P108 Is early surgery associated with a more benign disease course in Crohn's disease? Surgery rates in a population-based inception cohort from Western Hungary between 1977–2009

Abstract

Background: Carcinoid tumors of the small intestine are rare, often presenting with late stage disease not amenable to treatment, while early stage disease is surgically curable. Familial midgut carcinoid tumor has been described in rare case reports. However, the recent development of a relatively large cohort of high-risk asymptomatic members of familial midgut carcinoid tumor families has exposed the need for effective screening for occult tumor. Aim: Compare the utility of imaging modalities for the detection of occult midgut carcinoid tumor in an asymptomatic, high-risk cohort. Methods: This was a single center, prospective, natural history study. First-degree family members of affected individuals in families with at least two known cases of midgut carcinoid tumor were screened with computed tomography enterography (CTE), magnetic resonance imaging (MRI), [18F]fluorodihydroxyphenylalanine (FDOPA) positron emission tomography (PET), and (111)Inpentetreotide scintigraphy (SRS). Individuals with a family history of multiple endocrine neoplasia-1, multiple endocrine neoplasia-2, neurofibromatosis, or von Hippel Lindau, or any other known familial tumor syndrome were excluded. Patients with findings consistent with carcinoid tumor were referred for exploratory laparotomy and small bowel resection. Results: One-hundred-seven high-risk, asymptomatic patients underwent screening. A total of 20 patients were referred for surgery for abnormal small bowel findings. In patients referred for surgery, CTE was abnormal in 15 of 20 cases, MRI was abnormal in 2 of 20 cases, FDOPA-PET was abnormal in 14 of 20 cases, and SRS was abnormal in 1 of 20 cases. A total of 17 patients (16% yield of screening) were confirmed to have midgut carcinoid tumor. Median age of diagnosis was 56 (range 44-74) years. Nine of 17 patients were found with either stage 1 or 2 disease, while 8 of 17 patients were found with stage 3 or 4 disease. The positive predictive value of multi-modality screening for carcinoid tumor was 85%. The three falsely positive patients were found to have a lymphangioma, large lymphoid aggregates, or a lymphocele of the distal small bowel. Fifteen of 16 occult carcinoid patients have shown no evidence of disease on a median follow-up of 26 (range 3-35) months. Conclusions: Screening of asymptomatic, high risk individuals with multi-modality imaging diagnosed a high proportion of early stage midgut carcinoid tumors compared to previously reported series. CT and FDOPA-PET were most sensitive compared to MRI and SRS, both of which were of limited value. Sensitive diagnostic imaging appears promising for the detection of early disease and therefore may improve the surgical outcome of individuals at risk for midgut carcinoid.