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)

A large inbred family is described in which there were seven cases of Hodgkin's disease, three of lymphosarcoma, two of thymoma, two of common variable immunodeficiency, and single cases of retinoblastoma, neuroblastoma, and rhabdomyosarcoma. There have been no other lymphoma cases in the community during the past decade. Further study of this family may help to define the genetic basis for development of Hodgkin's disease and other disorders.
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PMID:Common variable immunodeficiency, Hodgkin's disease, and other malignancies in a Newfoundland family. 4 22

104 patients with various cancer, excluding malignant lymphoma and leukemia, underwent bone marrow biopsy using a Jamshidi needle, regular type. In 100 patients an adequate pice of bone marrow was obtained. In 24 patients metastases were detected in the bone marrow. Metastases were found in 10 of 38 (26.3%) patients with breast cancer, in 5 of 17 (29.4%) patients with lung cancer, in 5 of 10 (50%) patients with cancer of the prostate, in 1 patient with rhabdomyosarcoma, 1 with chordoma and in 2 of 14 patients who underwent biopsy in search of unknown cancer. 71% of the patients with positive findings in the bone marrow had clinical signs of bone involvement, 80% had positive X-ray film and 78.9% had positive skeletal isotope survey. Hemogram, serum alkaline phosphatase, serum calcium level and sedimentation rate were of no value in predicting whether the marrow was involved or not. No complications were documented following biopsy. The use of the Jamshidi bone marrow biopsy needle for staging and early detection of metastases in a select group cancer patients is suggested.
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PMID:Bone marrow biopsy in patients with malignant neoplasms other than lymphomas or leukemia. 11 9

Studies of murine leukemia virus expression in AKR mice are presented. Material from in vivo and in vitro sources of normal tissues and lymphomas was assayed for in vitro infectivity, using the XC plaque assay, and for oncogenicity, by assessing lymphoma-accelerating capacity after inoculation into newborn animals. Normal tissues from healthy young AKR mice up to 7 months of age were found to have XC but not oncogenic activity. XC activity persisted, and weak oncogenic activity appeared in older mice. Cocultivation of normal young cells with NIH Swiss mouse embryo cells did not result in the appearance of oncogenic activity, although XC virus increased in titer. A cell-free filtrate of a virus-accelerated lymphoma was studied for host range. Virus as measured by polymerase and gs antigen was found to be propagated on NIH Swiss mouse embryo and wild mouse embryo cells, but not on human rhabdomyosarcoma, normal rat kidney, rabbit corneal, and BALB/c embryo cells. Virus as measured by the XC assay grew better on NIH Swiss mouse than on BALB/c embryo cells. Both of these cell lines propagated virus as measured by the oncogenicity assay. Supernatants from an in vitro cell line from a virus-accelerated lymphoma did not produce XC plaques but were oncogenic. Those from two cell lines of spontaneous lymphomas were negative with both assays. Cultivation of supernatants from these cultured lymphoma cells with NIH Swiss mouse embryo cells resulted in material which produced small plaques on the XC assay. These findings are interpreted as showing the presence of two viruses in AKR mice. One is XC positive and present throughout life. The other is oncogenic, appears later in life, and could be a separate virus or a variant of the first one.
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PMID:A discrepancy in XC and oncogenicity assays for murine leukemia virus in AKR mice. 18 12

This report describes a unique clinicopathologic entity characterized as a malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents (average age 14.5 years). There was a female predilection (75%) for this tumor which appeared to originate in the soft tissues of the chest wall or the peripheral lung. The neoplasm tended to recur locally and did not seem to disseminate as widely as some of the other small cell tumors of childhood (rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma and malignant lymphoma). However, the median survival was only 8 months. Electron microscopy of 3 cases suggested a neuroepithelial derivation, but, at the present, the histogeneis remains a subject for further investigation.
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PMID:Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. 22 26

VP-16-213, a semisynthetic podophyliotoxin, was tested for antitumor and clinical toxicity in 126 children. The drug was administered iv daily x 5 days every 2 weeks at a starting dose of 75 mg/m2/day. The dose was increased by 25 mg/m2/day/course until clinical response or significant toxicity occurred. The only major toxicity was hematologic, with neutropenia as the most predominant feature. There was one local allergic reaction at the site of injection. No systemic allergic responses were reported. The drug demonstrated significant activity in acute myelomonocytic leukemia with four responses among 19 patients, less activity in acute myelocytic leukemia with two responses among 44 patients, and little activity in acute lymphocytic leukemia with only one partial response among 12 patients. Objective partial responses occurred in ten of 48 patients with solid tumors: two each with Wilms' tumor, lymphoma, and histiocytosis X, and one each with rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and undifferentiated carcinoma. The inclusion of VP-16-213 in combination chemotherapy for childhood acute myelomonocytic leukemia and acute myelocytic leukemia appears indicated in patients relapsing after initial therapy. For solid tumors this is an interim report, with further patient accrual required before specific comments can be made.
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PMID:Phase II study of VP-16-213 in childhood malignant disease: a Children's Cancer Study Group Report. 29 6

With modern techniques it is usually possible to deliver a high dose of radiation to an orbital tumour without destroying vision. Local cure of lymphoma is possible with no risk of complications. Rhabdomyosarcoma now has a very good prognosis if treated energetically by radiotherapy and chemotherapy. Radiotherapy also has a part to play in the management of lacrimal gland tumours, melanoma, and metastases. A recent series of cases provides evidence that radiotherapy may be the best treatment for some cases of pseudotumour.
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PMID:Role of the radiotherapist in orbital disease. 29 17

ICRF-159 is active in several animal tumor model systems and human adult malignancies. In this phase II study, ICRF-159 was given on a weekly schedule, 3000 mg/m2/day, orally in three divided doses at 6-hour intervals to 78 children with a variety of malignant neoplasms. Fifty-three patients were evaluable for tumor response. Toxicity was primarily hematopoietic and gastrointestinal. There were no responses in any of the eight patients with osteogenic sarcoma, four with lymphoma, five with Ewing's sarcoma, ten with neuroblastoma, or six with rhabdomyosarcoma. There was a transient partial response in one of four children with Wilms' tumor. Further trials with this drug using this schedule are not indicated for the common childhood solid tumors.
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PMID:ICRF-159 (razoxane) in the treatment of pediatric solid tumors: a Southwest Oncology Group study. 47 12

Six patients with malignant head and neck tumors are shown to have required electron microscopy for accurate diagnosis. In all of these tumors, there were ultrastructural features of cytodifferentiation that were not discernible by light microscopy, such as neurosecretory granules, desmosomes, cytoplasmic processes, tonofibrils, and myofilaments. Electron microscopy is helpful in the differential diagnosis of tumors in general, but its effectiveness is particularly apparent in small-cell "undifferentiated" tumors such as neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, undifferentiated squamous-cell carcinoma of the lymphoepithelioma type, and malignant lymphoma. It has also been helpful in the identification of amelanotic melanoma and spindle-cell carcinoma.
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PMID:Electron microscopy in the diagnosis of head and neck tumors. 50 Mar 59

Forty-one children with subdiaphragmatic rhabdomyosarcoma underwent bipedal lymphography. Twenty-two (53.5%) of the lymphograms were interpreted as being positive. In our series, the lower limbs were the most common primary site of involvement, were more frequently involved by the alveolar histologic subtype which carries a poor prognosis, and were associated with a higher incidence of lymph node metastases. Positive lymphographic findings in this group of children were similar to those seen in both adults and children with other solid tumors, i.e., the presence of discrete lymph node filling defects. However, in 3 cases, abnormalities more characteristic of lymphoma were identified. Evaluation of lymph node metastases as demonstrated by lymphography has prognostic significance.
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PMID:Lymphography in childhood rhabdomyosarcomas. 50 42

Rhabdomyosarcoma usually presents as a soft tissue mass, which may invade adjacent bone. However, the patient presents occasionally with bony metastases. Recognition of these is important for staging and management. Fifty-eight cases have been reviewed; 14 of these had local bone invasion by the soft tissue tumour. All bones involvel were flat bones; 12 showed permeated bone destruction and two showed geographic destruction. Bone expansion was seen in half the involved bones. Twelve of the 58 cases showed secondary bone deposits, which were the presenting feature in five. Although 10 cases had permeated bone destruction, two were very well defined with a wide range of radiological appearances. The radiological differential diagnosis includes neuroblastoma, leukaemic infiltration, lymphoma, histiocytosis X, solitary and multifocal osteosarcoma and other deposits.
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PMID:The radiology of bone changes in rhabdomyosarcoma. 62 1


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