A New Form of Diffuse Familial Polyposis

nd, Carter Bn; Gw, Horsley; Js, Horsley; Hughes, Rhidian

doi:10.1097/00000658-196806000-00015

Cited by 8 publications

(3 citation statements)

References 5 publications

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“…It must be stressed that it is not easy to distinguish minute adenomata or papillomata from retention cysts in mucus glands of the small intestine, particularly in children. Carter, Horsley, Horsley, and Hughes, (1968) have recorded 4 undoubted cases of small-intestine polyposis associated with the familial colonic lesion and in I of these the polyps were seen on the protruding mucosa of the ileostomy.…”

Section: Discussionmentioning

confidence: 99%

Familial polyposis of the colon with special reference to regression of rectal polyposis after subtotal colectomy

Shepherd

1971

Journal of British Surgery

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Two families with familial polyposis of the colon are reported. One family conforms to the established pattern and in the other there is a risk of an additional inheritance of sebaceous cysts and fibromata. Initially prophylactic surgery should usually consist of a one‐stage subtotal colectomy with ileorectal anastomosis. Thereafter the polyps in the rectal remnant are left untreated for 6 months in the hope of spontaneous regression of some years' duration. This routine is preferable to immediate total colectomy and ileostomy for the adolescent or young adult. Fulguration of residual polyps is deferred but may be necessary if there is no spontaneous regression. A second‐stage excision may be required if polyposis becomes uncontrollable or if there is malignant change. A case may be made for prophylactic rectal excision about 10 years after subtotal colectomy. The technique of ileorectal anastomosis is discussed briefly and a plan for family supervision is outlined. It is suggested that the phenomenon of spontaneous regression of polyps in the rectal remnant may be more frequent than accepted at present and that a long‐term follow‐up of patients with regression is required.

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Section: Discussionmentioning

confidence: 99%

Familial polyposis of the colon with special reference to regression of rectal polyposis after subtotal colectomy

Shepherd

1971

Journal of British Surgery

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“…Although our review of the literature yielded ten other patients with FAP harboring a pancreatic tumor, detailed data were given in only a few reports (Table 1). 3,4, The histologic diagnosis in two patients was adenocarcinoma 3,6. Even in a large series of 387 patients with pancreatic carcinoma, Chen and Baithun11 found only four cases (1.0%) of acinar cell carcinoma.…”

Section: Discussionmentioning

confidence: 92%

Pancreatic acinar cell carcinoma in familial adenomatous polyposis

Ohsato

Mibu

Nagai

et al. 1996

J Hep Bil Pancr Surg

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Pancreatic carcinoma is rarely associated with familial adenomatous polyposis (FAP). Ten patients with this association have been reported in the literature. However, detailed data were available in only a few cases. The first case of pancreatic acinar cell carcinoma associated with FAP is reported. A 51-year-old man who had undergone total colectomy and ileoproctostomy for colonic polyposis 29 years previously was admitted with a cancer in the residual rectum. Preoperative examinations revealed a tumor in the head of the pancreas. Pancreaticoduodenectomy and very low anterior resection of the rectum were performed. Histologically, the tumor of the pancreas was acinar cell carcinoma. In patients with FAP, we recommend careful surveillance of not only the gastrointestinal tract but also of other organs such as those of the pancreaticobiliary system and the endocrine organs.

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“…There may have been three individuals with this combination of pathology in the family reported by Hughes and Hueston [34]. Family clustering in non-Gardner familial polyposis of the colon is found in the reports of Jones and Nance [55], Carter [56,57], and Richardson [58] (Table 11).…”

Section: Familial Predispositionmentioning

confidence: 86%

Gardner syndrome and periampullary malignancy

Pauli

Hall

et al. 1980

Am. J. Med. Genet.

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In a family with colonic polyposis and the typical associated findings of Gardner syndrome (osteomas and soft-tissue tumors), two and possibly four of the affected members developed periampullary malignancy. A review indicates that individuals with Gardner syndrome may have a 100- to 200-fold increased risk of developing periampullary carcinoma when compared to the general population. While certain families and certain individuals (those with other duodenal involvement, males and those with all of the characteristics of Gardner syndrome expressed) may be most susceptible, all patients with Gardner syndrome should be periodically endoscopically evaluated for the presence of upper gastrointestinal tract disease.

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A New Form of Diffuse Familial Polyposis

Cited by 8 publications

References 5 publications

Familial polyposis of the colon with special reference to regression of rectal polyposis after subtotal colectomy

Familial polyposis of the colon with special reference to regression of rectal polyposis after subtotal colectomy

Pancreatic acinar cell carcinoma in familial adenomatous polyposis

Gardner syndrome and periampullary malignancy

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