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1. ENETS standardized (synoptic) reporting for endoscopy in neuroendocrine tumors

Author

Borbath, I., Pape, U. -F., Deprez, P. H., Bartsch, D. K., Caplin, M., Falconi, M., Garcia-Carbonero, R., Grozinsky-Glasberg, S., Jensen, R. T., Arnold, R., Ruszniewski, P., Toumpanakis, C., Valle, J. W., O'Toole, D., Belli, S. H., Castano, J. P., Chen, J., Costa, F. P., Couvelard, A., de Herder, W. W., Deroose, C. M., Dromain, C., Faggiano, A., Falkerby, J., Fazio, N., Frilling, A., Grande, E., Hand, P., Hicks, R. J., Horsch, D., Howe, J. R., Kloppel, G., Kolarova, T., Kos-Kudla, B., Koumarianou, A., Krejs, G. J., Krenning, E. P., Krishna, B. A., Leyden, S., Masui, T., Niederle, B., Nieveen van Dijkum, E. J., Oberg, K., Pavel, M., Perren, A., Prasad, V., Ramage, J. K., Reed, N. S., Rindi, G., Gemelli, A., Rinke, A., Rothmund, M., Singh, S., Sundin, A., Velthuysen, M. F. V., Verslype, C., Vullierme, M. P., Welin, S., Wiedenmann, B., Zhao, H., Graduate School, Surgery, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, UCL - SSS/IREC/GAEN - Pôle d'Hépato-gastro-entérologie, UCL - (SLuc) Centre du cancer, UCL - (SLuc) Service de gastro-entérologie, and UCL - (SLuc) Unité d'oncologie médicale

Subjects
Cellular and Molecular Neuroscience, Neuroendocrine Tumors, Endocrinology, neuroendocrine neoplasms, Endocrine and Autonomic Systems, Endocrinology, Diabetes and Metabolism, Humans, Endoscopy, endoscopy, standardised reporting
Abstract

Despite efforts from various endoscopy societies, reporting in the field of endoscopy remains extremely heterogeneous. Harmonisation of clinical practice in endoscopy has been highlighted by application of many clinical practice guidelines and standards pertaining to the endoscopic procedures and reporting are underlined. The aim of the proposed "standardised reporting" is to (1) facilitate recognition of gastrointestinal neuroendocrine neoplasms (NEN) on initial endoscopy, (2) to enable interdisciplinary decision making for treatment by a multidisciplinary team, (3) to provide a basis for a standardised endoscopic follow-up which allows detection of recurrence or progression reliably, (4) to make endoscopic reports on NEN comparable between different units, and (5) to allow research collaboration between NEN centres in terms of consistency of their endoscopic data. The ultimate goal is to improve disease management, patient outcome and reduce the diagnostic burden on the side of the patient by ensuring the highest possible diagnostic accuracy and validity of endoscopic exams and possibly interventions.

Published
2022

2. Unmet medical needs in pulmonary neuroendocrine (Carcinoid) neoplasms

Author

Baudin, E. Hayes, A.R. Scoazec, J.-Y. Filosso, P.L. Lim, E. Kaltsas, G. Frilling, A. Chen, J. Kos-Kudła, B. Gorbunova, V. Wiedenmann, B. Nieveen Van Dijkum, E. Cwikła, J.B. Falkerby, J. Valle, J.W. Kulke, M.H. Caplin, M.E.

Abstract

Pulmonary carcinoids (PCs) display the common features of all well-differentiated neuroendocrine neoplasms (NEN) and are classified as low- A nd intermediate-grade malignant tumours (i.e., typical and atypical carcinoid, respectively). There is a paucity of randomised studies dedicated to advanced PCs and management principles are drawn from the larger gastroenteropancreatic NEN experience. There is growing evidence that NEN anatomic subgroups have different biology and different responses to treatment and, therefore, should be investigated as separate entities in clinical trials. In this review, we discuss the existing evidence and limitations of tumour classification, diagnostics and staging, prognostication, and treatment in the setting of PC, with focus on unmet medical needs and directions for the future. © 2018 S. Karger AG, Basel. All rights reserved.

Published
2019

3. Unmet medical needs in metastatic lung and digestive neuroendocrine neoplasms

Author

Capdevila, Jaume, Bodei, Lisa, Davies, Philippa, Gorbounova, Vera, Jensen, Robert T., Knigge, Ulrich P., Krejs, Guenter J., Krenning, Eric, O'Connor, Juan Manuel, Peeters, Marc, Rindi, Guido, Salazar, Ramon, Vullierme, Marie-Pierre, Pavel, Marianne E., Arnold, R., Bartsch, D., Baudin, E., Bodei, L., Borbath, I, Capdevila, J., Caplin, M., Chen, J., Costa, F., Couvelard, A., Cwikla, J. B., Davies, P., de Herder, W. W., Falconi, M., Falkerby, J., Fazio, N., Ferone, D., Frilling, A., Garcia-Carbonero, R., Glasberg, S., Gorbunova, V, Grossman, A., Hoersch, D., Jensen, R. T., Kaltsas, G., Kloeppel, G., Knigge, U. P., Kos-Kudla, B., Krejs, G. J., Krenning, E., Kulke, M., Lamberts, S. W. J., van Dijkum, Nieveen E., O'Connor, J. M., O'Toole, D., Pape, U. F., Partelli, S., Pavel, M. E., Ramage, J., Reed, N. Simon Beatson, Rindi, G., Rinke, A., Ruszniewski, P., Sorbye, H., Sundin, A., Scoazec, J. Y., Taal, B. G., Tiensuu, J. E., Toumpanakis, C., Valle, J., Vullierme, M. P., Welin, S., Wiedenmann, B., Capdevila, J, Bodei, L, Davies, P, Gorbounova, V, Jensen, Rt, Knigge, Up, Krejs, Gj, Krenning, E, O'Connor, Jm, Peeters, M, Rindi, G, Salazar, R, Vullierme, Mp, Pavel, Me, on behalf the ENETS 2016 Munich Advisory Board, Participant, Partelli, S, Falconi, M, Erasmus MC other, Internal Medicine, ENETS 2016 Munich Advisory Board, and Dr. Heinz-Horst Deichmann Stiftung

Subjects
Biomedical Research, Lung Neoplasms, Endocrinology, Diabetes and Metabolism, Metastasi, Lanreotide, Digestive System Neoplasms, Metastasis, 030218 nuclear medicine & medical imaging, Imaging, chemistry.chemical_compound, EVEROLIMUS, 0302 clinical medicine, Endocrinology, LANREOTIDE, Treatment options, TUMORS, Clinical Practice, Neuroendocrine Tumors, Unmet medical needs, SURVIVAL, ENETS 2016 Munich Advisory Board Participants, Life Sciences & Biomedicine, medicine.medical_specialty, 030209 endocrinology & metabolism, SUNITINIB, Endocrinology & Metabolism, 03 medical and health sciences, Cellular and Molecular Neuroscience, Drug Development, Internal medicine, medicine, Biomarkers, Tumor, Humans, Limited evidence, Intensive care medicine, Science & Technology, Endocrine and Autonomic Systems, business.industry, Neurosciences, 1103 Clinical Sciences, Biomarker, Neuroendocrine neoplasm, chemistry, Neuroendocrine neoplasms, Neurosciences & Neurology, Human medicine, Therapy, 1109 Neurosciences, business, Who classification, Biomarkers
Abstract

Unmet medical needs are not infrequent in oncology, and these needs are usually of higher magnitude in rare cancers. The field of neuroendocrine neoplasms (NENs) has evolved rapidly during the last decade, and, currently, a new WHO classification is being implemented and several treatment options are available in the metastatic setting after the results of prospective phase III clinical trials. However, several questions are still unanswered, and decisions in our daily clinical practice should be made with limited evidence. In the 2016 meeting of the advisory board of the European Neuroendocrine Tumor Society (ENETS), the main unmet medical needs in the metastatic NENs setting were deeply discussed, and several proposals to try to solve them are presented in this article, including biomarkers, imaging, and therapy.

Published
2019

4. Unmet Medical Needs in Metastatic Lung and Digestive Neuroendocrine Neoplasms

Author

Capdevila, Jaume Bodei, Lisa Davies, Philippa Gorbounova, Vera Jensen, Robert T. Knigge, Ulrich P. Krejs, Guenter J. and Krenning, Eric O'Connor, Juan Manuel Peeters, Marc and Rindi, Guido Salazar, Ramon Vullierme, Marie-Pierre Pavel, Marianne E. Arnold, R. Bartsch, D. Baudin, E. Bodei, L. and Borbath, I Capdevila, J. Caplin, M. Chen, J. Costa, F. Couvelard, A. Cwikla, J. B. Davies, P. de Herder, W. W. Falconi, M. Falkerby, J. Fazio, N. Ferone, D. and Frilling, A. Garcia-Carbonero, R. Glasberg, S. Gorbunova, V and Grossman, A. Hoersch, D. Jensen, R. T. Kaltsas, G. and Kloeppel, G. Knigge, U. P. Kos-Kudla, B. Krejs, G. J. and Krenning, E. Kulke, M. Lamberts, S. W. J. van Dijkum, Nieveen E. O'Connor, J. M. O'Toole, D. Pape, U. F. and Partelli, S. Pavel, M. E. Peeters, M. Ramage, J. Reed, N. Simon Beatson Rindi, G. Rinke, A. Ruszniewski, P. and Sorbye, H. Sundin, A. Scoazec, J. Y. Taal, B. G. and Tiensuu, J. E. Toumpanakis, C. Valle, J. Vullierme, M. P. and Welin, S. Wiedenmann, B. ENETS 2016 Munich Advisory Board

Abstract

Unmet medical needs are not infrequent in oncology, and these needs are usually of higher magnitude in rare cancers. The field of neuroendocrine neoplasms (NENs) has evolved rapidly during the last decade, and, currently, a new WHO classification is being implemented and several treatment options are available in the metastatic setting after the results of prospective phase III clinical trials. However, several questions are still unanswered, and decisions in our daily clinical practice should be made with limited evidence. In the 2016 meeting of the advisory board of the European Neuroendocrine Tumor Society (ENETS), the main unmet medical needs in the metastatic NENs setting were deeply discussed, and several proposals to try to solve them are presented in this article, including biomarkers, imaging, and therapy.

Published
2019

5. ENETS Consensus Recommendations for the Standards of Care in Neuroendocrine Neoplasms: Follow-Up and Documentation

Author

Knigge, U, Capdevila, J, Bartsch, D K, Baudin, E, Falkerby, J, Kianmanesh, R, Kos-Kudla, B, Niederle, B, Nieveen van Dijkum, E, O'Toole, D, Pascher, A, Reed, N, Sundin, A, Vullierme, M-P, Ferone, D, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, CCA -Cancer Center Amsterdam, Other Research, and Surgery

Subjects
medicine.medical_specialty, Endocrinology, Diabetes and Metabolism, MEDLINE, TNM staging, 030209 endocrinology & metabolism, 610 Medicine & health, 5-Hydroxyindoleacetic acid, Neuron specific enolase, 03 medical and health sciences, Cellular and Molecular Neuroscience, 0302 clinical medicine, Endocrinology, Documentation, Neuroendocrine tumor, Internal medicine, medicine, Neuroendocrine carcinoma, Neuroendocrine neoplasm, Follow up, Chromogranin A, NT-pro-brain natriuretic peptide, Functional imaging, Somatostatin receptor imaging, Endocrine and Autonomic Systems, business.industry, 030220 oncology & carcinogenesis, TNM Staging, 570 Life sciences, biology, business
Abstract

ENETS consensus recommendations for the standards of care in neuroendocrine neoplasms (NEN) concerning follow-up and documentation are considered in this review. The documentation of patients with NEN should include the most relevant data characterizing an individual patient from the first contact with his/her physician/hospital until his/her last presentation during follow-up. It is advocated that follow-up occurs in specialized NEN centers with regular NEN tumor boards with expert panels. The follow-up should be in accordance with the ENETS consensus guidelines from 2011 and 2016, the present and coming WHO classification and ENETS/UICC recommendations for TNM staging. The recommendations for follow-up in patients with thymic, bronchopulmonary and gastroenteropancreatic NEN are given in Table 1. However, it should be stressed that evidence-based studies for follow-up are largely missing. (C) 2017 S. Karger AG, Basel

Published
2017
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6. ENETS Consensus Recommendations for the Standards of Care in Neuroendocrine Neoplasms:Follow-Up and Documentation

Author

Knigge, U., Capdevila, J., Bartsch, D. K., Baudin, E., Falkerby, J., Kianmanesh, R., Kos-Kudla, B., Niederle, B., Nieveen Van Dijkum, E., O'Toole, D., Pascher, A., Reed, N., Sundin, A., Vullierme, M. P., Knigge, U., Capdevila, J., Bartsch, D. K., Baudin, E., Falkerby, J., Kianmanesh, R., Kos-Kudla, B., Niederle, B., Nieveen Van Dijkum, E., O'Toole, D., Pascher, A., Reed, N., Sundin, A., and Vullierme, M. P.

Abstract

ENETS consensus recommendations for the standards of care in neuroendocrine neoplasms (NEN) concerning follow-up and documentation are considered in this review. The documentation of patients with NEN should include the most relevant data characterizing an individual patient from the first contact with his/her physician/hospital until his/her last presentation during follow-up. It is advocated that follow-up occurs in specialized NEN centers with regular NEN tumor boards with expert panels. The follow-up should be in accordance with the ENETS consensus guidelines from 2011 and 2016, the present and coming WHO classification and ENETS/UICC recommendations for TNM staging. The recommendations for follow-up in patients with thymic, bronchopulmonary and gastroenteropancreatic NEN are given in Table 1. However, it should be stressed that evidence-based studies for follow-up are largely missing.

Published
2017

7. Long-Term Stable Disease with Everolimus in a Patient with Advanced Pancreatic Neuroendocrine Tumor Enrolled in RADIANT-1.

Author

Falkerby, J., Granberg, D., Eriksson, B., Skogseid, B., Öberg, K., Janson, E., Kozlovacki, G., and Welin, S.

Subjects
*NEUROENDOCRINE tumors, *DRUG therapy, *GLUCAGONOMA, *LIVER diseases, *LIVER metastasis
Abstract

Introduction: The phase II open-label RADIANT-1 study evaluated the safety and efficacy of Everolimus (E, 10 mg/d) ± octreotide LAR (O) in patients (pts) with advanced pancreatic neuroendocrine tumors (pNET) after chemotherapy failure. Objective tumor response was 9.6% in the E group and 4.4% in the E+O group (all partial), and stable disease (SD) was 68% and 80%. Aim(s): We report long-term SD in a glucagonoma pt enrolled in RADIANT-1. Materials and methods: A man aged 46 was diagnosed with glucagonoma in 1990 with tumors in the pancreas (4 cm) and liver (5 cm): p-glucagon >1000 ng/L (ref, <140 ng/L), pancreatic polypeptide (PP)=170 pmol/L (ref, <100 pmol/L), Chromogranin A (CgA)=609 µg/L (ref, <350 µg/L). Pancreas, spleen, and liver metastases (met) were resected, and interferon-a 5x/wk was initiated. Recurrent disease was confirmed in Sept 1996 (CgA-/PP-/glucagon-positive liver tumor). Subsequent met were treated with surgery, radiofrequency ablation (RFA), and chemotherapy (streptozotocin+5-FU). In Feb 2007, multiple liver met not suitable for resection or RFA were identified; after 7 y, chemotherapy was stopped and the pt was enrolled in RADIANT-1 and received Everolimus alone. Results: While on Everolimus (since Feb 2007; 4 y, 9 mo), pt has had radiologically SD without side effects. At last visit, p-glucagon=319 pg/mL, and PP=826 pmol/L. Conclusion: A heavily pretreated patient with advanced metastatic pNET achieved SD lasting more than 4.5 y on Everolimus. Study supported by Novartis. [ABSTRACT FROM AUTHOR]

Published
2012

8. Unmet Medical Needs in Pulmonary Neuroendocrine (Carcinoid) Neoplasms

Author

Baudin, Eric, Hayes, Aimee R., Scoazec, Jean-Yves, Filosso, Pier Luigi, Lim, Eric, Kaltsas, Gregory, Frilling, Andrea, Chen, Jie, Kos-Kudla, Beata, Gorbunova, Vera, Wiedenmann, Bertram, van Dijkum, Els Nieveen, Cwikla, Jaroslaw B., Falkerby, Jenny, Valle, Juan W., Kulke, Matthew H., Caplin, Martyn E., Detlef, Bartsch, Rudolf, Arnold, Eric, Baudin, Lisa, Bodei, Ivan, Borbath, Jaume, Capdevila, Martyn, Caplin, Jie, Chen, Frederico, Costa, Anne, Couvelard, Jaroslaw, Cwikla B., Philippa, Davies, de Herder, Wouter W., Massimo, Falconi, Jenny, Falkerby, Nicola, Fazio, Diego, Ferone, Andrea, Frilling, Rocio, Garcia-Carbonero, Simona, Glasberg, Vera, Gorbunova, Ashley, Grossman, Dieter, Hoech, Robert, Jensen, Gregory, Kaltsas, Guenter, Kloeppel, Peter, Knigge Ulrich, Beata, Kos-Kudla, Guenter, Krejs J., Eric, Krenning, Matthew, Kulke, Steven, Lamberts W. J., van Elisabeth, Nieveen Dijkum, Juan Manuel, O'Connor, Dermot, O'Toole, Ulrich-Frank, Pape, Stefano, Partelli, Ellen, Pavel Marianne, Peeters, Marc, John, Ramage, Simon, Reed Nicholas, Guido, Rindi, Anja, Rinke, Philippe, Ruszniewski, Halfdan, Sorbye, Anders, Sundin, Jean-Yves, Scoazec, Babs, Taal G., Eva, Tiensuu Janson, Christos, Toumpanakis, Juan, Valle, Marie-Pierre, Vullierme, Staffan, Welin, Bertram, Wiedenmann, ENETS 2016 Munich Advisory Board, Internal Medicine, Baudin, E, Hayes, A R, Scoazec, J-Y, Filosso, Pl, Lim, E, Kaltsas, G, Frilling, A, Chen, J, Kos-Kudla, B, Gorbunova, V, Wiedenmann, B, Nieveen, Van Dijkum E, Cwikla, J B, Falkerby, J, Valle, J W, Kulke, M H, Caplin, M E, on behalf The ENETS 2016 Munich Advisory Board, Participant, Partelli, S, Falconi, M, and Dr. Heinz-Horst Deichmann Stiftung

Subjects
Oncology, Biomedical Research, Lung Neoplasms, SOMATOSTATIN RECEPTORS, Endocrinology, Diabetes and Metabolism, CHROMOGRANIN-A, 030218 nuclear medicine & medical imaging, 0302 clinical medicine, Endocrinology, PROGNOSTIC-FACTORS, PROLIFERATIVE INDEX, Prognosis, Neuroendocrine Tumors, Neuroendocrine Tumors/classification, CUSHINGS-SYNDROME, Tumour classification, ENETS 2016 Munich Advisory Board Participants, Life Sciences & Biomedicine, medicine.medical_specialty, Carcinoid Tumor/classification, 030209 endocrinology & metabolism, Carcinoid Tumor, ENETS CONSENSUS GUIDELINES, 03 medical and health sciences, Cellular and Molecular Neuroscience, Endocrinology & Metabolism, Biomedical Research/trends, Internal medicine, Pulmonary carcinoids, medicine, Humans, EUROPEAN ASSOCIATION, Science & Technology, Endocrine and Autonomic Systems, CLINICAL-FEATURES, Bronchial carcinoid tumour, Neurosciences, 1103 Clinical Sciences, ECTOPIC ACTH SYNDROME, Lung Neoplasms/classification, Clinical trial, NET, Neuroendocrine neoplasm, Bronchial carcinoid tumours, Ectopic ACTH syndrome, Neuroendocrine neoplasms, Neurosciences & Neurology, RECEPTOR RADIONUCLIDE THERAPY, Human medicine, Management principles, Atypical carcinoid, 1109 Neurosciences
Abstract

Pulmonary carcinoids (PCs) display the common features of all well-differentiated neuroendocrine neoplasms (NEN) and are classified as low- and intermediate-grade malignant tumours (i.e., typical and atypical carcinoid, respectively). There is a paucity of randomised studies dedicated to advanced PCs and management principles are drawn from the larger gastroenteropancreatic NEN experience. There is growing evidence that NEN anatomic subgroups have different biology and different responses to treatment and, therefore, should be investigated as separate entities in clinical trials. In this review, we discuss the existing evidence and limitations of tumour classification, diagnostics and staging, prognostication, and treatment in the setting of PC, with focus on unmet medical needs and directions for the future.

Published
2019

9. Clinical management of typical and atypical carcinoids/neuroendocrine tumors in ENETS centres of excellence (CoE): Survey from the ENETS lung NET task force.

Author

Koumarianou A, Filosso PL, Bodei L, Castano JP, Fernandez-Cuesta L, Deroose CM, Foll M, Dromain C, Reed NS, Caplin M, Capdevila J, Falkerby J, Faggiano A, Frilling A, Grande E, Hicks RJ, Kasajima A, Kos-Kudla B, Krishna BA, Lim E, Rinke A, Singh S, Thirlwell C, Volante M, and Walter T

Subjects
Humans, Surveys and Questionnaires, Advisory Committees, Disease Management, Carcinoid Tumor therapy, Carcinoid Tumor pathology, Carcinoid Tumor diagnosis, Lung Neoplasms therapy, Lung Neoplasms pathology, Lung Neoplasms diagnosis, Neuroendocrine Tumors therapy, Neuroendocrine Tumors diagnosis, Neuroendocrine Tumors pathology, Neuroendocrine Tumors epidemiology
Abstract

Lung carcinoid tumours are neuroendocrine neoplasms originating from the bronchopulmonary tract's neuroendocrine cells, accounting for only 1%-3% of all lung cancers but 30% of all neuroendocrine tumours. The incidence of lung carcinoids, both typical and atypical, has been increasing over the years due to improved diagnostic methods and increased awareness among clinicians and pathologists. The most recent WHO classification includes a subgroup of lung carcinoids with atypical morphology and higher mitotic count and/or Ki67 labelling index. Despite appropriate surgery, the 5-year survival rate for atypical carcinoids barely exceeds 50%-70%. The role of adjuvant therapy in lung carcinoids is not well-defined, and clinical decisions are generally based on the presence of high-risk features. Long-term follow-up is essential to monitor for recurrence, although the optimal follow-up protocol remains unclear. To address the lack of consensus in clinical management decisions, the European Neuroendocrine Tumor Society (ENETS) initiated a survey among 20 expert centres. The survey identified varied opinions on approaches to imaging, surgery, use of adjuvant therapy, and follow-up protocols. Notably, the absence of dedicated multidisciplinary lung neuroendocrine tumour boards in some centres was evident. Experts agreed on the need for a prospective adjuvant trial in high-risk patients, emphasizing the feasibility of such a study. In conclusion, the study highlights the need for a more uniform adoption of existing guidelines in the management of lung carcinoid tumours and emphasizes the importance of international collaboration to advance research and patient care. Close collaboration between healthcare providers and patients is vital for effective long-term surveillance and management of these rare tumours., (© 2024 British Society for Neuroendocrinology.)

Published
2024
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