Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids

Martyn E. Caplin, E. Baudin, P. Ferolla, P. Filosso, M. Garcia-Yuste, E. Lim, K. Oberg, G. Pelosi, A. Perren, R. E. Rossi, W. D. Travis, Detlief Bartsch, Jaume Capdevila, Frederico Costa, Jaroslaw Cwikla, Wouter de Herder, Gianfranco Delle Fave, Barbro Eriksson, Massimo Falconi, Diego FeroneDavid Gross, Ashley Grossman, Tetsuhide Ito, Robert Jensen, Gregory Kaltsas, Fahrettin Kelestimur, Reza Kianmanesh, Ulrich Knigge, Beata Kos-Kudla, Eric Krenning, Emmanuel Mitry, Marianne Nicolson, Juan O'Connor, Dermot O'Toole, Ulrich Frank Pape, Marianne Pavel, John Ramage, Eric Raymond, Guido Rindi, Andrea Rockall, Philippe Ruszniewski, Ramon Salazar, Aldo Scarpa, Eva Sedlackova, Anders Sundin, Christos Toumpanakis, Marie Pierre Vullierme, Wolfgang Weber, Bertram Wiedenmann, Zeng Zheng-Pei

Research output: Contribution to journalArticle

226 Citations (Scopus)

Abstract

Background: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. Patients and methods: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. Results: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Conclusions: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up.

Original languageEnglish
Pages (from-to)1604-1620
Number of pages17
JournalAnnals of Oncology
Volume26
Issue number8
DOIs
Publication statusPublished - 2015

Fingerprint

Neuroendocrine Tumors
Carcinoid Tumor
Practice Guidelines
Lung
temozolomide
Therapeutics
Chromogranin A
Neoplasms
Somatostatin Receptors
Etoposide
Somatostatin
Platinum
PubMed

Keywords

  • Atypical carcinoid
  • Bronchial
  • Carcinoid
  • Neuroendocrine tumor
  • Pulmonary
  • Typical carcinoid

ASJC Scopus subject areas

  • Oncology
  • Hematology

Cite this

Pulmonary neuroendocrine (carcinoid) tumors : European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. / Caplin, Martyn E.; Baudin, E.; Ferolla, P.; Filosso, P.; Garcia-Yuste, M.; Lim, E.; Oberg, K.; Pelosi, G.; Perren, A.; Rossi, R. E.; Travis, W. D.; Bartsch, Detlief; Capdevila, Jaume; Costa, Frederico; Cwikla, Jaroslaw; Herder, Wouter de; Fave, Gianfranco Delle; Eriksson, Barbro; Falconi, Massimo; Ferone, Diego; Gross, David; Grossman, Ashley; Ito, Tetsuhide; Jensen, Robert; Kaltsas, Gregory; Kelestimur, Fahrettin; Kianmanesh, Reza; Knigge, Ulrich; Kos-Kudla, Beata; Krenning, Eric; Mitry, Emmanuel; Nicolson, Marianne; O'Connor, Juan; O'Toole, Dermot; Pape, Ulrich Frank; Pavel, Marianne; Ramage, John; Raymond, Eric; Rindi, Guido; Rockall, Andrea; Ruszniewski, Philippe; Salazar, Ramon; Scarpa, Aldo; Sedlackova, Eva; Sundin, Anders; Toumpanakis, Christos; Vullierme, Marie Pierre; Weber, Wolfgang; Wiedenmann, Bertram; Zheng-Pei, Zeng.

In: Annals of Oncology, Vol. 26, No. 8, 2015, p. 1604-1620.

Research output: Contribution to journalArticle

Caplin, ME, Baudin, E, Ferolla, P, Filosso, P, Garcia-Yuste, M, Lim, E, Oberg, K, Pelosi, G, Perren, A, Rossi, RE, Travis, WD, Bartsch, D, Capdevila, J, Costa, F, Cwikla, J, Herder, WD, Fave, GD, Eriksson, B, Falconi, M, Ferone, D, Gross, D, Grossman, A, Ito, T, Jensen, R, Kaltsas, G, Kelestimur, F, Kianmanesh, R, Knigge, U, Kos-Kudla, B, Krenning, E, Mitry, E, Nicolson, M, O'Connor, J, O'Toole, D, Pape, UF, Pavel, M, Ramage, J, Raymond, E, Rindi, G, Rockall, A, Ruszniewski, P, Salazar, R, Scarpa, A, Sedlackova, E, Sundin, A, Toumpanakis, C, Vullierme, MP, Weber, W, Wiedenmann, B & Zheng-Pei, Z 2015, 'Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids', Annals of Oncology, vol. 26, no. 8, pp. 1604-1620. https://doi.org/10.1093/annonc/mdv041
Caplin, Martyn E. ; Baudin, E. ; Ferolla, P. ; Filosso, P. ; Garcia-Yuste, M. ; Lim, E. ; Oberg, K. ; Pelosi, G. ; Perren, A. ; Rossi, R. E. ; Travis, W. D. ; Bartsch, Detlief ; Capdevila, Jaume ; Costa, Frederico ; Cwikla, Jaroslaw ; Herder, Wouter de ; Fave, Gianfranco Delle ; Eriksson, Barbro ; Falconi, Massimo ; Ferone, Diego ; Gross, David ; Grossman, Ashley ; Ito, Tetsuhide ; Jensen, Robert ; Kaltsas, Gregory ; Kelestimur, Fahrettin ; Kianmanesh, Reza ; Knigge, Ulrich ; Kos-Kudla, Beata ; Krenning, Eric ; Mitry, Emmanuel ; Nicolson, Marianne ; O'Connor, Juan ; O'Toole, Dermot ; Pape, Ulrich Frank ; Pavel, Marianne ; Ramage, John ; Raymond, Eric ; Rindi, Guido ; Rockall, Andrea ; Ruszniewski, Philippe ; Salazar, Ramon ; Scarpa, Aldo ; Sedlackova, Eva ; Sundin, Anders ; Toumpanakis, Christos ; Vullierme, Marie Pierre ; Weber, Wolfgang ; Wiedenmann, Bertram ; Zheng-Pei, Zeng. / Pulmonary neuroendocrine (carcinoid) tumors : European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. In: Annals of Oncology. 2015 ; Vol. 26, No. 8. pp. 1604-1620.
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title = "Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids",
abstract = "Background: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. Patients and methods: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. Results: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80{\%} of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Conclusions: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up.",
keywords = "Atypical carcinoid, Bronchial, Carcinoid, Neuroendocrine tumor, Pulmonary, Typical carcinoid",
author = "Caplin, {Martyn E.} and E. Baudin and P. Ferolla and P. Filosso and M. Garcia-Yuste and E. Lim and K. Oberg and G. Pelosi and A. Perren and Rossi, {R. E.} and Travis, {W. D.} and Detlief Bartsch and Jaume Capdevila and Frederico Costa and Jaroslaw Cwikla and Herder, {Wouter de} and Fave, {Gianfranco Delle} and Barbro Eriksson and Massimo Falconi and Diego Ferone and David Gross and Ashley Grossman and Tetsuhide Ito and Robert Jensen and Gregory Kaltsas and Fahrettin Kelestimur and Reza Kianmanesh and Ulrich Knigge and Beata Kos-Kudla and Eric Krenning and Emmanuel Mitry and Marianne Nicolson and Juan O'Connor and Dermot O'Toole and Pape, {Ulrich Frank} and Marianne Pavel and John Ramage and Eric Raymond and Guido Rindi and Andrea Rockall and Philippe Ruszniewski and Ramon Salazar and Aldo Scarpa and Eva Sedlackova and Anders Sundin and Christos Toumpanakis and Vullierme, {Marie Pierre} and Wolfgang Weber and Bertram Wiedenmann and Zeng Zheng-Pei",
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TY - JOUR

T1 - Pulmonary neuroendocrine (carcinoid) tumors

T2 - European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids

AU - Caplin, Martyn E.

AU - Baudin, E.

AU - Ferolla, P.

AU - Filosso, P.

AU - Garcia-Yuste, M.

AU - Lim, E.

AU - Oberg, K.

AU - Pelosi, G.

AU - Perren, A.

AU - Rossi, R. E.

AU - Travis, W. D.

AU - Bartsch, Detlief

AU - Capdevila, Jaume

AU - Costa, Frederico

AU - Cwikla, Jaroslaw

AU - Herder, Wouter de

AU - Fave, Gianfranco Delle

AU - Eriksson, Barbro

AU - Falconi, Massimo

AU - Ferone, Diego

AU - Gross, David

AU - Grossman, Ashley

AU - Ito, Tetsuhide

AU - Jensen, Robert

AU - Kaltsas, Gregory

AU - Kelestimur, Fahrettin

AU - Kianmanesh, Reza

AU - Knigge, Ulrich

AU - Kos-Kudla, Beata

AU - Krenning, Eric

AU - Mitry, Emmanuel

AU - Nicolson, Marianne

AU - O'Connor, Juan

AU - O'Toole, Dermot

AU - Pape, Ulrich Frank

AU - Pavel, Marianne

AU - Ramage, John

AU - Raymond, Eric

AU - Rindi, Guido

AU - Rockall, Andrea

AU - Ruszniewski, Philippe

AU - Salazar, Ramon

AU - Scarpa, Aldo

AU - Sedlackova, Eva

AU - Sundin, Anders

AU - Toumpanakis, Christos

AU - Vullierme, Marie Pierre

AU - Weber, Wolfgang

AU - Wiedenmann, Bertram

AU - Zheng-Pei, Zeng

PY - 2015

Y1 - 2015

N2 - Background: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. Patients and methods: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. Results: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Conclusions: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up.

AB - Background: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. Patients and methods: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. Results: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Conclusions: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up.

KW - Atypical carcinoid

KW - Bronchial

KW - Carcinoid

KW - Neuroendocrine tumor

KW - Pulmonary

KW - Typical carcinoid

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