MARIANNY NAZARETH SULBARAN NAVA
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conferenceObject KI-67 EXPRESSION CAN BE A MARKER OF AGRESSIVENESS OF DUODENAL DISEASE IN FAMILIAL ADENOMATOUS POLYPOSIS(2020) SULBARAN, Marianny; CAMPOS, Fabio G.; PAES, Vitor R.; ALVES, Venancio A.; KISHI, Humberto; MOURA, Eduardo G. de; BUSTAMANTE-LOPEZ, Leonardo A.; NAHAS, Sergio C.; RIBEIRO, Ulysses; COUDRY, Renata; SAFATLE, Adriana V.- Risk factors for advanced duodenal and ampullary adenomatosis in familial adenomatous polyposis: a prospective, single-center study(2018) SULBARAN, M.; CAMPOS, F. G.; RIBEIRO JR., U.; KISHI, H. S.; SAKAI, P.; MOURA, E. G. H. de; BUSTAMANTE-LOPEZ, L.; TOMITAO, M.; NAHAS, S. C.; CECCONELLO, I.; SAFATLE-RIBEIRO, A. V.Background and study aims To determine the clinical features associated with advanced duodenal and ampullary adenomas in familial adenomatous polyposis. Secondarily, we describe the prevalence and clinical significance of jejunal polyposis. Patients and methods This is a single center, prospective study of 62 patients with familial adenomatous polyposis. Duodenal polyposis was classified according to Spigelman and ampullary adenomas were identified. Patients with Spigelman III and IV duodenal polyposis underwent balloon assisted enteroscopy. Predefined groups according to Spigelman and presence or not of ampullary adenomas were related to the clinical variables: gender, age, family history of familial adenomatous polyposis, type of colorectal surgery, and type of colorectal polyposis. Results Advanced duodenal polyposis was present in 13 patients (21%; 9 male) at a mean age of 37.61 +/- 13.9 years. There was a statistically significant association between family history of the disease and groups according to Spigelman (P=0.03). Seven unrelated patients (6 male) presented ampullary adenomas at a mean age of 36.14 +/- 14.2 years. The association between ampullary adenomas and extraintestinal manifestations was statistically significant in multivariate analysis (P=0.009). Five endoscopic types of non-ampullary adenoma were identified, showing that lesions larger than 10mm or with a central depression presented foci of high grade dysplasia. Among 28 patients in 12 different families, a similar Spigelman score was identified; 10/12 patients (83.3%) who underwent enteroscopy presented small tubular adenomas with low grade dysplasia in the proximal jejunum. Conclusions Advanced duodenal polyposis phenotype may be predictable from disease severity in a first-degree relative. Ampullary adenomas were independently associated with the presence of extraintestinal manifestations.
- Upper gastrointestinal neoplasia in familial adenomatous polyposis: prevalence, endoscopic features and management(2019) CAMPOS, Fabio Guilherme; MARTINEZ, Augusto Real; SULBARAN, Marianny; BUSTAMANTE-LOPEZ, Leonardo Alfonso; SAFADE-RIBEIRO, Adriana VazBackground: To evaluate the prevalence of upper gastrointestinal (GI) polyps in familial adenomatous polyposis (FAP), and to discuss current therapeutic recommendations. Methods: Clinical, endoscopic, histological and treatment data were retrieved from charts of 102 patients [1958-2016]. Duodenal adenomatosis was classified according to Spigelman stages. Results: this series comprised 59 women (57.8%) and 43 men (42.1%) with a median age of 32.3 years. Patients underwent 184 endoscopic procedures, the first at a median age of 35.9 years (range, 13-75 years). Fundic gastric polyps (n=31; 30.4%) prevailed in the stomach. While only 5 adenomas were found in the stomach, 33 patients (32.4%) presented duodenal ones. Advanced lesions (n=13; 12.7%) were detected in the stomach (n=2) and duodenum (n=11). During follow-up, Spigelman stages improved in 6 (12.2%) patients, remained unchanged in 25 (51.0%) and worsened in 18 (36.7%). Carcinomas were diagnosed in the stomach and duodenum (4 lesions each, 3.9%), at median ages of 50.2 and 55.0 years, respectively. Advanced lesions and carcinomas were managed through local or surgical resections. Severe complications occurred in only 2 patients (one death). Enteroscopy in 21 patients revealed jejunal adenomas in 12, 11 of whom also presented duodenal adenomas. Conclusions: There is a high prevalence of upper GI adenomas and cancer in FAP. There were diagnosed fundic gastric polyps (30.4%), duodenal (32.4%) and jejunal adenomas (11.8%), respectively. One third of duodenal polyps progressed slowly throughout the study. The rates of advanced gastroduodenal lesions (12.7%) and cancer (7.8%) raise the need for continuous surveillance during follow-up.
conferenceObject ADVANCED DUODENAL AND AMPULLARY ADENOMATOSIS IN A BRAZILIAN POPULATION OF FAMILIAL ADENOMATOUS POLYPOSIS: PROSPECTIVE CLINICAL AND MOLECULAR STUDY.(2017) NAVA, M. Sulbaran; CAMPOS, F.; RIBEIRO JUNIOR, U.; COUDRY, R.; MEIRELES, S.; LISBOA, B.; KISHI, H.; SAKAI, P.; MOURA, E. de; BUSTAMANTE-LOPEZ, L.; TOMITAO, M.; NAHAS, S.; RIBEIRO, A. Safatle- Duodenal adenoma surveillance in patients with familial adenomatous polyposis(2015) CAMPOS, Fabio Guilherme; SULBARAN, Marianny; SAFATLE-RIBEIRO, Adriana Vaz; MARTINEZ, Carlos Augusto RealFamilial adenomatous polyposis (FAP) is a hereditary disorder caused by Adenomatous Polyposis Gene mutations that lead to the development of colorectal polyps with great malignant risk throughout life. Moreover, numerous extracolonic manifestations incorporate different clinical features to produce varied individual phenotypes. Among them, the occurrence of duodenal adenomatous polyps is considered an almost inevitable event, and their incidence rates increase as a patient's age advances. Although the majority of patients exhibit different grades of duodenal adenomatosis as they age, only a small proportion (1%-5%) of patients will ultimately develop duodenal carcinoma. Within this context, the aim of the present study was to review the data regarding the epidemiology, classification, genetic features, endoscopic features, carcinogenesis, surveillance and management of duodenal polyps in patients with FAP.