IN SITU DUODENAL AND INVASIVE GALLBLADDER ADENOCARCINOMA IN A NON-CLASSIC FAP PATIENT: CASE REPORT

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All articles of this category (opens in new window) Case Presentation: A 77-year-old female patient underwent total prophylactic colectomy 23 years ago for multiple colonic adenomatous polyps. Considering the patient's advanced age at diagnosis, it was suggestive that it was about a non-classical form of Familial Adenomatous Polyposis (FAP). Follow-up upper GI endoscopy with biopsy showed multiple tubulo-villous adenomas with high-grade dysplasia in the second duodenal portion and Vater's ampoule. Gastroduodenopancreatectomy and cholecystectomy were performed. Anatomopathological examination revealed intraepithelial adenocarcinoma associated with extensive marked duodenal mucosa dysplasia, with pancreatic duct involvement. In the gallbladder, moderately differentiated adenocarcinoma with invasion of the lamina propria associated with tubular adenoma with high-grade dysplasia measuring 2.5 cm was identified. Surgical limits and lymph nodes were free. The patient has regular outpatient follow-up, with no evidence of local recurrence or metastasis. Discussion: FAP is the most common adenomatous polyposis syndrome, characterized by multiple colorectal adenomatous polyps. The attenuated form of FAP is characterized by less than 100 colorectal polyps between the ages of 40 and 70, with an estimated 70% risk for colorectal cancer. Patients with FAP have a high risk of developing malignancies in the upper gastrointestinal tract, the duodenum being the most frequent extracolonic topography of polyps (30-70%), with a cumulative risk of 3-10% for duodenal cancer. In addition, rare reports describe the association of FAP with adenomas and gallbladder cancer. Spigelman's classification assesses the severity of duodenal polyposis, defining five stages according to size, number, histology and severity of polyps. The therapeutic decision for duodenal polyposis is defined according to Spigelman's five stages, which estimate the frequency of endoscopy and the need for chemoprevention or surgery. Final comments: Our case demonstrates the importance of screening for extracolonic neoplasms in patients with FAP and generates a reflection about the approach of high gastrointestinal polyps, which have a chance of malignant transformation. The best therapeutic strategy for these lesions should be individualized according to the characteristics of the polyps and the patient clinical condition. Publication History Article published online: 23 October 2019 © 2019. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/) Thieme Revinter Publicações Ltda. Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil Bibliographical Record Renata Rauber Felkl, Carolina Matté Dagostini, Giovani Schulte Farina, Mariana Gabriela Mandelli, Rafael Fontana, Fernando de Marco dos Santos, Aline Caldart Tregnago. IN SITU DUODENAL AND INVASIVE GALLBLADDER ADENOCARCINOMA IN A NON-CLASSIC FAP PATIENT: CASE REPORT. Brazilian Journal of Oncology 2019; 15. DOI: 10.1055/s-0044-1797774

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