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Li-Fraumeni Syndrome
Definition: Rare autosomal dominant syndrome characterized by mesenchymal and epithelial neoplasms at multiple sites. MUTATION of the p53 tumor suppressor gene, a component of the DNA DAMAGE response pathway, apparently predisposes family members who inherit it to develop certain cancers. The spectrum of cancers in the syndrome was shown to include, in addition to BREAST CANCER and soft tissue sarcomas (SARCOMA); BRAIN TUMORS; OSTEOSARCOMA; LEUKEMIA; and ADRENOCORTICAL CARCINOMA.

Synonym(s): Li Fraumeni Syndrome /
See also:
Genes, p53   

MeSH 2010 © U.S. National Library of Medicine®.
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  More information from the Genetics Home Reference Website
What is Li-Fraumeni syndrome?
Li-Fraumeni syndrome is a rare disorder that greatly increases the risk of developing several types of cancer, particularly in children and young adults.
The cancers most often associated with Li-Fraumeni syndrome include breast cancer, a form of bone cancer called osteosarcoma, and cancers of soft tissues (such as muscle) called soft tissue sarcomas. Other cancers commonly seen in this syndrome include brain tumors, cancers of blood-forming tissues (leukemias), and a cancer called adrenocortical carcinoma that affects the outer layer of the adrenal glands (sma ...

How common is Li-Fraumeni syndrome?
The exact prevalence of Li-Fraumeni is unknown. One U.S. registry of Li-Fraumeni syndrome patients suggests that about 400 people from 64 families have this disorder.


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  Web resources for "Li-Fraumeni Syndrome"
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view the HON description of the site HONcode - Li-Fraumeni syndrome - Genetics Home Referenceghr.nlm.nih.gov 3
view the robot description of the site Whonamedit - Li-Fraumeni syndromewww.whonamedit.com  
view the HON description of the site HONcode - Orphanet: Li Fraumeni syndromewww.orpha.net 2
 
 

See also: - Genes, p53

Broader term(s): - Neoplastic Syndromes, Hereditary - Neoplasms - Metabolic Diseases - Nutritional and Metabolic Diseases
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  Medical image(s) for "Li-Fraumeni Syndrome"
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Broader term(s): - Neoplasms
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  Medical News for "Li-Fraumeni Syndrome"
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Broader term(s): - Neoplasms

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  Scientific articles from MEDLINE for "Li-Fraumeni Syndrome"
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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy

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  Clinical Trials for "Li-Fraumeni Syndrome"
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See also: - Genes, p53

Broader term(s): - Neoplasms - Metabolic Diseases

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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy


  Broader term(s):
      Diseases
          Nutritional and Metabolic Diseases
              Metabolic Diseases
                  DNA Repair-Deficiency Disorders 
      Diseases
          Congenital, Hereditary, and Neonatal Diseases and Abnormalities
              Genetic Diseases, Inborn
                  Neoplastic Syndromes, Hereditary 
      Diseases
          Neoplasms
              Neoplastic Syndromes, Hereditary 

MeSH 2010 © U.S. National Library of Medicine®.

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