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

Autor: Hughes MS; Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD. Electronic address: hughesm@mail.nih.gov., Azoury SC; Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD., Assadipour Y; Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD., Straughan DM; Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD., Trivedi AN; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Lim RM; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Joy G; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Voellinger MT; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Tang DM; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Venkatesan AM; Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD., Chen CC; Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD., Louie A; Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD., Quezado MM; Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD., Forbes J; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD., Wank SA; Digestive Diseases Branch, National Institute of Diabetes, Kidney and Digestive Diseases, National Institutes of Health, Bethesda, MD.
Jazyk: angličtina
Zdroj: Surgery [Surgery] 2016 Jan; Vol. 159 (1), pp. 350-6. Date of Electronic Publication: 2015 Oct 09.
DOI: 10.1016/j.surg.2015.05.041
Abstrakt: 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.
(Published by Elsevier Inc.)
Databáze: MEDLINE
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