Prospective evaluation and treatment of familial carcinoid small intestine neuroendocrine tumors (SI-NETs)

Surgery. 2016 Jan;159(1):350-6. doi: 10.1016/j.surg.2015.05.041. Epub 2015 Oct 9.


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.

Conclusion: 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.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Carcinoid Tumor / diagnosis*
  • Carcinoid Tumor / genetics
  • Carcinoid Tumor / surgery
  • Early Detection of Cancer
  • Female
  • Humans
  • Intestinal Neoplasms / diagnosis*
  • Intestinal Neoplasms / genetics
  • Intestinal Neoplasms / surgery
  • Intestine, Small / surgery
  • Male
  • Middle Aged
  • Prospective Studies