Histologic Characteristics and Outcome of Familial Non-Polyposis Colorectal Carcinoma

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Abstract
Familial cases of non-polyposis colorectal cancer have recently attracted much interest. Little is known about the characteristic histology or natural history of disease in such cases. Our aim was to determine, through a population-based study, whether mucin-secreting tumours were associated with a positive family history and whether 'familiality' was an independent prognostic variable. All patients under 55 years of age with histologically verified colorectal cancer in Northern Ireland during 1976-78 were studied. The family history was validated in 95% of all non-polyposis cases (n = 205), and the proband's histologic specimen reviewed in over 99%. Mucin-secreting tumours were significantly associated with a positive family history, but familiality was not found predictive of survival in a multivariate analysis controlling for age, sex, stage, site, symptom duration, differentiation, and histologic type.