Duodenal cancer is the main cause of death for patients with FAP syndrome (familial adenomatous polyposis) treated with a colectomy. The disease follows the adenoma to carcinoma sequence and is diagnosed during follow-up in 7-36% of patients. Endoscopic treatment is used during the first treatment stage of the disease and surgery is an adequate therapeutic option when endoscopic control is insufficient.
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http://dx.doi.org/10.17235/reed.2019.6096/2018 | DOI Listing |
Am J Clin Pathol
January 2025
Department of Pathology and Laboratory Medicine, University of Miami Miller School of Medicine and Jackson Memorial Hospital, Miami, FL, US.
Objectives: Abdominal wall and intra-abdominal fibromatoses are locally aggressive, nonmetastasizing neoplasms. Surgery has been the mainstay of local control, but new forms of therapy have been developed that may influence the clinical course and morbidity. We studied the clinical features and outcomes of patients with abdominal and intra-abdominal fibromatoses over time.
View Article and Find Full Text PDFJ Am Coll Surg
January 2025
Department of Surgery, Emory University School of Medicine, Atlanta, GA.
Introduction: Pancreatoduodenectomy (PD) may occasionally be indicated for complete removal of periampullary (duodenal and ampullary) adenomas (PAs). As compared with malignant indications, PD for benign or pre-malignant disease is often associated with increased morbidity. While the Spigelman classification assesses malignancy risk for familial adenomatous polyposis (FAP)-related duodenal adenomas, no malignancy risk score (MRS) exists for non-FAP related PAs.
View Article and Find Full Text PDFClin Exp Pediatr
January 2025
Nationwide Children's Hospital, Columbus, United States.
Adenomatous Polyposis Coli (APC) is a tumor suppressor gene expressed throughout the body. APC mutations increase the risk of malignancy and are often characterized by syndromes that encompass a spectrum of neoplastic manifestations, such as familial adenomatous polyposis (FAP). We present a rare case of palatal peripheral nerve sheath tumor in the context of APC gene mutation.
View Article and Find Full Text PDFAnn Gastroenterol
December 2024
Department of Gastroenterology, Center for Advanced Therapeutic Endoscopy at Porter Adventist Hospital (Douglas G. Adler).
Background: Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is a common surgical procedure for ulcerative colitis and familial adenomatous polyposis. IPAA strictures are a known complication, often requiring surgical intervention. Endoscopic interventions offer a less invasive alternative, but their safety and efficacy remain uncertain.
View Article and Find Full Text PDFAm J Gastroenterol
December 2024
Department of Gastroenterology and Hepatology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.
Objectives: To prevent colorectal cancer (CRC), most patients with familial adenomatous polyposis (FAP) undergo (procto)colectomy with ileorectal anastomosis (IRA) or ileal pouch-anal anastomosis (IPAA). After surgery, these patients remain at risk of developing cancer in the remnant rectum or rectal cuff/pouch. We aimed to compare the long-term risk of cancer following IRA or IPAA in FAP.
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