Duodenal adenomatosis in familial adenomatous polyposis

Bülow, S.; Björk, J.; Christensen, I. J.; Fousa, O.; Järvinen, H.; Moesgaard, F.; Vasen, H. F. A.
March 2004
Gut;Mar2004, Vol. 53 Issue 3, p381
Academic Journal
Background: The prevalence of duodenal carcinoma is much higher in familial adenomatous polyposis (FAP) than in the background population, and duodenal adenomatosis is found in most polyposis patients. Aims: To describe the long term natural history of duodenal adenomatosis in FAP and evaluate if cancer prophylactic surveillance of the duodenum is indicated. Methods: A prospective five nation study was carried out in the Nordic countries and the Netherlands. Patients: A total of 368 patients were examined by gastroduodenoscopy at two year intervals during the period 1990-2001. Results: At the first endoscopy, 238 (65%) patients had duodenal adenomas at a median age of 38 years. Median follow up was 7.6 years. The cumulative incidence of adenomatosis at age 70 years was 90% (95% confidence interval (CI) 79-100%), and of Spigelman stage IV 52% (95% CI 28-76%). The probability of an advanced Spigelman score increased during the study period (p<0.0001) due to an increasing number and size of adenomas. Two patients had asymptomatic duodenal carcinoma at their first endoscopy while four developed carcinoma during the study at a median age of 52 years (range 26- 58). The cumulative incidence rate of cancer was 4.5% at age 57 years (95% CI 0.1-8.9%) and the risk was higher in patients with Spigelman stage IV at their first endoscopy than in those with stages 0-III (p<0.01). Conclusions: The natural course of duodenal adenomatosis has now been described in detail. The high incidence and increasing severity of duodenal adenomatosis with age justifies prophylactic examination, and a programme is presented for upper gastrointestinal endoscopic surveillance.


Related Articles

  • Clinical characteristics of familial adenomatous polyposis and management of duodenal adenomas Morpurgo, Emilio; Vitale, Gary C.; Galandiuk, Susan; Kimberling, Jennifer; Ziegler, Craig; Polk Jr., Hiram C.; Polk, Hiram C Jr // Journal of Gastrointestinal Surgery;Jul2004, Vol. 8 Issue 5, p559 

    The aim of this study was to evaluate the clinical characteristics of patients with familial adenomatous polyposis (FAP) undergoing surgical treatment over a 10-year period and specifically to evaluate the incidence and clinical outcome of patients treated for duodenal adenomas. Patients with...

  • Intraduodenal sarcoma recurrence of retroperitoneal origin: an unusual cause for a duodenal obstruction. Bao, Jean J.; Mansour, John C.; Timmerman, Robert D.; Kirane, Amanda; Ewing, Gene E.; Schwarz, Roderich E. // World Journal of Surgical Oncology;2012, Vol. 10 Issue 1, p59 

    Soft tissue sarcomas are uncommon tumors, and intraduodenal soft tissue sarcoma manifestation is even more rare. Only three cases of intraduodenal sarcomas have been reported in the literature thus far. Here, we report a case of an intraduodenal recurrence of a retroperitoneal sarcoma causing...

  • Duodenal Cancer Arising From the Remaining Duodenum After Pylorus-Preserving Pancreatoduodenectomy for Ampullary Cancer in Familial Adenomatous Polyposis Murakami, Yoshiaki; Uemura, Kenichiro; Sasaki, Masaru; Morifuji, Masahiko; Hayashidani, Yasuo; Sudo, Takeshi; Sueda, Taijiro // Journal of Gastrointestinal Surgery;Mar2005, Vol. 9 Issue 3, p389 

    We herein report a rare occurrence of duodenal cancer arising from the remaining duodenum after pylorus-preserving pancreatoduodenectomy for ampullary cancer in familial adenomatous polyposis (FAP). In this patient, proctocolectomy and ileoanal anastomosis for FAP had been performed 11 years...

  • Malignant Change in a Duodenal Adenoma in Familial Adenomatous Polyposis. Nakatsubo, N.; Kashiwagi, H.; Okumura, M.; Kamoshida, T.; Takahashi, A.; Spigelman, A. D. // American Journal of Gastroenterology;Sep1998, Vol. 93 Issue 9, p1566 

    Patients with FAP (familial adenomatous polyposis) are known to be at high risk for duodenal cancer. Although the adenoma-carcinoma sequence is thought to exist in the duodenum, clinical observation of the development of duodenal adenoma to cancer has rarely been reported. We outline a 44-yr-old...

  • Extracolonic polyposis in familial adenomatous polyposis: so near and yet so far. Spigelman, A. D. // Gut;Mar2004, Vol. 53 Issue 3, p322 

    Extracolonic polyps in familial adenomatous polyposis were described in the stomach by Hauser in 1895. There were huge differences reported across the world in the observed proportion of polyposis patients who were affected by duodenal polyposis in the 1970s and 1980s. Those from Japan found...

  • BENIGN POLYPS OF THE DUODENUM. McQuitty, James T.; Levy, Morris // American Journal of Gastroenterology;Jul1959, Vol. 32 Issue 1, p59 

    In summary, ten patients were found at Touro Infirmary to have benign duodenal polyps during the ten-year period between January, 1947 to January, 1958. A brief review of embryology and histology was given. Because of lack of stasis and the alkalinity of the duodenum tumors may be less common in...

  • Evaluating causes of death in familial adenomatous polyposis. Campos, Fábio; Perez, Rodrigo; Imperiale, Antônio; Seid, Víctor; Nahas, Sérgio; Cecconello, Ivan; de Campos, Fábio Guilherme C M; Perez, Rodrigo Oliva; Imperiale, Antônio Rocco; Seid, Víctor Edmond; Nahas, Sérgio Carlos // Journal of Gastrointestinal Surgery;Dec2010, Vol. 14 Issue 12, p1943 

    Background: Familial adenomatous polyposis is a genetic syndrome associated with an increased risk of colorectal cancer (CRC) and different extracolonic manifestations.Goals: The goal of this study is to evaluate the frequency of death causes.Material and Methods:...

  • Clinical implications of unusual NeuroD and mASH1 expression in a patient with primary large-cell neuroendocrine carcinoma of the duodenum: Report of a case. Seiki Miura; Hiroyuki Yoshidome; Takashi Shida; Fumio Kimura; Hiroaki Shimizu; Masayuki Otsuka; Atsushi Kato; Hideyuki Yoshitomi; Satoshi Nozawa; Katsunori Furukawa; Noboru Mitsuhashi; Dan Takeuchi; Masaru Miyazaki // Surgery Today;Sep2008, Vol. 38 Issue 9, p857 

    Abstract  A 69-year-old woman was referred to our hospital for investigation of tarry stools. Abdominal computed tomography (CT) showed a slightly enhanced heterogeneous mass in the first to second part of the duodenum, adjacent to the gallbladder and head of the pancreas. Endoscopy showed...

  • Primary Duodenal Carcinoid. Wengrower, Dov; Fich, Alexander // American Journal of Gastroenterology;Oct1987, Vol. 82 Issue 10, p1069 

    Duodenal carcinoid tumors are rare. We report two patients with isolated duodenal carcinoid tumors diagnosed accidentally by endoscopy, who were not operated, and were followed for more than 2 yr without evidence of metastasis. Because duodenal carcinoid tumors are slow growing and have a...


Read the Article


Sign out of this library

Other Topics