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 review of 1415 patients without distant metastasis from the Intergroup Rhabdomyosarcoma Study (IRS) I and II revealed an overall 10% incidence of identified lymphatic spread at diagnosis, whereas 81 of 592 children with localized rhabdomyosarcoma who had grossly complete resection (and therefore with more complete pathologic data) had histologically proven lymphatic spread (14%). The percentage of patients in this latter group with nodal metastases was highest for the prostate (41%), paratesticular sites (26%), and genitourinary sites overall (24%). Sites with a small percentage of proven lymphatic involvement were the orbit (0%), nonorbital head and neck sites (7%), and truncal sites (3%), whereas the percentage of patients with nodal metastases from extremity lesions was 12%. The primary tumor mean diameter was significantly larger in the group with nodal metastases, but there was no evidence of a relationship between lymphatic spread and age, sex, or histologic subtype. Patients with lymph node metastases who had resection had a poorer survival rate (logrank P value = 0.001), with a 3-year survival estimate of 54%, compared with 78% for patients without lymphatic metastases. Patients with extremity lesions and positive lymph nodes also did poorly when compared with patients with normal nodes (P = 0.006), and a similar observation was made for patients with paratesticular sarcoma (P = 0.06).
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PMID:Lymphatic metastases with childhood rhabdomyosarcoma. A report from the Intergroup Rhabdomyosarcoma Study. 329 2

Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. Several decades ago it was associated with an 80% mortality rate. Recent advances with combined modality therapy have improved the 5-year survival rate to almost 70%. The focus for the improvement in treatment regimens is now shifting to concerns regarding the preservation of body function and the treatment of far advanced disease. Regardless of age, histology, stage, or site of primary tumor, there has been no group where chemotherapy has not been proven beneficial. The current recommendations used by the Intergroup Rhabdomyosarcoma Study, with regard to chemotherapy, are summarized. Radiation therapy adjunctive to surgery has a useful place in the treatment of rhabdomyosarcoma, but prophylactic nodal irradiation is not recommended. Involved nodes should, however, be treated. Since the potentially adverse side effects of chemotherapy and radiation are accentuated when combined, radiation therapy is no longer recommended in patients with group I rhabdomyosarcoma. Prognostic factors and future considerations from the operative standpoint are presented.
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PMID:Advances in the treatment of rhabdomyosarcoma. 333 Feb 73

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

A 79-year-old woman with primary liposarcoma of the heart, an extremely rare malignancy, coexistent with Brenner tumor was reported. The histological features of the sarcoma on the routine histological slides were so varied that it was necessary to differentiate the histology from that of malignant fibrous histiocytoma or pleomorphic rhabdomyosarcoma. Most of the tumor cells including fibroblastic and bizarre giant cells, however, were stained intensely with histochemical methods for lipids and consequently diagnosed as pleomorphic liposarcoma originated from the epicardium. Electron microscopically, lipid droplets were demonstrated in the cytoplasm of the tumor cells, supporting the histological diagnosis. No distant or nodal metastases were noted.
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PMID:[An extremely rare autopsy case of cardiac liposarcoma and Brenner tumor]. 400 75

Repeated observations in our laboratory show that the chloroethylnitrosourea of cysteamine ( CNCC ) induces slowed tumor growth rate and decreased lymph node metastasis in rats bearing a rhabdomyosarcoma but concomitantly enhances metastatic dissemination in the lung. Tumors obtained by sc graft of tumor cells, in syngeneic rats, gave a reproducible pattern of metastases at nodal and pulmonary sites after a 60-80-day period. CNCC was administered orally at a dose of 50 mg/kg once a week for 5 weeks beginning at the time of tumor appearance. Forty-five of 46 CNCC -treated rats had lung metastases with 95 (+/- 9.7) nodules; in the control group 29 of 41 rats had lung metastases with 7 (+/- 1.5) nodules. This amplifying effect was found after treatment with two other nitrosoureas (chlorozotocin and hydroxyethylchloroethylnitrosourea ) but not with cyclophosphamide and methotrexate. Lung metastatic amplification was also observed after treatment of the 13762 mammary adenocarcinoma in Fischer rats and treatment of nickel-induced soft tissue tumor. Several hypotheses have been proposed. The dissociated effect of nitrosourea on local tumor, lymph nodes, and pulmonary metastases does not support the concept of systemic immunosuppression as the main mechanism of this phenomenon, but a decrease of local immunological defenses exerted by NK cells, for example, could be possible. Alternatively, a direct effect of the drug on lung tissue, especially lesions of endothelial tissue, could be responsible for the observed effect. Nitrosourea treatment of rats after surgical excision of the tumor, as adjuvant chemotherapy, was responsible for an amplification effect in association with local recurrences. From this fact we hypothesized that nitrosourea treatment could modify the equilibrium of cell subpopulations in the tumor by selecting highly metastatic drug-resistant variants. Although the mechanism of the amplifying effect of nitrosoureas has not been elucidated, our study shows a possible risk in the use of these drugs for inductive or adjuvant chemotherapy.
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PMID:Amplified pulmonary metastases of a rat rhabdomyosarcoma in response to nitrosourea treatment. 623 4

A retrospective review of 112 cases treated by one author (J.C.) over the 21 year period 1953-1973 is performed. In 62% of the cases squamous cell carcinoma was the histologic diagnosis, with adenoid cystic carcinoma accounting for a further 16%. The remaining histologic types were few in number and included fibrosarcoma, muco-epidermoid carcinoma, melanoma, chondrosarcoma, adenocarcinoma, malignant Schwannoma, lymphosarcoma, malignant mixed tumor, rhabdomyosarcoma, angiosarcoma, and osteogenic sarcoma. Three years following diagnosis more than 1/3 of the squamous cell carcinomas were alive but by 10 years the survival rate had fallen to 16%. Orbital involvement at the time of presentation or noted at surgery had a poor prognosis with a 17% five year cure rate and only 2% alive at 10 years. Erosion of the pterygoids or pterygopalatine fossa invasion as diagnosed radiologically or intra-operatively revealed all patients dead of disease within five years despite radical surgery and irradiation. Cervical nodal metastases at primary presentation was a particularly ominous finding with all patients dead of disease within three years. Metastases developing later had a better prognosis with 39% surviving five years when treated aggressively.
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PMID:Carcinoma of the maxillary sinus. A correlation of the clinical course with orbital involvement, pterygoid erosion or pterygopalatine invasion and cervical metastases. 630 78

A retrospective review of all cases of rhabdomyosarcoma seen at UCLA was done. Survival prior to the advent of cyclic multidrug chemotherapy was compared to survival with this therapy. In the latter group, unlike other published series, survival was 150% better with gross total removal of disease as compared to biopsy only. All received at least 5000 rads. Fifteen percent presented with regional nodal metastases. However 75% of these also had intracranial extension and all died. An additional 19% developed late regional nodal metastases. Eighty percent of these also developed systemic metastases and all died. Survival in the absence of nodal disease was 41%. This is the first study to address the influence of regional nodal metastases, early and late on the outcome of treatment. In rhabdomyosarcoma of the head and neck, regional nodal metastases at any time implies a grave prognosis and more seriously adversely affects the progress than it does in squamous cell carcinoma in adults.
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PMID:Rhabdomyosarcoma of the head and neck. 668 42

A retrospective review of all cases of rhabdomyosarcoma seen at UCLA was done. Survival prior to the advent of cyclic multidrug chemotherapy was compared to survival with this therapy. In the latter group, unlike other published series, survival was 100% better with gross total removal of disease as compared to biopsy only. All received at least 5000 rads. Fifteen percent presented with regional nodal metastases. However 75% of these also had intracranial extension and all died. An additional 19% developed late regional nodal metastases; 80% of these also developed systemic metastases and all died. Survival in the absence of nodal disease was 41%.
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PMID:Rhabdomyosarcoma of the head and neck. 669

Seventy three children with rhabdomyosarcoma were treated by members of the Children's Solid Tumour Group during the period, 1974-1981. The extent of disease at diagnosis was found to be the major influence affecting outcome. Children with tumours confined to the tissue of origin with no evidence of nodal or metastatic spread, had a predicted actuarial 5-year survival rate of 86%. However children with 'unconfined' tumours, i.e. those with extension of disease outside the tissue of origin, had a much poorer prognosis with an actuarial 5-year survival rate of only 21%. Two other factors, histological type and site of primary tumour, appeared to affect prognosis but were not independent of the extent of disease at diagnosis. All children were treated according to protocol. Fifty-two patients showed a complete response to initial therapy and 4 of the 11 partial responders achieved a full remission after additional therapy. The overall complete response rate was therefore 77%. Nineteen children who achieved a complete response on initial treatment subsequently relapsed. Only 3 of these children were alive with no evidence of disease 3 years later, a salvage rate of 15%. "Late" relapses, defined as those occurring more than 2 years after diagnosis, were seen in only 5 children, 4 in boys with primary paratesticular tumours.
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PMID:Childhood rhabdomyosarcoma: experience of the Children's Solid Tumour Group. 688 60

Fifty-eight children with genitourinary rhabdomyosarcoma are reported. Lesions involved the bladder (22), prostate (14), vagina/uterus (6), and paratesticular tissues (16). Fifteen of 58 had positive sampling of regional lymph nodes. Eleven of 15 received radiation to no more than 4500 rad in most cases, and 9 of 11 are diseases free. Two of 15 had no radiation and are disease free also. Twenty-three of 58 had negative nodes. Six of 23 had radiation to these nodal areas and 4 of 6 are disease free. Fifteen of 17 patients had no radiation and are disease free also. Sixteen of 20 patients with no node sampling are disease free; 9 of 16 had radiation but 7 of 16 did not. All patients in the Study had intensive systemic maintenance chemotherapy. One 1 patient failed in regional nodes despite 4500 rad to these node echelons. We suggest chemotherapy and radiotherapy, not to exceed 3500 rad in four weeks, to known residual disease including regions from which positive nodes have been obtained.
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PMID:Radiation to regional nodes for rhabdomyosarcoma of the genitourinary tract in children: is it necessary? A report from the Intergroup Rhabdomyosarcoma Study No. 1 (IRS-1). 738 51


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