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Management of extracolonic tumours in patients with Lynch syndrome.

Abstract

Hereditary nonpolyposis colorectal cancer, or Lynch syndrome, is responsible for 2-3% of all colorectal cancers. Lynch syndrome is also associated with a high risk of extracolonic cancers, including endometrial, stomach, small bowel, pancreas, biliary tract, ovary, urinary tract, brain, and skin cancer. In this Review, we discuss the risks, surveillance tests, and guidelines for the management of extracolonic tumours associated with Lynch syndrome. For all types of extracolonic cancer, evidence supporting surveillance is scarce. A benefit of surveillance is evident only for endometrial cancer, where transvaginal ultrasound and endometrial sampling detect tumours in early stages. Surveillance is generally recommended for urinary tract and gastric cancer, especially in families with more than one member with these types of cancer. For the other types of cancer, surveillance is typically not recommended. Prophylactic hysterectomy and bilateral salpingo-oophorectomy should be considered for women with Lynch syndrome who are past childbearing age, especially during surgery for colorectal cancer. No data show efficacy of chemopreventive drugs in reducing the risk of extracolonic cancers for patients with Lynch syndrome.

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  • Authors+Show Affiliations

    ,

    Department of Gastroenterology and Hepatology, University Medical Centre Groningen, University of Groningen, Groningen, the Netherlands.

    , , , ,

    Source

    The Lancet. Oncology 10:4 2009 Apr pg 400-8

    MeSH

    Colorectal Neoplasms, Hereditary Nonpolyposis
    Female
    Humans
    Male
    Neoplasms
    Pedigree

    Pub Type(s)

    Journal Article
    Review

    Language

    eng

    PubMed ID

    19341971

    Citation

    Koornstra, Jan J., et al. "Management of Extracolonic Tumours in Patients With Lynch Syndrome." The Lancet. Oncology, vol. 10, no. 4, 2009, pp. 400-8.
    Koornstra JJ, Mourits MJ, Sijmons RH, et al. Management of extracolonic tumours in patients with Lynch syndrome. Lancet Oncol. 2009;10(4):400-8.
    Koornstra, J. J., Mourits, M. J., Sijmons, R. H., Leliveld, A. M., Hollema, H., & Kleibeuker, J. H. (2009). Management of extracolonic tumours in patients with Lynch syndrome. The Lancet. Oncology, 10(4), pp. 400-8. doi:10.1016/S1470-2045(09)70041-5.
    Koornstra JJ, et al. Management of Extracolonic Tumours in Patients With Lynch Syndrome. Lancet Oncol. 2009;10(4):400-8. PubMed PMID: 19341971.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Management of extracolonic tumours in patients with Lynch syndrome. AU - Koornstra,Jan J, AU - Mourits,Marian Je, AU - Sijmons,Rolf H, AU - Leliveld,Annemarie M, AU - Hollema,Harry, AU - Kleibeuker,Jan H, PY - 2009/4/4/entrez PY - 2009/4/4/pubmed PY - 2009/4/22/medline SP - 400 EP - 8 JF - The Lancet. Oncology JO - Lancet Oncol. VL - 10 IS - 4 N2 - Hereditary nonpolyposis colorectal cancer, or Lynch syndrome, is responsible for 2-3% of all colorectal cancers. Lynch syndrome is also associated with a high risk of extracolonic cancers, including endometrial, stomach, small bowel, pancreas, biliary tract, ovary, urinary tract, brain, and skin cancer. In this Review, we discuss the risks, surveillance tests, and guidelines for the management of extracolonic tumours associated with Lynch syndrome. For all types of extracolonic cancer, evidence supporting surveillance is scarce. A benefit of surveillance is evident only for endometrial cancer, where transvaginal ultrasound and endometrial sampling detect tumours in early stages. Surveillance is generally recommended for urinary tract and gastric cancer, especially in families with more than one member with these types of cancer. For the other types of cancer, surveillance is typically not recommended. Prophylactic hysterectomy and bilateral salpingo-oophorectomy should be considered for women with Lynch syndrome who are past childbearing age, especially during surgery for colorectal cancer. No data show efficacy of chemopreventive drugs in reducing the risk of extracolonic cancers for patients with Lynch syndrome. SN - 1474-5488 UR - https://www.unboundmedicine.com/medline/citation/19341971/Management_of_extracolonic_tumours_in_patients_with_Lynch_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1470-2045(09)70041-5 DB - PRIME DP - Unbound Medicine ER -