuroblastoma, rhabdomyosarcoma or lymphoma where diagnosis is currently difficult using conventional histological stains.
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PMID:Various sympathetic derived human tumors differ in neurofilament expression. Use in diagnosis of neuroblastoma, ganglioneuroblastoma and pheochromocytoma. 612 32

We attempted to determine the reliability of surface markers in distinguishing 21 small round cell tumors from lymphoid malignancies. Using immunofluorescence on tumor cell suspensions and immunoperoxidase on fresh frozen sections, we found that specimens of neuroblastoma (n = 7), rhabdomyosarcoma (n = 7), Ewing's tumor (n = 5), and two unclassified small round cell tumors all lacked human HLA-DR antigens. Each of eight tumors tested also lacked common leukocyte antigen (T200). In each of 13 cases studied, neither polyvalent surface immunoglobulin (sIg) nor receptors for sheep erythrocytes (E), complement (EAC), or the Fc portion of IgG immunoglobulin (EA) were found. Conversely, we found HLA-DR and/or T200 antigens, usually one or more receptors for E, EAC, or EA, and not infrequently, monoclonal sIg on malignant cells in each of 42 cases of lymphocytic lymphoma and leukemia. We conclude that study of surface DR and T200 antigens, sIg, and receptors for E, EAC, and EA aids the differential diagnosis of small round cell tumors from lymphocytic lymphoma and leukemia.
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PMID:Immunologic markers in the differential diagnosis of small round cell tumors from lymphocytic lymphoma and leukemia. 618 65

Cardiac tamponade resulting from malignant pericardial effusion is an uncommon initial presentation of various extracardiac malignancies. Only twenty-nine cases of extracellular malignancies with this initial presentation have been previously published. Lung carcinoma leads as the most common malignancy involved, followed by carcinoma of the stomach, pancreas, kidney and ovary, mediastinal rhabdomyosarcoma, malignant lymphoma and leukemia. This report describes a case of breast carcinoma in a 47-year-old woman who initially presented with cardiac tamponade. To the best of the authors' knowledge, no similar case has even been reported in the literature.
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PMID:Cardiac tamponade due to malignant pericardial effusion in breast cancer: a case report. 628 Aug 49

beta-Adrenergic receptors were demonstrated in membrane preparations from 6 human Ewing's sarcomas and compared to those from 46 other pediatric cancers with the use of the beta-adrenergic antagonist (-)-(3H)dihydroalprenolol [(-)[3H]DHA]. In contrast to the high numbers of receptor sites found in Ewing's sarcomas (55-640 fmol x mg-1 protein; dissociation constant Kd, 1-2 nM), other childhood cancers (neuroblastoma, rhabdomyosarcoma, brain tumors, lymphoma, osteosarcoma, hepatoblastoma, yolk sac, and Wilms' tumor) contained in general fewer beta-adrenergic receptor sites. Characteristics of (-)-[3H]DHA binding were therefore more fully characterized in the Ewing's tumors. Competition of (-)-[3H]DHA binding by classical catecholamine agonists, as well as by subtype selective agents metoprolol and zinterol, demonstrated the presence of a homogeneous population of beta 1-adrenergic sites in several Ewing's tumors. Adenylate cyclase activity in all Ewing's sarcomas was enhanced by GTP and NaF. However, in spite of high numbers of beta-adrenergic receptors, (-)-isoproterenol was not very effective in the activation of adenylate cyclase activity in several of the Ewing's tumors tested. Neither guanyl-5'-yl-imidophosphate nor GTP altered agonist potency for the receptor site in these catecholamine-insensitive tumors. Hill coefficients obtained from the competition experiments with (-)-isoproterenol (in the presence or absence of guanine nucleotide) were approximately 1.0. These uncoupled receptors were resistant to N-ethylmaleimide denaturation and were densensitized only 50% during culture in the presence of (-)-isoproterenol. Thus Ewing's sarcomas are relatively rich in beta-adrenergic sites, and several tumors appear to have a coupling lesion involving guanine nucleotide-dependent regulatory protein interaction with beta-adrenergic receptors and adenylate cyclase, similar in phenotype to that described in the (unc) variant of S49 mouse lymphoma.
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PMID:beta-Adrenergic receptors in pediatric tumors: uncoupled beta 1-adrenergic receptor in Ewing's sarcoma. 631 52

Of the solid tumors of childhood, neuroblastoma--the prototypic small-, round-, blue-cell neoplasm--occurs in the youngest patients and has shown the least predictable biologic behavior and response to therapy. It is often confused clinically and histologically with Wilms' tumor, rhabdomyosarcoma, lymphoma, and especially, Ewing's sarcoma. Certain clinical and histologic features that may be useful in prognosis have been identified, however, and a variety of distinctive light microscopic, electron microscopic, and immunohistochemical features may be useful in differentiating this and related tumors. Many of the varied techniques useful in the differential diagnosis of these tumors can now be employed routinely in most laboratories.
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PMID:Neuroblastoma and the differential diagnosis of small-, round-, blue-cell tumors. 634 38

During the past 2 years percutaneous fine-needle aspiration biopsy has been employed to help establish or confirm diagnosis in 32 infants and children. A 22-gauge needle is used to aspirate the site of suspected disease. For lesions inside the chest or abdomen, the needle is guided with the help of fluoroscopy, ultrasound, or computed tomography (CT) scan. Nineteen of the 32 patients had malignant disease, including lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, osteosarcoma, and leukemia. In all patients with solid tumors, tissue obtained at operation confirmed the accuracy of the diagnosis. In seven children with suspiciously enlarged lymph nodes, an open biopsy was avoided when the needle aspirate was clearly benign. In four children, the early appearance of metastatic or recurrent malignancy was confirmed without the need for open biopsy. In this small series, there were no false-negative or false-positive needle aspirates, and no complications directly related to the procedure. The skill and experience of the cytopathologist is essential to the success of this technique. Percutaneous fine-needle aspiration biopsy is a safe and reliable alternative method of establishing a diagnosis in infants and children with suspected malignant disease.
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PMID:The usefulness of percutaneous fine-needle aspiration biopsy in infants and children. 657 17

The effects of adriamycin and daunomycin on cardiac function were studied in 33 patients with acute leukemia (16 cases), neuroblastoma (5 cases), osteosarcoma (4 cases), malignant lymphoma (3 cases), rhabdomyosarcoma (3 cases) and malignant histiocytosis (2 cases). The left ventricular function was evaluated by serial echocardiographic assessment. Ejection fraction (E.F.) and shortening fraction (S.F.) of left ventricule were calculated from echocardiographic measurements. Seven of 33 cases (21.2%) revealed the decrease of E.F. and S.F. There was the significant correlation between total doses of daunomycin and E.F. Three patients died of severe congestive heart failure probably due to daunomycin administration. Usually, cardiac dysfunction caused by these drugs has improved within 3 months after the discontinuation.
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PMID:[Effects of adriamycin and daunomycin on cardiac functions]. 663


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