Rhabdomyosarcoma – working out the pathways

@article{Merlino1999RhabdomyosarcomaW,
  title={Rhabdomyosarcoma – working out the pathways},
  author={Glenn Merlino and Lee J. Helman},
  journal={Oncogene},
  year={1999},
  volume={18},
  pages={5340-5348}
}
Rhabdomyosarcomas constitute a collection of childhood malignancies thought to arise as a consequence of regulatory disruption of skeletal muscle progenitor cell growth and differentiation. Our understanding of the pathogenesis of this neoplasm has recently benefited from the study of normal and malignant myogenic cells in vitro, facilitating the identification of diagnostic cytogenetic markers and the elucidation of mechanisms by which myogenesis is regulated. It is now appreciated that the… 
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References

SHOWING 1-10 OF 132 REFERENCES
Rhabdomyosarcomas and radiation hypersensitivity in a mouse model of Gorlin syndrome
TLDR
It is shown that ptc heterozygous mice exhibit increased incidence of radiation-induced teratogenesis, which suggests a role for ptc in the response to ionizing radiation and provides a model for both the systemic and stochastic abnormalities observed in Gorlin syndrome.
Molecular differential pathology of rhabdomyosarcoma
TLDR
The underlying simplicity of the strategy used to define rhabdomyosarcoma subtypes with molecular markers suggests a model by which tumors can be unequivocally identified, which may apply equally well to other human solid tumors.
Expression of the retinoblastoma susceptibility gene in childhood rhabdomyosarcomas.
TLDR
The findings reported here clearly imply that the RB1 gene is structurally and functionally normal in childhood rhabdomyosarcoma, which is distinct from those for other adult asarcomas, in which RB1 expression frequently is reported as abnormal.
Redundancy of autocrine loops in human rhabdomyosarcoma cells: induction of differentiation by suramin.
TLDR
Rhabdomyosarcoma cells of embryonal and alveolar histotype can show a redundancy of growth-sustaining autocrine loops, and suramin could interfere with them by acting on both growth inhibition and induction of myogenic differentiation.
Allelotype of pediatric rhabdomyosarcoma
TLDR
The data suggest that genes involved in the development of RMS and WT may not only be similar for chromosome 11 but also for chromosome 16, and that LOH of chromosome 16 is also found in other tumors, including WT.
Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms.
TLDR
Germ line p53 mutations have been detected in all five LFS families analyzed and can now be examined in additional families with LFS, and in other cancer patients and families with clinical features that might be attributed to the mutation.
Germ-line transmission of a mutated p53 gene in a cancer-prone family with Li–Fraumeni syndrome
TLDR
The p53 gene in a family affected by Li–Fraumeni syndrome, a rare autosomal dominant syndrome characterized by the occurrence of diverse mesenchymal and epithelial neoplasms at multiple sites, had the same point mutation in codon 245 (GGC→GAC), which leads to substitution of aspartic acid for glycine in one of the regions identified as a frequent target of point mutations in p53.
Separate amplified regions encompassing CDK4 and MDM2 in human sarcomas
TLDR
The data suggest that amplification of at least three different regions within the 12q13–15 segment may be selected for in tumor cells involving MDM2, CDK4, or a more distally located gene, possibly near D12S8.
Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma
TLDR
The PAX3 gene alterations are associated with two completely unrelated human diseases, paediatric solid tumour alveolar rhabdomyosarcoma and Leigh's syndrome.
...
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