HONselect PATIENT / INDIVIDUAL MEDICAL PROFESSIONAL WEB PUBLISHER

Hierarchy  - English - French - German - Spanish - Portuguese - Italian - Dutch    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®. Browse - New search

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. More information from the Genetics Home Reference Website © Genetics Home Reference Browse - New search       Web resources for "Li-Fraumeni Syndrome"   English (5)  French (3)  German (2)         = Site with HON description   -   = Site with a robot description info: enter the site: (click below) domain of the site:   HONcode - Li-Fraumeni syndrome - Genetics Home Reference ghr.nlm.nih.gov 3 Whonamedit - Li-Fraumeni syndrome www.whonamedit.com   HONcode - Orphanet: Li Fraumeni syndrome www.orpha.net 2 See also: - Genes, p53 Broader term(s): - Neoplastic Syndromes, Hereditary - Neoplasms - Metabolic Diseases - Nutritional and Metabolic Diseases Browse - New search       Medical image(s) for "Li-Fraumeni Syndrome" No results for this term. Browse other medical images in HONmedia? Broader term(s): - Neoplasms Browse - New search       Medical News for "Li-Fraumeni Syndrome" No results for this term. More health news Broader term(s): - Neoplasms Browse - New search       Scientific articles from MEDLINE for "Li-Fraumeni Syndrome"   - English - French - German - Spanish - Portuguese - Italian - Dutch     All recent articles  Therapy:  by recall    /  by precision  Diagnosis:  by recall    /  by precision  Etiology:  by recall    /  by precision  Prognosis:  by recall    /  by precision Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy Browse - New search       Clinical Trials for "Li-Fraumeni Syndrome" ClinicalTrials.gov - Linking patients to medical research Role of p53 Gene in Metabolism Regulation in Patients With Li-Fraumeni Syndrome p53 Gene Therapy for Head and Neck Malignant Tumors in Advanced Stage rAd-p53 Gene Therapy for Advanced Malignant Thyroid Tumors rAd-p53 Gene Therapy for Advanced Oral and Maxillofacial Malignant Tumors Wild Type p53 Adenovirus for Oral Premalignancies Biomarker (p53 Gene) Analysis and Combination Chemotherapy Followed by Radiation Therapy and Surgery in Treating Women With Large Operable or Locally Advanced or Inflammatory Breast Cancer   More results See also: - Genes, p53 Broader term(s): - Neoplasms - Metabolic Diseases Browse - New search       Medical Conferences/Events for "Li-Fraumeni Syndrome" No results for this term. Consult HON's world-wide database of medical meetings Browse - New search       Refine the search for  "Li-Fraumeni Syndrome" 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®. Browse - New search

Most frequently used terms List of Rare Disease Add HONselect Extract MeSH Home About us MediaCorner HON newsletter Site map Ethical policies Contact /HONselect/RareDiseases/EN/C04.700.600.html last modified: May 1 2013 © copyright HON 2013