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)

The original diagnosis of rhabdomyosarcoma (RM) was critically evaluated by histology, immunohistochemistry, and electron microscopy in a retrospective series of 25 patients older than 40 years of age. Only two of the 25 patients (8%) were verified to have RM by strict criteria. By light microscopy, the true RM had a spindle or round cell appearance and were subtyped as embryonal RM, although some pleomorphism was present. Sarcomeric differentiation was revealed by electron microscopy, and desmin and muscle actin by immunohistochemistry. Both of these tumors arose in the urogenital organs, one in the urinary bladder and the other in the prostatic region. Both patients died within 3 months of the diagnosis. The other tumors not verified as RM were pleomorphic or spindle cell sarcomas (n = 17), ten of which were considered to represent malignant fibrous histiocytoma, or had desmin and/or muscle actin, and were verified as leiomyosarcomas by electron microscopy (n = 2). There were single cases of undifferentiated carcinoma, probable neuroendocrine carcinoma, extraskeletal osteosarcoma, and pleomorphic liposarcoma. The average survival for the non-RM tumor patients was 32 months. The results show that true RM do occur in the elderly, but they are very rare. Such tumors may have clinicopathologic properties similar to embryonal RM in children both in regard to the site of origin and to the histologic picture. True RM seems to carry a worse prognosis than other pleomorphic sarcomas, and this emphasizes the need for accurate diagnosis.
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PMID:Rhabdomyosarcoma in patients older than 40 years of age. 316 18

Five patients with nonlymphomatous sarcoma metastatic to the brain are presented and the literature reviewed. In 50 patients, brain metastasis was verified pathologically and some case data were available, including the five reported here. In five patients, there was pathologic verification but no case data. Thirty-nine other patients were found without such verification. Reportedly, most types of sarcomas are able to metastasize to the brain, and are represented in these 94 patients. Fibrosarcoma and alveolar soft-part sarcoma tumors metastasize to the brain without regard to treatment technique. There may be a group of tumors, including malignant fibrous histiocytoma, rhabdomyosarcoma, and perhaps leiomyosarcoma and osteosarcoma, in which the incidence of brain metastases has increased with improved sarcoma chemotherapy (CT). In this group particularly, but also in alveolar soft-part sarcoma and others, the presence of lung metastases may increase the probability of brain metastasis occurring subsequently.
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PMID:Sarcoma metastatic to the brain. 327 83

A case of embryonal rhabdomyosarcoma of the femur observed in an 11-year-old boy is reported, which simulated an osteosarcoma clinically and radiographically. To our knowledge, this is a second documented case of rhabdomyosarcoma of the long bone.
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PMID:Primary rhabdomyosarcoma of long bone. A case report. 345 39

Seven patients underwent resection of a sarcoma of the extremity requiring excision of a vital artery and revascularization. These included osteosarcoma of the pubis, osteosarcoma of the distal femur, undifferentiated sarcoma of the thigh, liposarcoma of the thigh, liposarcoma of the popliteal space, chondrosarcoma of the proximal tibia and rhabdomyosarcoma of the thumb. Preoperative evaluation included computed tomographic scan, magnetic resonance imaging and angiography. Operation involved excision of the iliac artery in one instance, femoral artery in three, popliteal artery in two instances and radial artery in one. Wide local resection including revascularization was undertaken when the tumor could be resected with the artery but separated from the nerve and it was a low grade malignant disease or the patient refused amputation for a high grade malignant disease. In five patients, the tumor margins were adequate. One patient with an osteosarcoma of the pubis with distant disease had a palliative resection and one patient had positive margins but refused amputation. Coverage of the soft tissue and vascular grafts was achieved using a distant pedicle flap in two patients. Amputation was avoided and each patient remained ambulatory. Five patients remained free of disease with patent grafts at six months to six years of follow-up study. One patient died of late myocardial infarction and one who underwent palliative resection died eight months later of metastatic disease. Involvement of the major arterial circulation does not preclude adequate resection of sarcomas of the extremity with limb salvage.
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PMID:Limb sparing operations for sarcomas of the extremities involving critical arterial circulation. 347 4

Studies of the presenting height of children with malignancies have produced conflicting results, from an excess of taller patients to an excess of shorter patients. The problems of measurement bias, inadequate comparison populations, small numbers of patients, subgroup analyses, and overreliance on simple significance tests are all possible reasons for the variation in results. To clarify this issue, we studied heights at diagnosis of 3657 children and adolescents aged under 18 years. Their malignancies included acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, acute non-lymphoblastic leukaemia, osteosarcoma, retinoblastoma, neuroblastoma, Wilms' tumour, rhabdomyosarcoma, and Ewing's sarcoma. Compared with published standards for the heights of children in control populations, no significant deviation from population norms was found for patients in any of the 10 disease categories after proper adjustment for multiple significance testing.
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PMID:Height at diagnosis of malignancies. 360 84

During a 37-year period, 26 patients were seen who had chondrosarcoma with additional mesenchymal components ("dedifferentiated low-grade chondrosarcoma"). Sixteen were men and 10 were women aged 30 to 85 years (median, 61 years). The tumors' chondroid areas were of borderline or low-grade malignancy. The additional mesenchymal component was histologically classified as malignant fibrous histiocytoma (16), rhabdomyosarcoma (4), low-grade fibrosarcoma (3), osteosarcoma (2), and undifferentiated sarcoma (1). Preferred locations were pelvis (10) and femur (8). Symptoms had been present for 1 year or less in most cases. Pain was the most common symptom. In 15 of 26, major amputation was the primary treatment. Twelve patients received chemotherapy, usually after developing metastatic disease, but only one achieved a partial response. Median disease-free interval after diagnosis was 4 months, median survival was 6 months, and 19 patients died within 1 year. Of 4 who survived longer than 18 months, 3 presented with a low-grade fibrosarcoma. Survival and development of metastasis appeared unrelated to cell type, initial treatment, or chemotherapy, except when the tumor's initial nonchondroid component was low-grade fibrosarcoma.
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PMID:Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathologic study of 26 cases. 371 21

Local control of the primary tumour is a fundamental requirement for clinical cure. Towards this aim, the primary tumour must be diagnosed early and identified histologically. The size, extension, and spread within the patient must be defined precisely. In planning effective local therapy, additional questions must be answered including resectability, mutilation, sensitivity to radio- and chemotherapy, anticipated morbidity from therapeutic measures, etc. For osteosarcoma there is no reasonable alternative to radical surgery. Because of the 20% local recurrence rate of Ewing's sarcoma following radiotherapy, radical surgical removal of the primary tumour should be attempted whenever possible. For rhabdomyosarcoma, particularly for its embryonal histology, non-radical removal of the primary sarcoma is still compatible with a cure, provided adequate radio- and chemotherapy is also administered. Primary irradiation is indicated in radiosensitive unresectable primary tumours and may convert these into an operable state. Chemotherapy is the domain of prevention and treatment of metastatic disease. It has, however, also a proved effect on primary tumours and, in several recent protocols, precedes local therapy.
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PMID:Local therapy of rhabdomyosarcoma, osteosarcoma and Ewing's sarcoma of children and adolescents. 386 80

Sarcomas of childhood rank fifth in incidence of malignant tumors in children younger than 15 years. Among the soft tissue sarcomas, approximately 50% are rhabdomyosarcomas. The remainder represent a heterogeneous group of diverse sarcomas which are not unique to children and include fibrosarcoma, synoviosarcoma, malignant fibrous histiocytoma, malignant schwannoma, angiosarcoma, leiomyosarcoma, and others. The most common bone cancers in childhood are osteosarcoma and Ewing's sarcoma. Although a multidisciplinary approach utilizing surgery, irradiation, and combination chemotherapy is routinely used in management of virtually all children with solid tumors, the value of adjuvant chemotherapy in select bone and rare soft tissue sarcomas is currently being tested. Multiagent chemotherapy including vincristine, dactinomycin, cyclophosphamide, and Adriamycin (doxorubicin) contribute to cure rates in 65% to 75% of children with localized rhabdomyosarcoma, Stages I to III, when combined with surgery and/or irradiation. Other drugs which hold promise include platinum, DTIC, methotrexate, and VP-16. The efficacy of similar drugs in the rarer pediatric soft tissue sarcomas other than rhabdomyosarcoma and its variants requires prospective randomized trials evaluating histologic grade, tumor size, and nodal status. It has been suggested that the high-grade sarcomas presenting with minimal tumor bulk are most sensitive to combined radiotherapy-chemotherapy, whereas the low-grade sarcomas are more resistant to such therapy. Tumor cell heterogeneity contributes to biologic diversity and response to treatment. Chemotherapy as adjuvant therapy to irradiation is currently recommended and utilized for Ewing's sarcoma with survival rates approaching 80%, and disease-free survival of approximately 75% for those with localized disease. Children with widespread and metastatic disease at presentation fare less well. Although multiple single agents exhibit response rates ranging from 40% to 60%, including cyclophosphamide, Adriamycin, dactinomycin, BCNU, mithramycin, and 5-fluorouracil, new and more effective agents are needed. Controversy regarding the value of multiagent chemotherapy in osteosarcoma has stimulated prospective randomized trials. Evaluation of local control rates as well as sites and occurrence of metastases are essential in assessing the contribution of aggressive combined modality therapy in the pediatric sarcomas. Emphasis on refinement of therapy in determining the risk/benefit ratio from adjuvant chemotherapy in pediatric sarcomas is mandatory. Enhancement of early local reactions is apparent when adjuvant chemotherapy is used with local radiotherapy.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The value of adjuvant chemotherapy in the management of sarcomas in children. 388 37

An autopsy case of a huge tumor in the right thorax is reported. A 71-year-old man complained of dyspnea and died after three months of hospitalization. The tumor, consisting of a circumscribed mass measuring 24 X 24 X 8 cm and weight 3.7 kg, originated from the right diaphragm and had no metastasis. Histologically, the tumor comprised multiple mesenchymal elements of fibrosarcoma, rhabdomyosarcoma, osteosarcoma and chondrosarcoma, and there was no distinct border between these different components. This tumor fulfills the criterion of malignant mesenchymoma (Stout). The histogenesis of the tumor is discussed with a survey of literature.
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PMID:[An autopsy case of malignant mesenchymoma of the diaphragm]. 403 73

Twenty children with recurrent or unresponsive tumours (10 Wilms', 3 rhabdomyosarcoma, 4 Ewings's, 1 osteosarcoma, 1 hepatoblastoma, 1 hepatoma) and one untreated patient with renal carcinoma were given ifosfamide as a 24-h infusion (5 mg/m2), with mesna as uroprotective. The number of courses ranged from 1 to 13 (median 3), and the interval between them was 2-3 weeks. Sixteen of these patients had previously received cyclophosphamide. Complete clinical responses were seen in 3 cases (2 Wilms' and 1 Ewing's) and lasted 5, 7, and 9 months. Partial responses were seen in 3 instances, mixed response or stable disease in 4, and progressive disease in 11. Treatment was well tolerated in most patients, with no cystitis or severe myelosuppression, but 2 children developed transient neurological symptoms and 1 became hypertensive. Nausea and vomiting were controlled by high-dose dexamethasone in most children. Plasma ifosfamide levels were estimated by means of gas-liquid chromatography in 10 patients. Peak concentrations ranged from 38 to 125 micrograms/ml (median 80). The elimination half-life, at 2.5-5.2 h (median 3.2) was shorter than previously reported in adults. Future studies should test the possibility that ifosfamide-containing combination chemotherapy may be more effective than the regimens, usually including cyclophosphamide, that are currently used as front-line treatment of embryonal and Ewing's sarcoma.
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PMID:A phase II study of ifosfamide in children with recurrent solid tumours. 405 69


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