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)

An embryonal rhabdomyosarcoma was analyzed cytogenetically after short-term culturing. The tumor was located in the scrotum of an 18-year-old man who, at the age of 4, had been treated for a rhabdomyosarcoma in the orbita. The chromosome number was 100. No structural aberrations were present. There were 2-7 copies of each chromosome type with particular excess of chromosomes 8, 12, and 22, and a relative deficit of chromosome 15.
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PMID:Embryonal rhabdomyosarcoma with 100 chromosomes but no structural aberrations. 160 66

Cytogenetic and restriction fragment length polymorphism (RFLP) analyses were performed on a mediastinal germ cell tumor comprising distinct teratoma and embryonal rhabdomyosarcoma components in a 31-year-old male and a hepatoblastoma in a 2 month-old male child. Clonal relationship between the teratoma and rhabdomyosarcoma of the germ cell tumor was established by the presence in both of i(12p), the characteristic marker of germ cell tumors. Both the rhabdomyosarcoma component of the mediastinal germ cell tumor and the hepatoblastoma exhibited rearrangements of 2q. These data suggest that malignant differentiation of a teratoma is accompanied by the development of chromosome abnormalities specific for the transformed histology and further suggest that 2q abnormalities may be the common genetic link in the development of the two histologically unrelated tumor types, embryonal rhabdomyosarcoma and hepatoblastoma.
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PMID:Abnormalities of 2q: a common genetic link between rhabdomyosarcoma and hepatoblastoma? 167 6

We studied the human embryonal rhabdomyosarcoma cell line RD and 8 derivatives obtained in our laboratory either by cell cloning or by culturing in vitro cells from tumors or secondaries grown in nude mice. The expression of desmin and of the embryonic isoform of myosin and the formation of multinuclear myotube-like structures were studied as specific markers of myogenic differentiation. During continuous growth, each derivative contained a proportion (ranging from 5 to 80% among derivatives) of desmin-positive cells and a small number of myosin-positive or multinuclear elements. Cells from continuous cultures were injected intravenously in nude mice, producing lung and kidney/adrenal nodules. No correlation was found between the proportion of cells expressing desmin and metastatic capacity. When cultures were grown in differentiation medium (Dulbecco's minimal essential medium + 2% horse serum) some derivatives (designated type A) showed a strong increase in the proportion of myosin-positive cells, while others (type B) showed no increase. In vitro differentiation significantly reduced the metastatic ability of type A cells, while no modification was observed in type B after growth in differentiation medium. The proliferative ability of type A and type B cells grown in differentiation medium did not correlate with the proportion of myosin-positive cells, and extensive formation of multinuclear myotubes was never observed. It was concluded that reduction of experimental metastatic ability was mediated by events related to late, though not necessarily terminal, differentiation of rhabdomyosarcoma cells.
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PMID:Reduced metastatic ability of in vitro differentiated human rhabdomyosarcoma cells. 191 85

Soft tissue sarcomas of the paraspinal region comprised 3.3% (56 of 1,688) of the patients entered and eligible on Intergroup Rhabdomyosarcoma Studies I (IRS-I) and II (IRS-II) (1972 to 1984). These lesions tended to be greater than 5 cm in diameter at diagnosis, invaded the spinal extradural space, and were of the extraosseous Ewing's sarcoma or undifferentiated sarcoma subtype in 55% (30 of 56) of the cases. Patients with tumors in clinical groups II, III, and IV were treated with radiotherapy (XRT) and vincristine-dactinomycin (VA) or VA plus cyclophosphamide (VAC) +/- doxorubicin. Clinical group I patients treated on IRS-II did not receive XRT, while those on IRS-I were randomized to receive VAC +/- XRT. Forty-four of the paraspinal patients (79%) achieved a complete response (CR) compared with 77% (1,260 of 1,632) for patients with disease in other sites. Twenty-seven patients (55%) subsequently relapsed (five local, three regional, four local and distant, and 14 distant). The proportion of patients surviving 5 years by clinical group (stage) from I to IV were 50%, 50%, 62%, and 27%, respectively. Paraspinal patients had somewhat poorer survival than patients with disease in other sites, both in IRS-I and IRS-II; the percentage of paraspinal patients surviving 5 years was 50% and 52% for IRS-I and IRS-II, respectively, whereas these percentages were 55% and 63% for patients with disease in other sites. Histology did not influence the CR rate, but unexpectedly, patients who had embryonal rhabdomyosarcoma (RMS) had the poorest overall survival rate. We concluded that patients with paraspinal lesions may require extended-field radiation therapy to reduce the high local failure rate and more intensive chemotherapy to achieve better local and systemic tumor control.
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PMID:Clinical features and results of therapy for children with paraspinal soft tissue sarcoma: a report of the Intergroup Rhabdomyosarcoma Study. 201 22

Embryonal rhabdomyosarcoma of orbit presenting as a case of rapid proptosis of the right eye is reported in a 4 year old male child. There was no evidence of recurrence during a follow up of 6 months. Rhabdomyosarcoma is one of the most common primary malignant orbital neoplasms of child hood. It usually produces a precipitously progressing unilateral proptosis of sudden onset. It is a highly malignant neoplasm of pleuripotential embryonic mesoderm, which commonly differentiates to form cells similar to rhabdomyoblasts of the foetus. Because of the presence of elongated cells that contain abundance of eosinophilic glycogen rich cytoplasm, it is generally referred to as embryonal form of rhabdomyosarcoma.
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PMID:Embryonal rhabdomyosarcoma of orbit--a case report. 208 80

Embryonal rhabdomyosarcoma of the vagina in adults per se is uncommon and pregnancy complicated by such tumors is extremely rare. We wish to report one case of vaginal embryonal rhabdomyosarcoma, diagnosed at the age of 23 years who was carrying at the time of initial presentation. The problem of management of rhabdomyosarcoma in adults, particularly when they are associated with pregnancy is discussed. We advocate the use of multiagent chemotherapy and pelvic radiotherapy in its overall management.
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PMID:Adult embryonal rhabdomyosarcoma of the vagina complicating pregnancy: a case report and review of the literature. 209 25

The effect of 12-O-tetradecanoyl phorbol-13-acetate (TPA) on proliferation and differentiation of the human embryonal rhabdomyosarcoma cell line RD was investigated. The proliferation of RD cells is drastically and reversibly inhibited by 100 nM TPA. The effect is evident after 24 h of treatment and is maximal after 50-70 h. The reduction of proliferation in treated cells is followed by increased expression of differentiative characters such as a large increase in muscle myosin expression and in the binding of 125I-alpha-bungarotoxin. Moreover TPA induces the appearance of myotube-like structures, which contain bundles of thick and thin myofilaments along with Z bodies. The described effects are not observed if the TPA-containing medium is replaced daily, thus suggesting that these effects might be related to substances secreted by treated cells. The phosphorylation of three proteins is significantly stimulated by TPA within minutes of its administration to RD cells. Although with a different pattern, the stimulation of protein phosphorylation is still clearly detectable after 6 days of incubation with TPA. These results on human rhabdomyosarcoma cells are, to our knowledge, the first evidence for a growth-inhibiting and a differentiative effect of TPA on a solid tumor of mesodermal origin.
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PMID:12-O-tetradecanoylphorbol-13-acetate-induced differentiation of a human rhabdomyosarcoma cell line. 215 81

Pure rhabdomyosarcomas of the female genital tract most commonly occur in infancy or childhood as sarcoma botryoides (embryonal rhabdomyosarcoma) and involve the vagina and cervix. Such tumors rarely occur in adults. A pure rhabdomyosarcoma of the uterus that arose in a postmenopausal patient is described. The pertinent literature is discussed.
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PMID:Rhabdomyosarcoma of the uterus in a postmenopausal patient. 219 Aug 75

This is a case report of embryonal rhabdomyosarcoma of the epididymal appendage in a 15-month old infant. It is a rare tumor with a good prognosis when treated promptly. The ultrasound examination showed the testicle to be enlarged but hypo-echoic in comparison to the epididymis which was infiltrated with tumor. An inflammatory mass or enlargement of a testicle demands careful diagnosis and appropriate therapy with immediate referral to a urologist if the normal clinical course deviates. Representative images are included. Rhabdomyosarcoma of the testicle or extra testicular scrotal structures is rare. A report in 1983 by Kage et al from Japan revealed two cases of the tumor, however, both were in adult males. This case report is an embryonal rhabdomyosarcoma in a fifteen month old infant which presented with a confusing clinical picture. This case is presented in an effort to alert the clinician to the possibility of a rare, non-germ cell tumor presenting as an inflammatory lesion.
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PMID:Rhabdomyosarcoma of the testicular appendage in an infant: case report. 219 21

We report an unusual case of primary cutaneous embryonal rhabdomyosarcoma presenting as a solitary skin lesion on the anterior chest of a 20-month-old child. The tumor was characterized by small, round to oval, poorly differentiated cells. Immunohistochemically, the tumor was negative for NSE, S-100 protein, LCA, and keratin but positive for muscle-specific actin, myoglobin, desmin, and vimentin, thus indicating the presence of myogenous differentiation. Ultrastructural analysis demonstrated thick and thin filaments. Special studies showed no evidence of a primary rhabdomyosarcoma in the patient at a more typical location, nor was there any evidence of metastases.
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PMID:Primary cutaneous rhabdomyosarcoma. 220 84


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