title: Prospective evaluation and treatment of familial carcinoid small intestine neuroendocrine tumors (SI-NETs).
abstract: BACKGROUND
  The aim of this study was to prospectively screen patients with a positive family history of carcinoid small intestine neuroendocrine tumors (SI-NETs) to elucidate the benefits of early detection and operative intervention.
  
  
  METHODS
  A single-center, prospective trial was conducted from 2008 to 2014 that evaluated patients with 2 or more blood relatives with carcinoid SI-NETs. All eligible patients were screened with urine/serum biochemistries and various imaging modalities. Operative intervention was elected in patients found to have at least 1 positive diagnostic study.
  
  
  RESULTS
  Twenty-nine patients from 13 families had occult carcinoid SI-NETs (15 female, 14 male). Twenty-four of the 29 patients (83%) had multifocal disease found in either the distal jejunum or ileum. On average, 75.9 cm (range, 13-195) of bowel was resected in 1 segment. Three patients were found to have stage IV disease at operation. All stage I-IIIB patients who had R0 resections have remained disease-free, with a median follow-up of 35 months.
  
  
  CONCLUSIONS
  Familial carcinoid SI-NETs often are asymptomatic and can be diagnosed with aggressive screening. With early detection, there may be a window of opportunity for operative resection to change the natural history of this disease and even prove to be curative.
authors.affiliation: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD. Electronic address: hughesm@mail.nih.gov.
authors.forename: Marybeth S
authors.initials: MS
authors.lastname: Hughes
authors.affiliation: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
authors.forename: Saïd C
authors.initials: SC
authors.lastname: Azoury
authors.affiliation: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
authors.forename: Yasmine
authors.initials: Y
authors.lastname: Assadipour
authors.affiliation: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
authors.forename: David M
authors.initials: DM
authors.lastname: Straughan
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Apurva N
authors.initials: AN
authors.lastname: Trivedi
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Ramona M
authors.initials: RM
authors.lastname: Lim
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Grishma
authors.initials: G
authors.lastname: Joy
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Mark T
authors.initials: MT
authors.lastname: Voellinger
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Derek M
authors.initials: DM
authors.lastname: Tang
authors.affiliation: Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD.
authors.forename: Aradhana M
authors.initials: AM
authors.lastname: Venkatesan
authors.affiliation: Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD.
authors.forename: Clara C
authors.initials: CC
authors.lastname: Chen
authors.affiliation: Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD.
authors.forename: Adeline
authors.initials: A
authors.lastname: Louie
authors.affiliation: Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
authors.forename: Martha M
authors.initials: MM
authors.lastname: Quezado
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Joanne
authors.initials: J
authors.lastname: Forbes
authors.affiliation: Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Stephen A
authors.initials: SA
authors.lastname: Wank
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002276:Carcinoid Tumor / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D055088:Early Detection of Cancer; D005260:Female; D006801:Humans; D007414:Intestinal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D007421:Intestine, Small / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies