ENETS Guidelines Research Articles

Radiological follow-up in patients with resected pulmonary carcinoids: Should we reduce radiation exposure?

IntroductionAfter primary resection of pulmonary carcinoids, the recurrence rate is low (approximately 10 %). However, long-term radiological follow-up is generally recommended due to the risk of late recurrence. This must be weighed against risk of radiation-induced cancer, particularly in young patients. MethodsThe frequency and modality of radiological follow-up according to the ENETS, ESMO, and CommNETs-NANETS guidelines were assessed. Cumulative radiation exposure per guideline and subsequent increased lifetime cancer risk were estimated using sex- and age-dependent risk factors. Data from the Netherlands Cancer Registry (2003–2012) of adults with resected pulmonary carcinoids were used as a reference. ResultsOf 706 reference patients, 32 (4.5 %) were 18–30 years (y). After median follow-up of 127 months, none of the patients aged 18-30y at diagnosis developed recurrence. For these patients, the additional radiation exposure at the age of 40y due to follow-up ranges from 140-308 mSv following ENETS and 35–42 mSv following ESMO guidelines. The additional risk of death due to carcinogenic effects ranged from 0.7 % (male 30y) to 3.1 % (female 18y) following ENETS and 0.2 % (male) to 0.4 % (female) following ESMO guidelines. ConclusionsIndividualised, less extensive follow-up for young patients with resected carcinoids and a low risk of recurrence are worth exploring to decrease radiation exposure and the corresponding risk of cancer induction. The use of predictive biomarkers to personalise follow-up is warranted.

Claims-based analysis of treatment practices of patients with metastatic gastroenteropancreatic neuroendocrine tumors (mGEP-NET) in the USA.

e16286 Background: GEP-NET is a rare disease with variable tumor biology. An indolent disease course with subtle symptoms renders timely diagnosis difficult; 2/3 of patients (pts) are diagnosed at locally advanced or metastasized stage. In clinical decision-making, disease-specific factors, patient (pt) age and long-term disease control must be balanced against the aggressiveness of therapies. NCCN, NANETS and ENETS guidelines recognize the diversity of mGEP-NET by recommending individualized treatment approaches. We aimed to describe treatment (Tx) utilization patterns in mGEP-NET. Methods: Retrospective cohort study using MarketScan® Commercial and Medicare Databases spanning 07/01/2017-01/31/2023. Eligible were GEP-NET pts with ≥1 inpatient or ≥2 outpatient claims, a subsequent new diagnosis of metastatic disease (index), no secondary malignancy, and follow-up time of ≥12 months (m) after index. Results: Of 16218 GEP-NET pts, 2002 (mean age 60y; 50% female) met inclusion criteria. Mean follow-up time after index was 17m, with 49%, 27% and 15% followed up for ≥12, 24 and 36m, resp. Medical history (12m baseline before index) included mean Charlson Comorbidity Index of 2.62, with hypertension (HTN), type 2 diabetes (T2DM), chronic liver disease (CLD), asthma/COPD, cardiovascular disease (CVD), and chronic kidney disease (CKD) present in 56, 26, 22, 18, 17 and 10% of pts, resp.; 5% had carcinoid syndrome. In total, 949 pts (47.4%) received first-line (1L) systemic Tx: somatostatin analogs (SSA; 61.6%) of either very short or long duration (median 116d vs 775d), chemotherapy (38.8%), targeted therapies (3.4%) and PRRT (0.5%). Relative to NCCN cancer surveillance guidelines, 49.5% received more and 36.8% received fewer than 4 scans in the first year of treatment. Conversely, 1053 (52.6%) did not receive any 1L systemic Tx. With the exception of CLD, pts with vs without 1L Tx had similar proportions of medical conditions during the 12m baseline and during follow-up (HTN 72.4 vs 66.5%; T2DM 34.0 vs 30.3%; asthma/COPD 16.5 vs 19.3%; CVD 27.3 vs 36.9%; CKD 18.9 vs 17.9%; however, CLD 54.0 vs 36.7%). See Table comparing both cohorts on insurance, treatments, and health care utilization during follow-up. Conclusions: This claims analysis reveals treatment differences among mGEP-NET pts that cannot be explained by the parameters assessed herein. Further research should investigate factors influencing diagnosis and treatment decision-making, applying also pt-level health record data; and analyze economic barriers to diagnosis and treatment. [Table: see text]

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for appendiceal neuroendocrine tumours (aNET).

The aim of the present guidance paper is to update the previous ENETS guidelines on well differentiated appendiceal neuroendocrine tumours (NET), providing practical guidance for the diagnosis and management of appendiceal NET (aNET); poorly differentiated neoplasms are dealt with in a separate guidance paper. This paper is structured on a question-answer format in order to also address controversial issues and areas where uncertainty regarding the management and follow-up of aNET exists. All recommendations are offered on the basis of the best available evidence, along with the authors' experiences in managing these neoplasms. Each recommendation for treatment will provide a level of evidence and grade of recommendation as per the GRADE system (adapted in Infectious Disease Society of United States Public Health Service grading system).

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1-G3.

The aim of the present guidance paper was to update the previous ENETS guidelines on well-differentiated gastric and duodenal neuroendocrine tumours (NETs), providing practical guidance for specialists in the diagnosis and management of gastroduodenal NETs. Type II gastric NETs, neuroendocrine carcinomas (NECs), and functioning duodenal NETs are not covered, since they will be discussed in other ENETS guidance papers.

Radical surgical treatment of neuroendocrine tumors of the appendix in children - a Polish multicenter study.

IntroductionThe aim of the study was to examine management of pediatric appendiceal neuroendocrine tumors (ANETs) in Poland.MethodsRecords of 27 patients with ANET diagnosed incidentally after appendectomy in the last decade.ResultsWell-differentiated NET G1/G2 was diagnosed in 25 and well-differentiated neuroendocrine carcinoma G3 in 2 patients. Extended surgery was performed primarily in one instance and secondarily in 10 patients (right hemicolectomy in 9, ileocecal resection in 1) without adjuvant chemotherapy. Follow-up range was 1–121 months. Recurrence after secondary surgery was observed in 1 (3.7%) patient.ConclusionsApplying ENETS guidelines resulted in 100% overall survival of patients with NET.

Neuroendocrine neoplasms of the appendix: Characterization of 335 patients referred to the Copenhagen NET Center of Excellence

IntroductionNeuroendocrine neoplasms (NEN) of the appendix are often incidentally discovered after appendectomy. Appropriate management is debated. The purpose was to characterize a cohort of 335 appendix NEN and evaluate the risk of recurrence. MethodsRetrospective collection of data from 335 patients referred to the Neuroendocrine Tumor Center at Rigshospitalet 2000–2019. Appendix goblet cell carcinoids and mixed neuroendocrine non-neuroendocrine neoplasms were excluded.Patients were followed until December 31st, 2019. No patients were lost to follow-up. ResultsSixty-three percent of the patients were female. The median (range) age at diagnosis was 34 (9–92) years. Median follow-up was 66 (1–250) months. Median tumor size was 7 (1–45) mm with 10 (3%) tumors >20 mm. In 18 specimens (5%) resection margins were positive. Mesoappendiceal invasion was found in 113 (35%). Sixty-three (19%) patients underwent right-sided completion hemicolectomy (RHC) after appendectomy according to ENETS guidelines. Among these, 11 (17%) had lymph node metastases in the resected tissue. Further, one patient who underwent initial RHC due to colonic adenocarcinoma had lymph node metastases.All lymph node metastases were detected in patients with serotonin positive tumors. No patients with glucagon positive tumors (n = 85) had lymph node metastases. Mesoappendiceal invasion >3 mm and positive resection margins were associated with presence of lymph node metastases. No recurrences were recorded. ConclusionFollowing ENETS guidelines may lead to overtreatment of patients with respect to completion RHC. The risk of over- and undertreatment needs to be further evaluated.

A “Watch and Wait” Strategy Involving Regular Endoscopic Surveillance Is Safe for Many Patients with Small, Sporadic, Grade 1, Non-Ampullary, Non-Functioning Duodenal Neuroendocrine Tumours

Introduction: Duodenal neuroendocrine tumours (d-NETs) are rare but are increasing in incidence. Current ENETS guidelines advocate resection of all localized d-NETs. However, “watch and wait” may be appropriate for some localized, small, grade 1, non-functioning, non-ampullary d-NETs. We evaluated whether patients with such d-NETs who chose “watch and wait” involving regular endoscopic surveillance had equivalent disease-related outcomes to patients undergoing endoscopic or surgical resection. Methods: Retrospective review of patients with histologically confirmed d-NETs at Liverpool ENETS Centre of Excellence 2007–2020. Results: Sixty-nine patients were diagnosed with d-NET of which 50 were sporadic, non-functioning, non-ampullary tumours. Patient treatment groups were similar in terms of age, gender, and tumour location and grade, but unsurprisingly, larger tumours (median diameter 17 mm [p < 0.0001]) were found in the surgically treated group. Five patients underwent surgical resection with no evidence of tumour recurrence or disease-related death. Twelve patients underwent endoscopic resection (ER), with 1 local recurrence detected during follow-up. Thirty patients (28 with d-NETs ≤10 mm) underwent “watch and wait” with resection only if tumours increased in size. The d-NETs in 28/30 patients remained stable or decreased in size over a median 27 months (IQR: 15–48, R: 3–98). In 7 patients, the d-NET was completely removed by avulsion during diagnostic biopsy and was not seen at subsequent endoscopies. Only 2 patients showed increased d-NET size during surveillance, of whom only one was fit for ER. No NET-related deaths were documented during follow-up. Conclusions: All of the localized, ≤10 mm, grade 1, non-functioning, non-ampullary d-NETs in this cohort behaved indolently with very low risks of progression and no tumour-related deaths. “Watch and wait,” therefore, appears to be a safe alternative management strategy for selected d-NETs.

Role of Primary Tumor Resection for Metastatic Small Bowel Neuroendocrine Tumors.

While small bowel resection is well established as standard of care for curative-intent management of localized and loco-regional small bowel neuroendocrine tumors (SB-NETs), resection of the primary tumor in the setting of metastatic disease is debated. This review addresses the role of primary tumor resection for stage IV well-differentiated grade 1 and 2 SB-NETs. While survival benefits have been reported for primary tumor resection in the setting of metastatic disease, these studies are limited by selection bias and thus controversial. The main clinical benefits of primary tumor resection for stage IV disease involve the prevention of potentially debilitating complications associated with mesenteric fibrosis, including intestinal obstruction, mesenteric ischemia and angina, venous congestion, malabsorption, and malnutrition. Patients with metastases undergoing initial resection of the primary SB-NETs appear to have fewer episodes of care and re-intervention for loco-regional complications than those who do not undergo resection. As recommended by the NANETS and ENETS guidelines, resection of the primary tumor for stage IV SB-NETs should be strongly considered to avoid future loco-regional complications and potentially to improve survival. All patients with stage IV SB-NETs should be assessed by a surgeon experienced in the management of NETs to consider surgical therapies, including resection of the primary tumor despite metastatic disease.

Radical intended surgery for highly selected stage IV neuroendocrine neoplasms G3

BackgroundStage IV gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) G3 are the NENs with the worst prognosis. According to ENETS guidelines, platinum-based chemotherapy is the standard treatment for this population. Surgery is only considered in highly selected “resectable” NENs with usually lower Ki67. However, the role of surgery with curative intent has been poorly investigated. ObjectiveTo describe, in a retrospective series of stage IV GEP-NENs G3, overall survival (OS) and recurrence-free survival (RFS) rates after curatively intended surgery. MethodsMulticenter analysis of stage IV GEP-NENs G3 receiving radical resection (R0/R1) from 2007 to 2017, with minimum post-surgical follow-up time of 3 months. ResultsFifteen patients from 6 NEN referral centers, with median follow-up of 29 months (8-86), were included. Eight cases had a neuroendocrine carcinoma (NEC) and 7 a neuroendocrine tumor G3 (NET G3). Median OS after radical surgery was 59 months. All patients recurred, with a median RFS of 8 months. ConclusionsRadical surgery might be considered for highly selected stage IV GEP-NENs G3. Larger series are needed to confirm these results.

Liver transplantation in patients with liver metastases from neuroendocrine tumors.

The prevalence of metastatic disease in neuroendocrine tumors (NETs) is very high (60-80%) and cancer-related death among these patients is generally due to metastatic disease. Numerous treatment options for cure and disease control have been investigated for patients with neuroendocrine liver metastases (NELM). Despite the success of liver directed therapy on slowing tumor progression and palliating symptoms, the chance of being cured by liver resection is 40-50% and only roughly 20% of patients have potentially resectable disease. As such, there has been interest in liver transplantation (LT) as a potentially curative option for patients with unresectable disease. Several criteria have been proposed in order to balance long-term outcomes of patients with NELM and the problem of organ shortage including the Milan-NET criteria, the UNOS criteria and the ENETS guidelines. In the most representative studies, recurrence rate after LT has ranged from 30% to 60% with a 5-year OS ranging from 50% to 97%. This large variability is due to the retrospective nature of the studies available, which used different inclusion criteria. As such, outcomes and the prognostic factors associated with LT for NELM warrant further investigation.

Implementation of Current ENETS Guidelines for Surgery of Small (≤2cm) Pancreatic Neuroendocrine Neoplasms in the German Surgical Community: An Analysis of the Prospective DGAV StuDoQ|Pancreas Registry.

ENETS guidelines recommend parenchyma-sparing procedures without formal lymphadenectomy, ideally with a minimally invasive laparoscopic approach for sporadic small pNENs (≤2cm). Non-functioning (NF) small pNENs can also be observed. The aim of the study was to evaluate how these recommendations are implemented in the German surgical community. Data from the prospective StuDoQ|Pancreas registry of the German Society of General and Visceral Surgery were analyzed regarding patient's demographics, tumor characteristics, surgical procedures, histology and perioperative outcomes. Eighty-four (29.2%) of 287 patients had sporadic pNENs ≤2cm. Forty-three (51.2%) patients were male, and the mean age at diagnosis was 58.8 ± 15.6years. Twenty-five (29.8%) pNENs were located in the pancreatic head. The diagnosis pNEN was preoperatively established in 53 (65%) of 84 patients. Sixty-two (73.8%) patients had formal pancreatic resections, including partial pancreaticoduodenectomy or total pancreatectomy (21.4%). Only 22 (26.2%) patients underwent parenchyma-sparing resections and 23 (27.4%) patients had minimally invasive procedures. A lymphadenectomy was performed in 63 (75.4%) patients, and lymph node metastases were diagnosed in 6 (7.2%) patients. Eighty-two (97.7%) patients had an R0 resection. Sixty (72%) tumors were classified G1, 24 (28%) tumors G2. Twenty-seven (32.2%) of 84 patients had postoperative relevant Clavien-Dindo grade ≥3 complications. Thirty- and 90-day mortalities were 2.4% and 3.6%. ENETS guidelines for surgery of small pNENs are yet not well accepted in the German surgical community, since the rate of formal resections with standard lymphadenectomy is high and the minimally invasive approach is underused. The attitude to operate small NF tumors seems to be rather aggressive.

The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma?

Analyze the role of somatostatin analogues (SSAs) in the treatment of sporadic and MEN1-related gastrinomas, trying to define whether recent trials have changed the landscape of gastrinoma therapy. We evaluate the rationale of SSA use in the treatment of gastrinomas, summarize the current literature concerning the effect of SSAs on the control of Zollinger-Ellison syndrome (ZES) and gastrinomas tumor progression and discuss their role in the most recent guidelines. The medical treatment of gastrinoma and related ZES is aimed at controlling acid hypersecretion and tumor progression, in inoperable patients. The use of proton pump inhibitors (PPIs) to control the syndrome is a cornerstone in the ZES therapy. SSAs are not usually indicated for antisecretory purpose, because PPIs are considered the treatment of choice, due to their long lasting high efficacy and oral availability. The antiproliferative effect of SSAs has been established by two placebo-controlled trials that have clearly demonstrated a significant increase in progression free survival in patients affected by non-functioning well-differentiated advanced neuroendocrine tumors (NETs). The recent ENETS guidelines recommend the use of SSAs in advanced well differentiated NETs as antiproliferative agents. The high sstr-expression in gastrinomas make them highly responsive to SSAs and support the use of such drugs to counteract the tumour growth in patients not amenable to surgical cure. Unfortunately, limited data, mainly case reports or small series, support the use of SSAs in advanced gastrinomas, therefore, it is difficult to quantify their ability to control tumour growth and disease progression.

Changes in the pathomorphological diagnosis of gastrointestinal neuroendocrine neoplasms in 2017

The following changes were introduced in 2017 WHO and TnM classifications: 1. a new group of well-differentiated neuroendocrine tumors with the proliferation index of more than 20% and mitotic count of 20 per 10 hpf (NET G3), formerly classified under neuroendocrine carcinomas (NEC G3) 2. the division of poorly differentiated neuroendocrine tumors (PD NET) with the Ki-67 index of more than 20% into two groups in terms of the degree of differentiation and prognosis: NET G3 and NEC. 3. the replacement of MANEC with MINEN within the mixed group 4. the verification of histological grading (G) criteria 5. new TNM staging criteria, based on ENETS guidelines.

Treatment of metastatic pancreatic neuroendocrine tumors: relevance of ENETS 2016 guidelines.

The choice of first-line treatment for metastatic pancreatic neuroendocrine tumors (mP-NET) is mainly based on prognostic factors. ENETS-2016 guidelines stratified treatment according to 3 groups: Group 1, patients in whom all lesions could be removed; Group 2, patients with Ki67 <10%, low tumor burden, no symptoms and stable disease, for whom a watch-and-wait strategy or somatostatin analogs are proposed; Group 3, symptomatic patients or with Ki67 >10% or significant tumor burden or progressive disease, for whom a systemic chemotherapy is proposed. This retrospective study aimed to determine patient distribution, characteristics and outcome among these 3 groups. Patients with mP-NET diagnosis from 2004 to 2016 were categorized into the three groups. Prognosis was calculated using the Kaplan-Meier method. All treatments were recorded, and consistency with ENETS guidelines was explored. 104 patients were analyzed: 64% synchronous mP-NET, 80% grade 2 tumors and median overall survival (OS) of 104 (95% CI: 65-143) months. There were 15 patients in ENETS Group 1, 16 in Group 2 and 73 in Group 3. Median OS was not reached in Groups 1 and 2 and was 64 months (35-93) in Group 3. High liver tumor volume, high-grade tumor and progressive disease were associated with worse OS in multivariate analysis. The first-line treatment was in accordance with guidelines in 82%. 77% percent of deceased patients received less than 4 lines of treatment. Most patients are in Group 3 and do not receive all available treatments. Thus, trials are warranted to improve first-line chemotherapy. Alternative treatments may be considered for less aggressive disease.

75IN - Tools to Evaluate Tumour Response to Therapy: Imaging

ABSTRACT With neuroendocrine tumor, liver metastases are by far the most common site of recurrence of the disease. Liver mets pattern are now better known (hypervascular 60-83%, hypovascular 5%, and micronodular 22%). In the detection of liver metastases, MRI is more sensitive that CT scan, and adding diffuse weighted sequences to MRI can improve this sensitivity to 78–80%. Once detected, current ENETS guidelines recommend a staged approach to the management of liver metastases, depending on liver extend. If only one lobe is affected, surgery should be considered. If one lobe is predominantly affected, surgery and local ablative treatment are recommended. When a diffuse pattern is present, medical therapy, systemic chemotherapy and endovascular therapy are recommended, along with molecular targeted therapy. Then it is a challenge to evaluate tumour response due to these various therapies. Imaging can be effective in measuring reducing of tumour size, with RECIST. RECIST criteria, are accurate in assessing tumor progression but sometimes inaccurate in assessing tumor response after locoregional therapy or under molecular targeted therapy. There is poor correlation between tumor necrosis and conventional methods of response assessment, which poses questions of how best to quantify efficacy of targeted therapy. Choi criteria might also be considered as an alternative to RECIST to evaluate the effects of necrosis in patients with advanced well-differentiated neuroendocrine tumors because it takes in account necrosis. They are based on internal changes, tumor size (>10% decrease) OR tumor density (>15 HU decrease) Should we abandon RECIST? No, RECIST is still used in many clinical trials and is useful for assessing tumor progression. Then ENETS Modified Criteria are on study, associating RECIST and CHOI criteria. We probably should go back from RECIST 1.1 to RECIST, because RECIST 1.1 studies 2 targets per organ and Net liver mets have a large discrepancy of response. To choose 5 targets probably will lower discrepancy. Functional tools, mainly obtained with MR imaging, show interesting results but lack standardization. In the future, they will be combined to morphologic criteria Disclosure: All authors have declared no conflicts of interest.

Diagnosis and workup of 522 consecutive patients with neuroendocrine neoplasms in Switzerland

Neuroendocrine neoplasms (NENs) are difficult to diagnose. We used SwissNET data to characterise NEN patients followed in the two academic centres of western Switzerland (WS), and to compare them with patients followed in eastern Switzerland (ES) as well as with international guidelines. SwissNET is a prospective database covering data from 522 consecutive patients (285 men, 237 women) from WS (n = 99) and ES (n = 423). Mean ± SD age at diagnosis was 59.0 ± 15.7 years. Overall, 76/522 experienced a functional syndrome, with a median interval of 1.0 (IQR: 1.0-3.0) year between symptoms onset and diagnosis. A total of 51/522 of these tumours were incidental. The primary tumour site was the small intestine (29%), pancreas (21%), appendix (18%) and lung (11%) in both regions combined. In all, 513 functional imaging studies were obtained (139 in WS, 374 in ES). Of these, 381 were 111In-pentetreotide scintigraphies and 20 were 68Ga-DOTATOC PET. First line therapy was surgery in 87% of patients, medical therapy (biotherapy or chemotherapy) in 9% and irradiation in 3% for both regions together. Swiss NEN patients appear similar to what has been described in the literature. Imaging by somatostatin receptor scintigraphy (SRS) is widely used in both regions of Switzerland. In good accordance with published guidelines, data on first line therapy demonstrate the crucial role of surgery. The low incidence of biotherapy suggests that long-acting somatostatin analogues are not yet widely used for their anti-proliferative effects. The SwissNET initiative should help improve compliance with ENETS guidelines in the workup and care of NEN patients.

Neuroendokrine Tumoren des Verdauungstrakts – Daten des deutschen NET-Registers

Neuroendocrine tumours (NET) are rare and heterogeneous neoplasia. To obtain valid data on epidemiology, diagnostics, therapy, prognosis and risk factors is the aim of the German NET registry. Data from 2009 histologically proven NET were collected from 35 NET centres between 1999 and 2010. Data collection has been performed prospectively since 2004. Results: Median follow-up was 34.5 months and median age at diagnosis 56.4 years. Primary tumour localisations were pancreas (34.2%), midgut (5.8%), stomach (6.5%), bowel (6.9%), duodenum (4.8%) and neuroendocrine CUP (12.6%). Synchronous metastases were seen in 46% and second malignancies in 12%. From 860 patients, 402 (46.7%) had functional tumours with the following hormone excess syndromes: carcinoid syndrome (19.1%; n = 164), persistent hyperinsulinaemic hypoglycaemia (17.7%; n = 152), Zollinger- Ellison syndrome (7.1%; n = 61), glucagonoma (0.7%; n = 15), Verner-Morrison syndrome (0.4%; n = 8) and somatostatinoma syndrome(0.1%; n = 2). Surgical therapy was performed in 78%, therapy with somatostatin receptor analogues(SSA) in 28%, peptide radioreceptor therapy (PRRT) in 19%, chemotherapy in 18% and interferon therapy in 6.5%. Only surgery was done in 47%, whereas 53% received a second therapy. General mortality rate during follow-up was 14.9%. The tumour-specific survival rates for 2, 5 and 10 years were 94, 85 and 70%. The 5-year survival is dependent on the surgical or non-surgical therapy (82 versus 61%, p < 0.001) and also on the primary tumour site (90/30% for midgut, 85/65% for pancreas, p < 0.001). Grading (G1, G2, G3) based on proliferation index Ki-67 recommended by the ENETS guidelines and WHO classification is highly correlated to the 5-year survival rate (88, 82, 33%, p < 0.001). The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.

Neuroendocrine gastroenteropancreatic tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Neuroendocrine gastroenteropancreatic tumours : ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumors: The Second Event and Some Final Considerations

Fifty-six experts active in the field of digestive endocrine tumors from 18 countries attended the second Consensus Conference. Attendees were invited on the basis of their acumen (proven publication track and clinical practice) in the field on NETs. The attendees represented all medical branches involved in managing patients with gastroenteropancreatic NETs. They were assigned to four working groups according to their specific clinical expertise: (1) Pathology and Genetics (11 participants); (2) Surgery (8 participants); (3) Imaging and Radiology (6 participants), and (4) Medicine and Clinical Pathology (31 participants). The complete list is provided at the end of this commentary, as well as at the end of each of the five following papers. The Conference was divided sequentially into five sessions devoted to specific topics on an anatomical basis (Small Intestine; Colon and Rectum; Appendix; Metastatic Liver, and Poorly-Differentiated Endocrine Carcinomas of Hindgut and Midgut Origin). The Organizing Committee (the four front authors) prepared in advance a working booklet, reporting the text of the ENETS guidelines with specific questions for different working groups. The booklet was provided to all the participants at the conference venue. The work was organized such that, after a short case presentation in a plenary session, each working group The Second Event

Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumors: Why Such Guidelines and How We Went about It

Sixty-two experts active in the field of digestive NETs from 20 different countries attended the first Consensus Conference. Attendees were invited on the basis of their proven expert scientific and clinical experience in NETs. The attendees represented all medical disciplines involved in managing patients with digestive NETs. They were assigned to four working groups according to their specific clinical expertise: (1) Pathology and Genetics (11 participants); (2) Surgery (10 participants); (3) Imaging and Radiology (10 participants), and (4) Medicine and Clinical Pathology (31 participants). The complete list of delegates is provided at the end of this commentary, as well as at the end of each of the six following papers. The Conference was divided sequentially into 8 sessions devoted to specific topics on an anatomical basis (Gastric NET, Sessions 1 and 2; Duodenal NET; Pancreatic NET, Sessions 1–4; Poorly Differentiated Endocrine Carcinomas of foregut origin). A working booklet was prepared in advance by the organizing committee, using as a basis the published text of the ENETS Guidelines so that specific questions could be prepared and presented to different working groups. The booklet was provided to the participants only at the conference venue. The Starting Point