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

Seventeen children with rhabdomyosarcoma stage I to III diagnosed since 1973 are presented. The coordinated management utilizing surgical excision, irradiation (5000 rad) and systemic adjuvant multiagent chemotherapy is described. The favorable sites were head and neck sites and genitourinary region. The mean survival is at present 19 months. 14 children are alive and well. 13 children had no tumor recidivation, 1 child had 8 months after beginning of the therapy lung metastases which after irradiation with 2000 rad disappeared. 3 children (stage III) died 9, 14 and 20 months after diagnosis during therapy by metastases. Acute and late effects on normal tissues from radiation and chemotherapy were noted in 12 cases. A review of the literature and therapeutic alternatives in the future are indicated.
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PMID:[Juvenile rhabdomyosarcoma. Diagnosis and new therapeutic possibilities]. 36 96

A newborn with a pleomorphic rhabdomyosarcoma of the eyelid was treated by chemotherapy, surgery, and radiation therapy and experienced a good response. The nine previous cases in the literature are reviewed and this patient's therapy discussed.
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PMID:Orbital rhabdomyosarcoma in a neonate. 38 75

Twenty-seven previously untreated children with gross residual (20) or metastatic (seven) rhabdomyosarcoma were treated with pulse-VAC (vincristine weekly for 12 doses plus dactinomycin and cyclophosphamide simultaneously given daily for 5 days) and radiotherapy. Toxicity during the 12-week induction period included 23 of 27 (85%) with an absolute neutrophil count (ANC) under 500/mm3; 16/27 (59%) were given intravenous (I.V.) antibodies. Three patients developed Gram-negative sepsis and two of them died. In the first 12 weeks, eight children had a complete response (CR) and another 10 a good partial response (PR), a total of 18 of 27 favorable responses (67%). At 12 weeks, 20 patients received either intermittent pulse-VAC (Regimen H) or a pulse of adriamycin plus vincristine and cyclophosphamide alternating with pulse-VAC (Regimen I) every 4--6 weeks. After this first "maintenance," only seven patients (35%) developed an ANC under 500/mm3 and only three (15%) were given I.V. antibiotics. Severe toxicity disappeared with drug dose reduction in subsequent courses. The overall CR rate was 59% with a PR rate of 15%, a total of 74% favorable responses. This rate is not significantly better than that obtained by previous IRS chemotherapy and radiotherapy schedules for patients with gross residual and metastatic rhabdomyosarcoma. Future studies in these patients will concentrate on diminishing myelosuppression while shortening the rest period between pulses, in order to deliver more drug per unit time.
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PMID:Evaluation of intensified chemotherapy in children with advanced rhabdomyosarcoma (clinical groups III and IV). The Intergroup Rhabdomyosarcoma Study (IRS) Committee of the Cancer and Leukemia Group B Children's Cancer Study Group, Southwest Oncology Group. 39 31

The classification of the histologic types of rhabdomyosarcoma is based on poorly defined criteria. This has resulted in marked disparities in studies reported from different institutions, as well as difficulties in assessment of the clinical behavior of the different histologic types. A retrospective morphologic analysis of 36 consecutive cases of rhabdomyosarcoma of childhood was undertaken according to predefined and strict guidelines for diagnosis. Undeflecting adherence to such criteria identified the embryonal type as the most common form, and the alveolar variant as a distinct clinicopathologic entity with a much more aggressive course; it also resulted in a large proportion (approximately one-fourth) of sarcomas of undertermined histogenesis. In spite of either prolonged follow-up observation with repeated biopsies, autopsy study, or electron-microscopic study of tumor tissue, no evidence could be obtained to substantiate the rhabdomyogenic derivation of the latter group of neoplasms. Precise systematization of the morphology of these cases may be contingent upon careful inventory of their fine structural features; current classifications appear to have disregarded the morphologic heterogeneity of this group of tumors.
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PMID:Rhabdomyosarcoma of infancy and childhood. Problems of morphologic classification. 39 6

Preliminary clinical trials using cryopreserved autologous bone marrow reinfusion have now been carried out at our institution in 5 children and 2 adults with advanced stages of neuroblastoma, rhabdomyosarcoma, non-Hodgkin's lymphoma and small cell carcinoma of the lung. Normal numbers of in vitro colony forming cells (CFU-C) were obtained from these patients despite prior courses of combination chemotherapy. The dose of marrow cells cryopreserved ranged from 1-6 X 10(8) cells/kg and recovery of CFU-C after thawing averaged 50%. Partial or complete hematologic reconstitution was achieved in all patients. The time for recovery ranged from 10-43 days for leukocytes (greater than 1000 cells/mm3) and 23-45 days for platelets (greater than 50,000/mm3). Two patients have died of interstitial pneumonitis due to cytomegalovirus. Three patients have died of recurrent tumor at 40, 48 and 156 days post-transplant. Two patients have had significant therapeutic benefit. One of these had a stable partial response permitting the use of further post-transplant therapy and is alive and well 16+ months post-transplant. The other patient had a complete response and remains tumor-free 25+ months following therapy. We conclude: 1) Autologous bone marrow reinfusion permits hematologic reconstitution following marrow-ablative therapy. 2) A quantity of marrow sufficient for this purpose remains viable following cryopreservation even when obtained from patients previously exposed to chemotherapy. 3) Autologous bone marrow reinfusion now allows the exploration of more intensive cytoreductive therapy in selected malignancies.
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PMID:Autologous bone marrow transplantation in the treatment of selected human malignancies: The Johns Hopkins Oncology Center Program. 40 Jun 94

The basic informations concerning pathology and clinical stagging of malignant tumours localized in urinary bladder and prostatic gland are introduction to proper presentation of own material collected and treated in the years 1962-1977. The material consists of 23 cases of rhabdomyosarcoma localized in minor basin. Formerly in the years 1962-1975, the basic form of treatment was surgery with/or radiotherapy. Chemotherapy that was given accidently in special indications as single dose or for a short time. Since 1975, we have started with systematic multidrug chemotherapy (VAC) used as an important part of complex therapy. Finally the authors discuss the place of chemotherapy in complementary treatment on the base of the literature and their own experience.
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PMID:[Chemotherapy of bladder and prostatic neoplasms in children]. 40 Jul 71

Results of management of rhabdomyosarcoma of childhood have improved in a dramatic manner during the recent 10 years as a consequence of the treatment by three modality approach which relies on an intensive multi-drug multicycle chemotherapy regimen combined with radiation therapy and or surgery. Both local results and total disease-free survival rates are markedly better with this more comprehensive approach. A staging system for sarcoma of soft tissue has just been developed by the A.J.C. Histopathological grade is the important parameter: stage 1,2, and 3 are tumors of histological grades 1, 2, and 3 respectively (an outline of the system is presented). Treatment results of 100 patients with sarcoma of soft tissue (extremities 89, torso 11) treated by radical dose radiation therapy (less than 6300 rad) and limited surgery at M.D. Anderson Hospital are presented. For both local control and disease free survival, results decreased with advancing stage and anatomic site was not a factor per se. Radiation therapy under tourniquet induced hypoxia was not found to be significantly more effective than conventional radical dose therapy. The necessity of planning treatment such that if subsequent surgery is required, the fields will provide the best distribution of unirradiated or low dose tissue for preparation of flaps, etc.
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PMID:Soft part tumors. 40 98

The production of a cytotoxic factor synthesized by human haemic killer cells growing in vitro is described. The factor can be found extra- and intra-cellularly. It is released from the cells by an apocrine form of secretion, illustrated by light and electron micrographs. The culture fluid from 14C-labelled killer cells reveals numerous radioactive bands following SDS-gel electrophoresis. The killing factor is precipitated by 30 to 60% saturation of ammonium sulphate. Cultures of human rhabdomyosarcoma and osteosarcoma cells are more susceptible to the killer cells than normal human dermal or lung fibroblasts. During contact or killer with target cells a higher level of cytotoxic activity can be detected in the culture fluid. The cell-killing activity is completely inactivated by 30 min at 60 degrees C, but it is not absorbed by target cells during 1 h of incubation. The cytotoxic factor is unlikely to be an interferon since it did not prevent the replication of a wide range of viruses and only a low level of interferon could be detected in the culture medium. The introduction of Strep. faecalis into cultures of killer cells caused their transformation into immunoblast-like cells, indicating their lymphoid origin. The cells did not phagocytose the microorganism. When the humoral factor was injected into fibro-sarcoma-bearing mice approximately 50% survived, whereas all control animals died.
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PMID:A humoral cytotoxic substance produced by a human killer cell line. 41 8

Masses in the mesenteries and omentum are often difficult to diagnose by conventional radiographic techniques. Gray scale sonography was a valuable adjunct to radiographic vector analysis in four children with such masses. Masses that are clearly separable from the liver and spleen and do not distort identifiable extraperitoneal structures are probably intraperitoneal. In children most cystic intraperitoneal masses are related to the mesenteries, omentum, ovary, or bile ducts. An anterior fluid collection with internal septa (which might be mistaken for loculated ascites) is the typical sonographic appearance of an omental cyst. Echogenic masses are more difficult to evaluate: careful study of the acoustical features yielded important information in cases of omental lipoma and rhabdomyosarcoma metastatic to the mesenteries and omentum.
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PMID:Sonographic evaluation of mesenteric and omental masses in children. 41 77

A case of primary cerebral rhabdomyosarcoma in a 51-year-old female is reported. The histogenesis of this tumor is discussed. The clinicopathological features of 10 previously reported similar tumors of the CNS are briefly reviewed. Histologically the tumor was polymorphic, but composed of poorly differentiated cells interpreted as rhabdomyoblasts without definite cross-striation. Electron microscopy established that the poorly differentiated cells were of rhabdomyosarcomatous nature, compatible with presumptive myoblasts and analogous to developing fetal muscle.
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PMID:Primary rhabdomyosarcoma of the cerebrum. An ultrastructural study. 41 33


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