The occurrence of desmoids in patients with familial polyposis coli

Abstract

Four cases of desmoid tumour are reported in conjunction with familial polyposis coli, and the salient clinical features of patients with this combination of conditions are examined in the literature and tabulated. Desmoids occur very rarely in the general population but they are comparatively common in people with familial polyposis, in whom they may be situated in the abdominal wall, within the abdomen, or occasionally in other sites remote from the abdomen. They may antedate the discovery of polyposis by months or years. Any patient who is found to have a desmoid should therefore be examined for polyposis coli. Intra-abdominal desmoids by virtue of their size and situation are often of serious significance to the patient, and their effects may belie the benign histological picture they present. Renal function may be impaired by pressure on the ureters, whilst surgical removal of the lesion, which is the principal method of treatment of desmoids, may be crippling to the patient or technically impossible. Radiotherapy seems to be of limited value in the management of these tumours. No mass occurring within the abdomen or in the abdominal wall after surgery for polyposis coli should be regarded as a recurrent adenocarcinoma until proved to be so by adequate biopsy.