Ramon Salazar

Gastroenteropancreatic neuroendocrine tumours

Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics.

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms of the Digestive System: Well-Differentiated Pancreatic Non-Functioning Tumors

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms of the Digestive System : Well-Differentiated Pancreatic Non-Functioning Tumors

Consensus guidelines for the management of patients with liver metastases from digestive (neuro)endocrine tumors: Foregut, midgut, hindgut, and unknown primary

a DRK Kliniken Westend, Berlin , Germany; b UFR Bichat-Beaujon-Louis Mourier, Service de Chirurgie Digestive, Hopital Louis Mourier, Colombes , France; c Medicine and Surgery, General Surgery Section, MED/18 – General Surgery and d Department of Pathology, University of Verona, Verona , Italy; e Netherlands Cancer Centre, Amsterdam , and f Department of Nuclear Medicine, Erasmus University Medical Center, Rotterdam , The Netherlands;

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Towards a Standardized Approach to the Diagnosis of Gastroenteropancreatic Neuroendocrine Tumors and Their Prognostic Stratification

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors : towards a standardized approach to the diagnosis of gastroenteropancreatic neuroendocrine tumors and their prognostic stratification

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

a Division of Hepatology and Gastroenterology, Department of Internal Medicine, Campus Virchow-Klinikum, Charite-Universitatsmedizin Berlin, Berlin , Germany; b Department of Pathology, Universitatsspital, Zurich , Switzerland; c Division of General Surgery, Department of Surgery, Medical University of Vienna, Vienna , Austria; d Department of Endocrinology and Metabolism, Hadassah University Hospital, Jerusalem , Israel; e Department of Internal Medicine, Philipps University, Marburg , Germany; f Hopital Sirio Libanes, Centro de Oncologia, Sao Paulo , Brazil; g Department of Radiology, Campus Virchow-Klinikum, Charite, University Medicine Berlin, Berlin , Germany; h Department of Oncology, Institut Catala d’Oncologia (IDIBELL), Barcelona , Spain; i Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford , UK

ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Somatostatin receptor imaging with IIIIn-pentetreotide

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors : Somatostatin Receptor Imaging with In-111-Pentetreotide

Rare functioning pancreatic endocrine tumors.

a Department of Gastroenterology, Beaujon Hospital, Clichy , France; b Department of Oncology, Institut Catala d’Oncologia, Barcelona , Spain; c Department of Surgery, Verona University, Verona , Italy; d Department of Endocrinology and Metabolism, Genimatas Hospital, Athens , Greece; e Department of Gastroenterology, Beaujon Hospital, Clichy , France; f Department of Endocrinology, Erasmus MC University, Rotterdam , The Netherlands; g Department of Internal Medicine, Martin University, Martin , Slovakia; h Department of Propaedeutic Internal Medicine, Laiko Hospital, Athens , Greece; i Department of Nuclear Medicine, Erasmus MC University, Rotterdam , The Netherlands; j Department of Gastroenterology, Beaujon Hospital, Clichy , France; k Department of Gastroenterology, Royal Free Hospital, London , UK; l Department of Cell Biology, National Institute of Health, Bethesda, Md. , USA; m Department of Endocrinology, University Hospital, Uppsala , Sweden

Consensus guidelines for the management of patients with digestive neuroendocrine tumours: Well-differentiated colon and rectum tumour/carcinoma

a Department of Gastroenterology, North Hampshire Hospital, Basingstoke , UK; b Stadtisches Klinikum Neuss, Lukaskrankenhaus, Chirurgische Klinik I, Neuss , Germany; c Istituto di Radiologia, Policlinco GB Rossi, Verona , Italy; d Institute for Pathology, Kantonsspital, Baden , Switzerland; e Departments of Internal Medicine and Endocrinological and Metabolic Sciences, University of Genoa, Genoa , Italy; f II. Internal Medical Department, University Hospital Martin, Martin , Slovakia; g G. Genimatas Hospital, Athens , Greece; h Erciyes University Medical School, Department of Endocrinology and Metabolism, Kayseri , Turkey; i H. Lee Moffitt Cancer Center/ University of South Florida, Tampa, Fla. , USA; j Anatomie Pathologique, Hopital Edouard Herriot, Lyon , France;

Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumours: Well-Differentiated Tumour/Carcinoma of the Appendix and Goblet Cell Carcinoma

Consensus guidelines for the management of patients with digestive neuroendocrine tumours : well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Follow-Up and Documentation

Summary of follow-up recommendations in patients with benign and malignant neuroendocrine tumorsFollow-upyes/no endoscopy US/CT/MRI Octreoscan CgABenign insulinoma noType 1 gastric carcinoid yes yearlyRectal carcinoid no (if completely resected)Appendiceal carcinoid T1 noAppendiceal carcinoid T2 ? (see text)Resectable tumor (uncertain behavior)G1 every 6–12 months yes(gastric carc.)yes every 2 years 2 yes 1 Resectable malignant tumor with/without nodal involvementG1 every 6–12 months yes every 2 years 2 yes 1 G2 every 6 months yes yearly 2 yes 1 G3 every 3 months yes yearly 2 yes 3 Non-resectable malignant tumor with/without nodal involvement and/or liver and other metastasesG1 every 6–12 months yes every 2 years 2 yesG2 every 6 months yes yearly 2 yesG3 every 3 months yes yearly 2 yes 31 Only in the presence of a visible tumor. 2 Recommendations regarding the time frames of Octreoscan should be adjusted to the individual situation. 3 In poorly differentiated tumors and negative CgA NSE may act as a suitable marker.