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Familial Adenomatous Polyposis
Published in Savio George Barreto, Shailesh V. Shrikhande, Dilemmas in Abdominal Surgery, 2020
Paul Kolarsick, Steven D. Wexner
Attenuated familial adenomatous polyposis (AFAP) syndrome is a phenotypic variant in which less than one hundred synchronous adenomas are detected (Figure 24.2). Attenuated familial adenomatous polyposis is associated with a later onset of polyposis, frequently a right-sided polyp distribution, and a lower lifetime colorectal cancer risk (up to 70%). Average age of colorectal cancer diagnosis is also later, generally greater than 50 years of age. However, there are a number of other disorders that can yield a similar phenotype to AFAP, such as MYH-associated polyposis syndrome, Lynch syndrome, polymerase proofreading-associated polyposis, or simply multiple sporadic adenomas. The diagnosis of AFAP is made based on clinical findings including adenoma number (typically ranging from 20 to 100, with an average of 30 adenomatous polyps), family history, and results of genetic testing [3]. Upper gastrointestinal findings and the risk of duodenal cancer are similar to classic FAP.
Familial Adenomatous Polyposis
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Mariann Unhjem Wiik, Bente A. Talseth-Palmer
There are five stages (O−IV) to describe the severity of duodenal polyps, also known as Spigelman classification [129]. Stage O indicates absence of polyps, stage I mild disease, while stages II−IV are associated with more severe duodenal polyposis [2]. The system is based on points, which are given relative to number of polyps, the size of polyps, histology, and the adenomas degree of dysplasia. Screening of the duodenum may contribute to detection of patients with advanced duodenal polyps (Spigelman stage III and IV) and reduction of mortality caused by duodenal cancer [2]. The most important factors contributing to duodenal polyps seem to be age and mutation site [2,4]. The risk of developing duodenal cancer among all FAP patients is relatively low at 3%–5% [2,4]. However, patients with stage III−IV duodenal polyps have a 7%–36% risk of duodenal cancer and it is therefore very important to identify these patients to give them necessary treatment [2,96]. Periampullary carcinoma is in fact the leading cause of death in patients with FAP who have had a colectomy [13].
The Pancreas and the Periampullary Area
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Routine upper GI series by barium studies may suggest the diagnosis of a large ampullary or ulcerated duodenal cancer. Encroachment upon the medial wall of the duodenum will be seen in 50% of large pancreatic carcinomas.
Appropriate endoscopic treatment selection and surveillance for superficial non-ampullary duodenal epithelial tumors
Published in Scandinavian Journal of Gastroenterology, 2021
Kingo Hirasawa, Yuichiro Ozeki, Atsushi Sawada, Chiko Sato, Ryosuke Ikeda, Masafumi Nishio, Takehide Fukuchi, Ryosuke Kobayashi, Makomo Makazu, Masataka Taguri, Shin Maeda
The present study also investigated the long-term outcomes after ER of SNADETs. The five patients with a submucosal invasive carcinoma had poor long-term outcomes. To date, few studies have reported long-term outcomes after endoscopic treatment, all of which reported a DFS of 100%. Furthermore, although containing a small number of submucosal invasive cases, there are no reports of death due to duodenal cancer after ER [30,31]. This discrepancy from our results might be caused by missing an accurate follow-up in the previous studies, because submucosal invasive duodenal carcinoma reportedly showed a 20% prevalence of lymph node metastasis [13]. Considering the malignant potential of submucosal invasive duodenal carcinomas, accurately assessing tumor invasion preoperatively is very important. However, as Takinami et al. [13] reported, a definitive finding cannot be obtained with imaging techniques, including EUS. All our invasive cases were also diagnosed as endoscopic mucosal neoplasms. This matter needs further research.
Comparative Analysis of Outcomes and Clinicopathological Characteristics of Duodenal Adenocarcinoma: A SEER Analysis
Published in Cancer Investigation, 2020
Duodenal cancer, which arises from the duodenum, is a rare disease entity. It represents a mere 0.5% of gastrointestinal tumors and 18.4% to 45% of small intestinal tumors (1–3). In 1746, duodenal cancer was first described by Hamburger, after which there have been a few case reports of small numbers of patients (4). The most common subtype of duodenal cancer is adenocarcinoma, with other types including carcinoids, sarcomas, lymphomas, and signet ring cell carcinomas (5,6). Because of the low incidence of duodenal cancer and the lack of specificity of symptoms, patients are usually misdiagnosed or diagnosed at advanced stage with adverse prognosis, resulting in a five-year overall survival rate of 11–39% (7–9). Surgical resection remains the only potentially curative treatment. The surgical procedures for duodenal adenocarcinoma vary, with duodenal segmental resection or pancreaticoduodenectomy most often performed. In light of the low incidence of duodenal adenocarcinoma, it is challenging to accumulate ample patients in a single center for clinicopathological characteristics and survival analysis. Therefore, in this study, we utilized data obtained from the SEER database to investigate the clinicopathological characteristics and survival of patients with duodenal adenocarcinoma.
Adjuvant and first-line palliative chemotherapy regimens in patients diagnosed with periampullary cancer: a short report from a nationwide registry
Published in Acta Oncologica, 2022
Evelien J. M. de Jong, Irene Mommers, Arantza Fariña Sarasqueta, Lydia G. van der Geest, Lara Heij, Ignace H. J. T. de Hingh, Marjolein Y. V. Homs, Vivianne C. G. Tjan-Heijnen, Liselot B. J. Valkenburg-van Iersel, Johanna W. Wilmink, Sandra M. E. Geurts, Judith de Vos-Geelen
To conclude, this population-based study reflected the limited guidelines available for patients diagnosed with ampullary and duodenal cancer. Patients diagnosed with pancreatic ductal adenocarcinoma and distal cholangiocarcinoma were treated according to the available guidelines and recruiting trials. The chemotherapy regimens in patients diagnosed with duodenal adenocarcinoma were in agreement with the colorectal cancer guidelines, while the large variation in chemotherapy regimens in ampullary cancer reflects the different guidelines expected to be consulted.