Familial polyposis coli is a heterogeneous disease with a broad spectrum of clinical manifestations including not only multiple polyposis of the small bowel, but also multiple primary tumours, such as carcinoma of the ampulla of Vater, subcutaneous tumours, bone tumours, central nervous system tumous and gynaecological malignancies. This report is of two brothers with familial polyposis, each showing peculiar distinctive features. In one case, polyposis was diagnosed during emergency surgery for ileo-colic intussusception. The patient later developed a tumour of the uterine cervix. Polyposis coli was identified late in the second patient who showed an evolution towards colonic adenocarcinoma with multiple hepatic metastases. The possible association of familial polyposis and extracolonic malignancies has already been emphasized in the literature. In this report we wish to stress the advisability of periodic gynaecological check-ups in affected patients.
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