ENETS Consensus Guidelines for the Management of Patients with Neuroendocrine Neoplasms from the Jejuno-Ileum and the Appendix Including Goblet Cell Carcinomas

Pape, Ulrich‐Frank; Perren, Aurel; Niederle, Bruno; Gross, David J.; Gress, Thomas M.; Costa, Frederico; Arnold, R.; Denecke, Timm; Plöckinger, Ursula; Salazar, Ramón; Grossman, Ashley

doi:10.1159/000335629

Cited by 410 publications

(489 citation statements)

References 168 publications

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“…In both adults and children, aNENs are usually diagnosed incidentally, during or after surgical treatment for acute appendicitis or other abdominal conditions (Moertel et al 1990, Parkes et al 1993, Corpron et al 1995, Bethel et al 1997, Plockinger et al 2008, Scott & Upadhyay 2011, Pape et al 2012. The rate of aNENs presence in patients undergoing appendicectomy is reported to be 0.3-0.9% (Moertel et al 1968, Connor et al 1998, Tchana-Sato et al 2006, Debnath et al 2008, In't Hof et al 2008, Shapiro et al 2010, Yilmaz et al 2013; however, in some series, it has been reported to be as high as 2.3% (Hatzipantelis et al 2010, Ozer et al 2011, Van Gompel et al 2007 or as low as 0.16% (Coskun et al 2006, Doede et al 2000.…”

Section: Epidemiologymentioning

confidence: 99%

“…Double-contrast GI studies, either CT or magnetic resonance imaging (MRI), exhibit the highest sensitivity in identifying primary neoplasms , Pape et al 2012. Imaging of the abdomen and pelvis has been the standard means to assess local and distant spread and, along with somatostatin receptor imaging (SRI), are used as staging imaging modalities for GI-NENs (Kwekkeboom et al 2009, Pape et al 2012.…”

Section: Imaging Featuresmentioning

confidence: 99%

“…An analysis of a large series of aNENs demonstrated that a tumour size O2 cm (greatest dimension) represents one of the most valuable predictive factors of lymph node (LN) metastasis (Groth et al 2011, Pape et al 2012. The great majority of aNENs (95%) are !2 cm in diameter: 60-85% are !1 cm and 4-27% are 1-2 cm, whereas only 2-17% are O2 cm (Moertel et al 1987, Roggo et al 1993.…”

Section: Histopathological Features Used To Identify High-risk Neoplasmsmentioning

confidence: 99%

“…Observational studies have shown that the natural history of these neoplasms is rather indolent, with only a minority developing extensive disease (Mullen & Savarese 2011). For this reason, there has been considerable debate as to whether these patients should be investigated for the presence of residual disease and the intensity and duration of subsequent follow-up (Plockinger et al 2008, Pape et al 2012, Grozinsky-Glasberg et al 2013. Goblet cell carcinomas (GCCs) are no longer regarded as a subset of aNENs but exhibit characteristics of both NENs and appendiceal adenocarcinomas, differing substantially in respect of their natural history, treatment and prognosis.…”

Section: Introductionmentioning

confidence: 99%

“…Goblet cell carcinomas (GCCs) are no longer regarded as a subset of aNENs but exhibit characteristics of both NENs and appendiceal adenocarcinomas, differing substantially in respect of their natural history, treatment and prognosis. They should be treated as adenocarcinomas and will not be discussed further in the present review (Pape et al 2012).…”

Section: Introductionmentioning

confidence: 99%

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Appendiceal neuroendocrine neoplasms: diagnosis and management

Alexandraki¹,

Kaltsas²,

Grozinsky‐Glasberg³

et al. 2015

Endocrine-Related Cancer

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Gastrointestinal neuroendocrine neoplasms (GI-NENs) are increasingly being recognised, while appendiceal NENs (aNENs) currently constitute the third most common GI-NEN. Appendiceal NENs are generally considered to follow an indolent course with the majority being localised at diagnosis. Thus, the initial surgical approach is not that of a planned oncological resection. Due to the localised nature of the disease in the majority of cases, subsequent biochemical and radiological assessment are not routinely recommended. Histopathological criteria (size, mesoappendiceal invasion, Ki-67 proliferation index, neuroand angio-invasion) are mainly used to identify those patients who are also candidates for a right hemicolectomy. Goblet cell carcinoids are a distinct entity and should be treated as adenocarcinomas. Despite the absence of any substantial prospective data regarding optimal management and follow-up, recent consensus statements and guidelines have been published. The purpose of this review is to overview the published studies on the diagnosis and management of appendiceal NENs and to suggest a possible management protocol.

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Section: Epidemiologymentioning

confidence: 99%

Section: Imaging Featuresmentioning

confidence: 99%

Section: Histopathological Features Used To Identify High-risk Neoplasmsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Appendiceal neuroendocrine neoplasms: diagnosis and management

Alexandraki¹,

Kaltsas²,

Grozinsky‐Glasberg³

et al. 2015

Endocrine-Related Cancer

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Association of a Prophylactic Surgical Approach to Stage IV Small Intestinal Neuroendocrine Tumors With Survival

et al. 2018

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Neuroendocrine Tumours

O’Toole¹,

Tang²,

Jensen³

et al. 2017

TNM Online

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Summary Gastroenteropancreatic neuroendocrine neoplasms (NEN) are complex tumours arising from many different sites and expressing a broad range of biological behaviours. Their incidence and that of bronchopulmonary NEN are rising. Recent staging and pathological classification systems aim at improving risk stratification and better categorizing NEN with respect to further therapy. Most tumours are well differentiated, but have frequently metastasized at time of diagnosis; despite this, patients often live for a relatively long time. Some tumours secrete biologically active hormones or peptides resulting in specific symptoms (or syndromes) and may require specific therapy for this, e.g., somataotatin analogue therapy. In advanced disease, a large number of treatments have been shown to be effective, including surgery, systemic chemotherapy, biotherapy (using somatostatin analogues or interferon), targeted therapies, liver‐directed strategies (liver embolization or radioembolization) and peptide receptor radionuclide therapy. Recent phase III trials have opened newer therapeutic possibilities, but strategic phase III trials testing the correct sequence of therapy are required to improve overall management in this group of patients. The multidisciplinary approach is essential in optimizing management in patients with NEN.

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ENETS Consensus Guidelines for the Management of Patients with Neuroendocrine Neoplasms from the Jejuno-Ileum and the Appendix Including Goblet Cell Carcinomas

Cited by 410 publications

References 168 publications

Appendiceal neuroendocrine neoplasms: diagnosis and management

Appendiceal neuroendocrine neoplasms: diagnosis and management

Association of a Prophylactic Surgical Approach to Stage IV Small Intestinal Neuroendocrine Tumors With Survival

Neuroendocrine Tumours

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