Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

Ramage, John; Ahmed, Alia; Ardill, Joy; Bax, N. D. S.; Breen, David J.; Caplin, Martyn; Corrie, Pippa; Davar, Joseph; Davies, Amy; Lewington, Val; Meyer, Tim; Newell‐Price, John; Poston, Graeme J.; Reed, Nicholas; Rockall, Andrea; Steward, W.; Thakker, Rajesh V.; Toubanakis, Christos; Valle, Juan W; Verbeke, Caroline; Grossman, Ashley

doi:10.1136/gutjnl-2011-300831

Cited by 626 publications

(486 citation statements)

References 372 publications

(261 reference statements)

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“…Others claim a tumor size O2 cm as indication for surgery, but it is clear that a large proportion of these patients already have metastases (Bartsch et al 2005, Kouvaraki et al 2006, Triponez et al 2006, Triponez & Cadiot 2007, You et al 2007, Ekeblad et al 2008, Akerstrom & Hellman 2009. Clearly this remains an open question as outlined in current guidelines and deserves a prospective analysis , Ramage et al 2012. Due to the high rate of multicentric lesions, intraoperative ultrasound is mandatory.…”

Section: Nonfunctioning Pnensmentioning

confidence: 99%

Neuroendocrine tumor disease: an evolving landscape

Frilling

Åkerström

Falconi

et al. 2012

Endocrine-Related Cancer

143

104

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Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) represent a heterogenous group of tumors arising from a variety of neuroendocrine cell types. The incidence and prevalence of GEPNENs have markedly increased over the last three decades. Symptoms are often absent in early disease, or vague and nonspecific even in advanced disease. Delayed diagnosis is thus common. Chromogranin A is the most commonly used biomarker but has limitations as does the proliferative marker Ki-67%, which is often used for tumor grading and determination of therapy. The development of a multidimensional prognostic nomogram may be valuable in predicting tumor behavior and guiding therapy but requires validation. Identification of NENs that express somatostatin receptors (SSTR) allows for SSTR scintigraphy and positron emission tomography imaging using novel radiolabeled compounds. Complete surgical resection of limited disease or endoscopic ablation of small lesions localized in stomach or rectum can provide cure; however, the majority of GEP-NENs are metastatic (most frequently the liver and/or mesenteric lymph nodes) at diagnosis. Selected patients with metastatic disease may benefit from advanced surgical techniques including hepatic resection or liver transplantation. Somatostatin analogs are effective for symptomatic treatment and exhibit some degree of antiproliferative activity in small intestinal NENs. There is a place for streptozotocin, temozolomide, and capecitabine in the management of pancreatic NENs, while new agents targeting either mTOR (everolimus) or angiogenic (sunitinib) pathways have shown efficacy in these lesions.

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Section: Nonfunctioning Pnensmentioning

confidence: 99%

Neuroendocrine tumor disease: an evolving landscape

Frilling

Åkerström

Falconi

et al. 2012

Endocrine-Related Cancer

143

104

View full text Add to dashboard Cite

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“…In these patients, resection of the primary lesion is a palliative treatment that could reduce local complications and might even increase survival [4,5]. Therefore, a multimodality imaging approach including computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), somatostatin receptor scintigraphy (SSRS) and endoscopy is often necessary for detecting the primary tumor [6]. The recently updated European NET Society (ENETS) guidelines for the management of NETs consider wireless capsule endoscopy (WCE) NET with unknown primary, but its impact on the clinical management of these patients is still debated and adequate data is lacking.…”

Section: Introductionmentioning

confidence: 99%

The Role of Wireless Capsule Endoscopy (WCE) in the Detection of Occult Primary Neuroendocrine Tumors

Furnari

Buda²,

Delconte

et al. 2017

JGLD

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Background & Aims: Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms with unclear etiology that may show functioning or non-functioning features. Primary tumor localization often requires integrated imaging. The European Neuroendocrine Tumors Society (ENETS) guidelines proposed wireless-capsule endoscopy (WCE) as a possible diagnostic tool for NETs, if intestinal origin is suspected. However, its impact on therapeutic management is debated. We aimed to evaluate the yield of WCE in detecting intestinal primary tumor in patients showing liver NET metastases when first-line investigations are inconclusive.Method: Twenty-four patients with histological diagnosis of metastatic NET from liver biopsy and no evidence of primary lesions at first-line investigations were prospectively studied in an ENETS-certified tertiary care center. Wireless-capsule endoscopy was requested before explorative laparotomy and intra-operative ultrasound. The diagnostic yield of WCE was compared to the surgical exploration.Results: Sixteen subjects underwent surgery; 11/16 had positive WCE identifying 16 bulging lesions. Mini-laparotomy found 13 NETs in 11/16 patients (9 small bowel, 3 pancreas, 1 bile ducts). Agreement between WCE and laparotomy was recorded in 9 patients (Sensitivity=75%; Specificity=37.5%; PPV=55%; NPV=60%). Correspondence assessed per-lesions produced similar results (Sensitivity=70%; Specificity=25%; PPV=44%; NPV=50%). No capsule retentions were recorded.Conclusions: Wireless-capsule endoscopy is not indicated as second-line investigation for patients with gastro-entero-pancreatic NETs. In the setting of a referral center, it might provide additional information when conventional investigations are inconclusive about the primary site.Abbreviations: DBE: double balloon enteroscopy; GEP-NET: gastro-entero-pancreatic neuroendocrine tumor; GI: gastrointestinal; ENETS: European Neuroendocrine Tumor Society; NET: neuroendocrine tumor; SSRS: somatostatin receptor scintigraphy; WCE: wireless capsule endoscopy.

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“…For example octreotide is a safe and effective adjunct to surgical strategies for the management of GEP neoplasia in hypergastrinemic MEN-1 patients [25]. Despite that surgery should be offered when NETs are resectable and there is curative intent (or when debulking offers palliation) [26].…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia type 1: a case report and review of the literature

et al. 2014

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AbstractMultiple endocrine neoplasia syndrome, type 1 (MEN1) is an underdiagnosed autosomal dominant inherited cancer predisposition syndrome with inter- and intrafamilial variability without a known genotype-phenotype correlation. Disease is caused by mutations in the MEN1 gene located on chromosome 11, but other genes (CDKN1B, AIP) and mechanisms might be involved too. We performed retrospective case series study of MEN1 syndrome patient and his family and present our experience of management of genetically confirmed 9 MEN1 syndrome large family members from Lithuania with novel MEN1 gene mutation (MEN1 exon 6 — c. 879delT (p.Pro293Profs*76)) and delineate its clinical phenotype. At present the diagnosis of MEN1 syndrome must be established by direct mutation testing. MEN1 syndrome patients, their relatives and patients suspected of MEN1 are eligible for mutation testing. Patients with MEN1 have a shorter life expectancy than the general population. MEN1 patients and mutation carriers should be subjected to periodic screening in order to detect manifestations in an early stage. Early genetic diagnosis and subsequent periodic screening is associated with less morbidity and mortality at follow-up. Our study confirmed the absence of genotype-phenotype correlation and showed high intrafamilial clinical expression variability of the MEN1 syndrome.

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Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

Cited by 626 publications

References 372 publications

Neuroendocrine tumor disease: an evolving landscape

Neuroendocrine tumor disease: an evolving landscape

The Role of Wireless Capsule Endoscopy (WCE) in the Detection of Occult Primary Neuroendocrine Tumors

Multiple endocrine neoplasia type 1: a case report and review of the literature

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