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Familial nervous system tumour syndromes

Written2001-01Anne Marie Capodano
Laboratoire de Cytogénétique Oncologique, Hôpital de la Timone, 264 rue Saint Pierre, 13005 Marseille, France

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Identity

Atlas_Id 10067
Genes implicated inAPC  CHEK2  MLH1  MHS6  MSH2  NF1  NF2  PMS2  PTCH1  PTCH2  PTEN  SDHB  SDHD  SUFU  TP53  TSC1  TSC2  VHL  
Inheritance eight genetic syndromes are associated with nervous system tumours; these are:
  • Neufibromatosis 1 (NF1)
  • Neufibromatosis 2 (NF2)
  • Tuberous sclerosis
  • Turcot syndrome
  • Von Hippel-Lindau syndrome
  • Li-Fraumeni syndrome
  • Gorlin syndrome
  • Cowden syndrome
    		•

    Clinics

    Neoplastic risk
  • Neufibromatosis 1 (NF1):
  • Neufibromatosis 2 (NF2):
  • Tuberous sclerosis:
    • Nervous System Tumors Astrocytomas, Subependymal giant cell tumors
    • Other tumors Cutaneous angio-fibroma, Cardiac rhabdomyomas, Adenomatous polyps of duodenum, Renal hamartomatous tumors, Cysts of the lung and kidney.
    • Genes TSC1 and TSC2 located in 9q34 and 16p13 respectively
  • Turcot syndrome:
  • Von Hippel-Lindau syndrome:
  • Li-Fraumeni syndrome:
  • Gorlin syndrome:
  • Cowden syndrome:
    • Nervous System Tumors Dysplastic gangliocytoma of the cerebellum
    • Other tumors Hamartomatous polyps of the colon, Thyroid neoplasms, Breast carcinoma
    • Genes PTEN located in 10q23

    • Bibliography

    Familial partial monosomy 7 and myelodysplasia: different parental origin of the monosomy 7 suggests action of a mutator gene.
    Minelli A, Maserati E, Giudici G, Tosi S, Olivieri C, Bonvini L, De Filippi P, Biondi A, Lo Curto F, Pasquali F, Danesino C
    Cancer genetics and cytogenetics. 2001 ; 124 (2) : 147-151.
    PMID 11172908
     

    Citation

    This paper should be referenced as such :
    Capodano, AM
    Familial nervous system tumour syndromes
    Atlas Genet Cytogenet Oncol Haematol. 2001;5(2):141-141.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Tumors/FamilNervousCanID10067.html

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