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The Lynch syndromes

H T Lynch1, J F Lynch

  • 1Department of Preventive Medicine, Creighton University School of Medicine, Omaha, NE 68178.

Current Opinion in Oncology
|July 1, 1993
PubMed
Summary
This summary is machine-generated.

Lynch syndromes, or hereditary nonpolyposis colorectal cancer, require careful monitoring due to their genetic predisposition to early-onset colorectal and extracolonic cancers. Understanding their natural history is key for effective surveillance and management strategies.

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Area of Science:

  • Oncology
  • Genetics
  • Gastroenterology

Background:

  • Lynch syndromes, also known as hereditary nonpolyposis colorectal cancer (HNPCC), are inherited cancer predispositions.
  • These syndromes significantly increase the risk of early-onset colorectal cancer and extracolonic malignancies.

Purpose of the Study:

  • To review the natural history, diagnosis, surveillance, and management of Lynch syndromes.
  • To highlight the importance of understanding disease progression for patient care.

Main Methods:

  • Literature review focusing on hereditary nonpolyposis colorectal cancer.
  • Analysis of clinical features, inheritance patterns, and associated cancer risks.

Main Results:

  • Lynch syndrome I: Autosomal dominant predisposition to early-onset colorectal cancer, primarily in the proximal colon, with high rates of synchronous and metachronous tumors.

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  • Lynch syndrome II: Includes features of Lynch syndrome I plus an increased risk of extracolonic cancers, notably endometrial and ovarian carcinomas.
  • Absence of physical signs or biomarkers necessitates reliance on natural history for risk identification.

    • Conclusions:

    • The natural history of Lynch syndromes is critical for effective surveillance and management.
    • Early diagnosis and proactive management are essential due to the high risk of multiple and early-onset cancers.