Multiple Polypoid Disease of the Colon and Rectum

Familial polyposis coli associated with extracolonic abnormalities

“…Fibromata may be found superficially (Devic and Bussy, 1912;Gardner and Richards, 1953) or in the mesentery of the small or large intestine (Pugh and Nesselrod, 1945;O'Brien and Wels, 1955;Gumpel and Carballo, 1956;Shepherd, 1958). Fibromata may also be encountered in or near the scar after colectomy (O'Brien and Wels, 1955;Shepherd, 1958).…”

Abdominal desmoid masses in Gardner's syndrome

“…Miller and Sweet [6], in 1937, and Pugh and Nesselrod [7], in 1945, each described a patient with diffuse colonic polyposis, who had developed an abdom- [8].…”

The occurrence of desmoids in patients with familial polyposis coli

“…As growth continues, they may reach a very large size (17 kg., Rokitansky, 1880) and they may infiltrate the tissues from which they arise so widely that they may become inoperable. On rare occasions abdominal wall desmoids extend through the peritoneum to involve the underlying bowel (Moore, Rowe, and King, 1950;Strode, 1954;Mock, 1959;Rivkin and Kaliteevsky, 1967), but they never invade the skin. Although voluntary muscle is infiltrated, desmoids do not usually invade bloodvessels, nerves, or joint capsules (Gerstman and Nimberg, 1969), but occasionally they become so fixed to periosteum that bone has to be excised to free the tumour (Cohen and Goldenberg, 1965;Cole and Guiss, 1969).…”

“…Intra-abdominal desmoids are important in so far as they may interfere with the colectomy which is being undertaken to cure the polyposis (O' Brien and Wels, 1955;Nora and Verduron, 1961;Simpson and others, 1964;Rivkin and Kaliteevsky, 1967)~ although desmoids arising in the mesentery tend not to invade the wall of the bowel. They may also inconvenience the patient (our Case 3, and Smith's Case 2, 1958)~ or by their effect on retroperitoneal structures may cause his death (Case 2 above, and Zuidema and Elte, 1941;Gardner, 1962;Simpson and others, 1964).…”

“…Among the tumours of this kind described by him and his colleagues (Gardner, 1951;Gardner and Plenk, 1952;Gardner and Richards, 1953) were osteomas, fibromas, and epidermal or sebaceous cysts. This combination of ectodermal or mesodermal tumours, or both, with familial polyposis coli is now generally known as ' Gardner's syndrome ' , and it is probable that approximately 10 per cent of individuals with polyposis manifest this syndrome (Waugh, Hays, and Spencer, 1964;Rivkin and Kaliteevsky, 1967). Another type of connective-tissue lesionoccasionally found in association with familial polyposis coli is desmoid tumour of the abdomen.…”