Details

Autor(en) / Beteiligte

• Hughes, Marybeth S., MD, FACS
• Azoury, Saïd C., MD
• Assadipour, Yasmine, MD
• Straughan, David M., MD
• Trivedi, Apurva N., MD
• Lim, Ramona M., MD
• Joy, Grishma, MD
• Voellinger, Mark T., MD
• Tang, Derek M., MD
• Venkatesan, Aradhana M., MD
• Chen, Clara C., MD
• Louie, Adeline, MD
• Quezado, Martha M., MD
• Forbes, Joanne, MS, CRNP
• Wank, Stephen A., MD

Titel

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

Ist Teil von

• Surgery, 2016-01, Vol.159 (1), p.350-357

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2016

Quelle

ScienceDirect

Beschreibungen/Notizen

• 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.