Colon cancer genetics / edited by Patrick M. Lynch and Henry T. Lynch.

Date:
[1985], ©1985
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Licence: Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)

Credit: Colon cancer genetics / edited by Patrick M. Lynch and Henry T. Lynch. Source: Wellcome Collection.

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    Clinical Management of Hereditary Colon Cancer William A. Albano, M.D. and Henry T. Lynch, M.D. Hereditary colon cancer (HCC) is heterogeneous, encompassing a variety of distinct genetic entities. These entities are conveniently divided into two broad categories: syndromes where cancer is preceded by multiple polyps (polyposis syndromes) and syndromes without a polyp manifestation (nonpolyposis syndromes.) The physician's responsibility begins with the recognition that HCC syn¬ dromes are not uncommon in clinical practice.' The suspicion of a potential hereditary syndrome is made on the basis of the modified nuclear family history (see Modified Nuclear Pedigrees), which can be easily performed on a patient's initial contact.^ The elucidation of specific historical findings, that is, the cardinal features of HCC (early onset, multiple primary-cancer excess, vertical transmission, proximal colon cancer predominence, or a history of polyposis) within the modified nuclear family history, provide the nidus for such association. In these situations, consultation with knowledge¬ able specialists may allow the clinician to institute specific management protocols for already-affected persons and their high-risk, unaffected family members. MODIFIED NUCLEAR PEDIGREES A nuclear pedigree encompasses information regarding the proband's parents, siblings, and children. However, because of the young age of most cancer- affected probands, children are rarely old enough for cancer to be manifested, decreasing the efficacy of the nuclear pedigree as a screening test. To circumvent these problems, the modified nuclear pedigree, as we define it, is expanded to include grandparents, aunts and uncles (Fig. 5-1). Such a pedigree is easily compiled by either the attending physician or office personnel on the initial contact. It must be stressed that the key is to ask specific questions and record both pertinent positive and negative medical 99