Tumor Suppressor Genes In Hereditary Cancers

As described in Chapter 3, several recessively inherited syndromes caused by defects in DNA repair genes are associated with an increased cancer risk, often for leukemias and lymphomas (^Table 3.2). Other 'cancer syndromes' are inherited in a dominant mode. Most of these predispose to a restricted range of cancers or even to a single tumor type, while a few are rather unselective, e.g., the rarer Li-Fraumeni and Cowden syndromes (Table 5.1).

Table 5.1. Some dominantly inherited cancer syndromes in man

Syndrome

Gene

Location

Cancer site

Function

Retinoblastoma

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