Gallagher, M.C.; Phillips, R.K.S.
April 2003
Gut;Apr2003 Supplement 1, Vol. 52, pA16
Academic Journal
Background: Despite > 90% of patients with familial adenomatous polyposis having duodenal adenomas, only 5% develop cancer. This risk rises to more than 30% in patients with more advanced disease in whom prophylactic surgery is often advised. Aims: The outcome of prophylactic pylorus preserving pancreatoduodenal resection performed for Spigelman Stage IV duodenal polyposis detected in an endoscopic surveillance programme is presented. Methods: FAP patients entered into the surveillance programme at St Mark's Hospital were included in this study. Endoscopy is performed with a side viewing duodenoscope to a set protocol. Data were collected prospectively on the Polyposis Register, and the case notes of patients undergoing prophylactic surgery reviewed retrospectively. Results: 419 FAP patients have entered the endoscopic surveillance programme since 1989. Between 1994 and 2002, 15 patients with advanced duodenal polyposis (six male, average age 54 years and 4 months) were referred for PPPDR. Six suffered major postoperative complications (40%). Although the pathology of the resected specimen revealed less severe changes in two patients, five with Stage IV disease showed adenocarcinoma, all ampullary. One patient (with benign histology) died from a pulmonary embolus shortly after hospital discharge. Four of the five patients with adenocarcinoma have died (10-36 months postoperatively) and one further patient has died form a brain tumour. The remainder are alive at a mean of 36 months (2-103). Conclusion: Surprise invasive adenocarcinoma was already present in 33% of patients. Survival once cancer has developed is poor. Prophylactic surgery may be most appropriate for individuals with large ampullary polyps. Advising resection for earlier stage disease is limited by the complication rate.


Related Articles

  • Familial Occurrence of Parathyroid Adenomas, Pheochromocytoma, and Medullary Carcinoma of the Thyroid with Amyloid Stroma (Sipple's Syndrome). Sarosi, George; Doe, Richard P. // Annals of Internal Medicine;Jun68, Vol. 68 Issue 6, p1305 

    Presents a case report on the familial association of parathyroid adenomas, pheochromocytoma and medullary carcinoma of the thyroid with amyloid stroma. Physical examination of the patient; Laboratory examination; Hospital admission.

  • AN AGGRESSIVE DESMOID TUMOR IN A PATIENT WITH FAMILIAL ADENOMATOUS POLYPOSIS: IMMUNOHISTOCHEMICAL FINDINGS. Arai, Nobuyasu; Mitomi, Hiroyuki; Uesugi, Hidenaga; Aihara, Sigeaki; Ohtani, Yoshimasa; Okayasu, Isao // American Journal of Gastroenterology;Feb1999, Vol. 94 Issue 2, p530 

    A case of an aggressive desmoid tumor in a patient with familial adenomatous polyposis is described. The lesion rapidly enlarged with compression of adjacent structures including the ureter and small bowel, and the patient died because of small bowel perforation and hydronephrosis 3 years after...

  • Duodeno-gastric reflux and gastric adenomas: a scintigraphic study in patients with familial adenomatous polyposis. Spigelman, A. D.; Granowska, M.; Phillips, R. K. S. // Journal of the Royal Society of Medicine;Aug1991, Vol. 84 Issue 8, p476 

    To test whether the presence of gastric adenomas (dysplasia) was associated with gastric reflux of duodenal contents, six patients with familial adenomatous polyposis (FAP) who had gastric adenomas and nine matched FAP patients without gastric adenomas underwent scintigraphic duodeno-gastric...

  • Possible Role of Telomerase Activation in the Multistep Tumor Progression of Periampullary Lesions in Patients With Familial Adenomatous Polyposis. Mizumoto, Kazuhiro; Ogawa, Yoshiaki; Niiyama, Hideaki; Nagai, Eishi; Sato, Norihiro; Urashima, Taro; Matsumoto, Takayuki; Iida, Mitsuo; Tanaka, Masao // American Journal of Gastroenterology;Apr2001, Vol. 96 Issue 4, p1261 

    OBJECTIVES: The role of telomerase in periampullary tumor progression in patients with familial adenomatous polyposis (FAP) was investigated. METHODS: Relative telomerase activity was measured using telomerease amplification protocol in periampullary biopsy specimens of normal mucosa and adenoma...

  • Genetic Counseling in an Extended Attenuated Familial Adenomatous Polyposis Kindred. Lynch, Henry T.; Smyrk, Thomas; Lynch, Jane; Lanspa, Stephen; McGinn, Thomas; Cavalieri, R. Jennifer // American Journal of Gastroenterology;Mar1996, Vol. 91 Issue 3, p455 

    Objectives: To provide DNA-based genetic counseling to family members in the direct genetic lineage of a family fulfilling phenotypical criteria for the autosomal, dominantly inherited, attenuated familial adenomatous polyp (AFAP) syndrome. This enabled highly targeted cancer risk estimation...

  • Pituitary adenomas in the framework of hereditary syndromes. MAMEDOVA, E. O.; PRZHIYALKOVSKAYA, E. G.; PIGAROVA, E. A.; MOKRYSHEVA, N. G.; DZERANOVA, L. K.; TYULPAKOV, A. N. // Problemy Endokrinologii;2014, Vol. 60 Issue 4, p51 

    The overwhelming majority of the pituitary tumours are benign adenomas that remain a serious challenge to endocrinologists and neurosurgeons by virtue of great variety of their early manifestations, the impossibility to predict the neoplastic growth, and the influence exerted on the patients'...

  • Clinical characterization and the mutation spectrum in Swedish adenomatous polyposis families. Kanter-Smoler, Gunilla; Fritzell, Kaisa; Rohlin, Anna; Engwall, Yvonne; Hallberg, Birgitta; Bergman, Annika; Meuller, Johan; Grönberg, Henrik; Karlsson, Per; Björk, Jan; Nordling, Margareta // BMC Medicine;2008, Vol. 6, Special section p1 

    Background: The dominantly inherited condition familial adenomatous polyposis (FAP) is caused by germline mutations in the APC gene. Finding the causative mutations has great implications for the families. Correlating the genotypes to the phenotypes could help to improve the diagnosis and...

  • Ileal pouch adenocarcinoma after proctocolectomy for familial adenomatous polyposis. Tajika, Masahiro; Nakamura, Tuneya; Bhatia, Vikram; Komori, Koji; Kato, Tomoyuki; Yamao, Kenji // International Journal of Colorectal Disease;Dec2009, Vol. 24 Issue 12, p1487 

    The article discusses the formation of adenocarcinoma in the ileal pouch following a proctocolectomy for familial adenomatous polyposis (FAP), an inherited disease characterized by the development of colorectal adenomas. It cites the case of a 55-year-old female who suffered from gastric adenoma...

  • Liver adenomas and carcinomas: correlations and relationship to body weight in long-term rodent cancer bioassays. Gray, G.M.; Linkov, I.; Polkanov, M.; Wilson, R. // Toxicology & Industrial Health;2000, Vol. 16 Issue 6, p211 

    The most common cancers induced in laboratory rodents are liver cancers — both adenomas and carcinomas. There has been a long argument about the relative merits of combining them or considering them separately in the interpretation of long-term bioassays for chemical carcinogenesis. In...


Read the Article


Sign out of this library

Other Topics