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 oncogenic effects of radiation are well-established. Osteosarcomas and fibrosarcomas are the two most common histologic types of secondary sarcoma. In this article a case of postradiation osteosarcoma is presented in which four discrete foci of sarcomatous transformation have occurred in the tibia and fibula after irradiation for a rhabdomyosarcoma of the calf 8 years earlier. A review of the literature reveals no similar case. Although synchronous, multifocal osteosarcoma without prior radiation has been described, this case differs in clinical, radiographic, and pathologic features; it best fits the description of postradiation multicentric osteosarcoma.
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PMID:Postradiation multicentric osteosarcoma. 316 32

This paper describes a model for the response of the volume of a tumor to a single treatment or a series of identical treatment fractions. The model is built on three assumptions: a fixed proportional cell killing for each treatment fraction, a constant removal rate of dead cells and a constant growth rate of the remaining clonogenic cells. For each individual tumor these parameters may be estimated from sequential tumor volume measurements. The model is applied to volume measurements of 173 rhabdomyosarcoma tumors implanted in the flanks of female rats after single or fractionated radiation with total doses between 0 and 90 Gy, with or without hyperthermia. Although the assumptions on which the model is built are evidently too strong, the model serves as an acceptable first-order approximation. A comparison between fitted and measured volumes showed the best fits for single radiation treatments in the period between treatment and regrowth to about the original volume. The overall shape of the fitted and observed growth curves of tumors exposed to fractionated radiation or combined treatment with hyperthermia were similar but, especially in the period of volume reduction, the fit was moderate. The model offers the opportunity for an alternative computation of growth delay and other parameters for the effect of treatment, and for the efficient planning of experiments based on the treatment of a few animals.
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PMID:Modeling of sequentially measured volumes of rat tumors treated with single- or multiple-fraction radiotherapy, with or without hyperthermia. 801 12

A 56-year-old male suffered from primary rhabdomyosarcoma on the left internal thoracic wall, which was treated by chemotherapy, and local irradiation following biopsy. Four months after the diagnosis, he suddenly complained of headache and left paresthesia occurred followed by generalized convulsion and left hemiplegia. CT scan revealed a high density mass in the right parietal lobe. The patient was referred to our department and underwent emergency evacuation of the hematoma together with tumor removal. The pathological specimen showed spindle or oval-like cells with hypercellularity and some mitotic figures. Immunohistochemical study demonstrated that many cells were positive for desmin and myoglobin, which is specific to myogenic tumor. These characteristics were compatible with those of the primary thoracic lesion, and a diagnosis of metastatic rhabdomyosarcoma was made. Five weeks after the craniotomy, the metastatic brain tumor recurred in the same site and also in the bilateral occipital lobes. Although radiotherapy to the brain decreased the tumor size, the patient died of respiratory failure eleven and a half months after the initial diagnosis. Sarcomas metastasizing to the brain are rare and only 16 cases of rhabdomyosarcoma metastasizing to the brain have been reported so far. Recent advances in chemotherapy, however, have been able to show the increased incidence of sarcoma metastasis to the brain. Therefore, the necessity of follow-up CT scan for sarcoma patients of long survival is to be stressed, even if the patient shows no neurological symptoms.
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PMID:[Brain metastasis of rhabdomyosarcoma with intratumorous hemorrhage: a case report and literature review]. 825 24

We conducted a prospective pilot study to assess the feasibility and safety of high-dose busulfan/melphalan as conditioning therapy prior to autologous PBPC transplantation in pediatric patients with high-risk solid tumors. From January 1995 to January 1999, 30 patients aged 2-21 years (median 8) were entered into the study. There were 14 females and 16 males. Diagnoses included neuroblastoma in 10 patients; Ewing's sarcoma and peripheral neuroectodermal tumor (PNET) in 15 patients and rhabdomyosarcoma in five patients. Treatment consisted of busulfan 16 mg/kg, orally over 4 days (from days -5 to -2) in 6 hourly divided doses, and melphalan at a dose of 140 mg/m2 given by intravenous infusion over 5 min on day -1. G-CSF mobilized PBPC were used as autologous stem-cell rescue. One patient developed a single generalized convulsion during busulfan therapy. The most relevant non-hematologic toxicity was gastrointestinal, manifesting as grade 2-3 mucositis and diarrhea in 12 patients. Two patients died of procedure-related complications, one from veno-occlusive disease of liver and multiorgan failure and the other from adult respiratory distress syndrome. Probability of treatment-related mortality was 6.6 +/- 4.5%. With a median follow-up of 18 months (range, 1-48), 19 patients are alive and disease-free, the actuarial EFS at 4 years being 55 +/- 12% for the whole group. We conclude that high-dose busulfan/melphalan for autologous transplantation in children with solid tumors is feasible even in small patients. It is well-tolerated, with an acceptable transplant-related mortality and has proven antitumor activity.
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PMID:High-dose busulfan/melphalan as conditioning for autologous PBPC transplantation in pediatric patients with solid tumors. 1064 2

The transcription regulatory protein PAX3 binds to cognate DNA sequences through two DNA-binding domains, a paired domain and a homeodomain, and has important functions during neurogenesis and myogenesis. In humans, mutations in the PAX3 gene cause Waardenburg syndrome, whereas a chromosomal translocation that generates a PAX3-FOXO1 fusion gene is associated with the development of alveolar rhabdomyosarcoma. We have determined the crystal structure of the human PAX3 homeodomain in complex with a palindromic DNA containing two inverted TAATC sequences at 1.95 A resolution. Two homeodomains bind to DNA as a symmetric dimer, inducing a 3 degrees bend in the DNA helix. The N-terminal arm of the homeodomain inserts into the minor groove and makes direct and water-mediated interactions with bases and the sugar-phosphate backbone. The recognition helix fits directly into the major groove, and an elaborate network of structurally conserved water molecules mediates the majority of protein-DNA interactions. The structure elucidates the role of serine 50 in selection of the CG sequence immediately 3' of the TAAT motif by PAX class homeodomains and provides insights into the molecular mechanisms by which certain Waardenburg syndrome-associated missense mutations could destabilize the fold of the PAX3 homeodomain whereas others could affect its interaction with DNA.
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PMID:Structural basis for DNA recognition by the human PAX3 homeodomain. 1919 74