The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Medard F M Van Den Broek; Joanne M De Laat; Rachel S Van Leeuwaarde; Annenienke C Van De Ven; Wouter W De Herder; Olaf M Dekkers; Madeleine L Drent; Michiel N Kerstens; Peter H Bisschop; Bas Havekes; Wenzel M Hackeng; Lodewijk A A Brosens; Menno R Vriens; Wieneke A Buikhuisen; Gerlof D Valk

doi:10.1210/clinem/dgaa800

The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Medard F M Van Den Broek, Joanne M De Laat, Rachel S Van Leeuwaarde, Annenienke C Van De Ven, Wouter W De Herder, Olaf M Dekkers, Madeleine L Drent, Michiel N Kerstens, Peter H Bisschop, Bas Havekes, Wenzel M Hackeng, Lodewijk A A Brosens, Menno R Vriens, Wieneke A Buikhuisen, Gerlof D Valk

Guided Treatment in Optimal Selected Cancer Patients (GUTS)

Research output: Contribution to journal › Article › Academic › peer-review

15 Citations (Scopus)

162 Downloads (Pure)

Abstract

INTRODUCTION: Multiple Endocrine Neoplasia type 1 (MEN1)-related neuroendocrine tumors (NETs) of the lung are mostly indolent with a good prognosis. Nevertheless, cases of aggressive lung NET do occur, and therefore the management of individual patients is challenging.

AIM: To assess tumor growth and survival of patients with MEN1-related lung NETs at long-term follow-up.

METHODS: The population based Dutch MEN1 Study Group (DMSG) database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.

RESULTS: In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 64.6% - 94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A>G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after six years of indolent disease, presumably explaining the sudden change in course.

CONCLUSION: MEN1-related lung NETs are slow-growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.

Original language	English
Pages (from-to)	E1014-E1027
Number of pages	14
Journal	Journal of Clinical Endocrinology and Metabolism
Volume	106
Issue number	2
Early online date	2-Nov-2020
DOIs	https://doi.org/10.1210/clinem/dgaa800
Publication status	Published - Feb-2021

Keywords

multiple endocrine neoplasia type 1
lung NET
tumor growth
survival
surveillance

Access to Document

10.1210/clinem/dgaa800

The management of neuroendocrine tumors of the lung in MEN1 results from the Dutch MEN1 Study GroupFinal publisher's version, 819 KBLicence: Taverne

Handle.net

Persistent link

Cite this

Van Den Broek, M. F. M., De Laat, J. M., Van Leeuwaarde, R. S., Van De Ven, A. C., De Herder, W. W., Dekkers, O. M., Drent, M. L., Kerstens, M. N., Bisschop, P. H., Havekes, B., Hackeng, W. M., Brosens, L. A. A., Vriens, M. R., Buikhuisen, W. A., & Valk, G. D. (2021). The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group. Journal of Clinical Endocrinology and Metabolism, 106(2), E1014-E1027. https://doi.org/10.1210/clinem/dgaa800

@article{85d8263596bd4861ab6b4cd9a44ca8e8,

title = "The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group",

abstract = "INTRODUCTION: Multiple Endocrine Neoplasia type 1 (MEN1)-related neuroendocrine tumors (NETs) of the lung are mostly indolent with a good prognosis. Nevertheless, cases of aggressive lung NET do occur, and therefore the management of individual patients is challenging.AIM: To assess tumor growth and survival of patients with MEN1-related lung NETs at long-term follow-up.METHODS: The population based Dutch MEN1 Study Group (DMSG) database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.RESULTS: In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 64.6% - 94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A>G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after six years of indolent disease, presumably explaining the sudden change in course.CONCLUSION: MEN1-related lung NETs are slow-growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.",

keywords = "multiple endocrine neoplasia type 1, lung NET, tumor growth, survival, surveillance",

author = "{Van Den Broek}, {Medard F M} and {De Laat}, {Joanne M} and {Van Leeuwaarde}, {Rachel S} and {Van De Ven}, {Annenienke C} and {De Herder}, {Wouter W} and Dekkers, {Olaf M} and Drent, {Madeleine L} and Kerstens, {Michiel N} and Bisschop, {Peter H} and Bas Havekes and Hackeng, {Wenzel M} and Brosens, {Lodewijk A A} and Vriens, {Menno R} and Buikhuisen, {Wieneke A} and Valk, {Gerlof D}",

year = "2021",

month = feb,

doi = "10.1210/clinem/dgaa800",

language = "English",

volume = "106",

pages = "E1014--E1027",

journal = "Journal of Clinical Endocrinology and Metabolism",

issn = "0021-972X",

publisher = "ENDOCRINE SOC",

number = "2",

}

Van Den Broek, MFM, De Laat, JM, Van Leeuwaarde, RS, Van De Ven, AC, De Herder, WW, Dekkers, OM, Drent, ML, Kerstens, MN, Bisschop, PH, Havekes, B, Hackeng, WM, Brosens, LAA, Vriens, MR, Buikhuisen, WA & Valk, GD 2021, 'The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group', Journal of Clinical Endocrinology and Metabolism, vol. 106, no. 2, pp. E1014-E1027. https://doi.org/10.1210/clinem/dgaa800

TY - JOUR

T1 - The Management of Neuroendocrine Tumors of the Lung in MEN1

T2 - Results From the Dutch MEN1 Study Group

AU - Van Den Broek, Medard F M

AU - De Laat, Joanne M

AU - Van Leeuwaarde, Rachel S

AU - Van De Ven, Annenienke C

AU - De Herder, Wouter W

AU - Dekkers, Olaf M

AU - Drent, Madeleine L

AU - Kerstens, Michiel N

AU - Bisschop, Peter H

AU - Havekes, Bas

AU - Hackeng, Wenzel M

AU - Brosens, Lodewijk A A

AU - Vriens, Menno R

AU - Buikhuisen, Wieneke A

AU - Valk, Gerlof D

PY - 2021/2

Y1 - 2021/2

N2 - INTRODUCTION: Multiple Endocrine Neoplasia type 1 (MEN1)-related neuroendocrine tumors (NETs) of the lung are mostly indolent with a good prognosis. Nevertheless, cases of aggressive lung NET do occur, and therefore the management of individual patients is challenging.AIM: To assess tumor growth and survival of patients with MEN1-related lung NETs at long-term follow-up.METHODS: The population based Dutch MEN1 Study Group (DMSG) database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.RESULTS: In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 64.6% - 94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A>G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after six years of indolent disease, presumably explaining the sudden change in course.CONCLUSION: MEN1-related lung NETs are slow-growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.

AB - INTRODUCTION: Multiple Endocrine Neoplasia type 1 (MEN1)-related neuroendocrine tumors (NETs) of the lung are mostly indolent with a good prognosis. Nevertheless, cases of aggressive lung NET do occur, and therefore the management of individual patients is challenging.AIM: To assess tumor growth and survival of patients with MEN1-related lung NETs at long-term follow-up.METHODS: The population based Dutch MEN1 Study Group (DMSG) database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.RESULTS: In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 64.6% - 94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A>G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after six years of indolent disease, presumably explaining the sudden change in course.CONCLUSION: MEN1-related lung NETs are slow-growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.

KW - multiple endocrine neoplasia type 1

KW - lung NET

KW - tumor growth

KW - survival

KW - surveillance

U2 - 10.1210/clinem/dgaa800

DO - 10.1210/clinem/dgaa800

M3 - Article

C2 - 33135721

SN - 0021-972X

VL - 106

SP - E1014-E1027

JO - Journal of Clinical Endocrinology and Metabolism

JF - Journal of Clinical Endocrinology and Metabolism

IS - 2

ER -

The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Abstract

Keywords

Access to Document

Handle.net

Fingerprint

Cite this