Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1

A single-centre study, systematic review and meta-analysis

Lei Ye, Weixi Wang, Naykky Singh Ospina, Lei Jiang, Ioannis Christakis, Jieli Lu, Yulin Zhou, Wei Zhu, Yanan Cao, Shu Wang, Nancy D. Perrier, William Francis Young, Guang Ning, Weiqing Wang

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Objective: Thymic neuroendocrine tumour (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumours and prognostic factors for clinical outcomes will be helpful in clinical practice. Design and Patients: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features and prognosis. Results: TH-NET was detected in 9 (7.4%) of 121 patients with MEN1 seen in our institution, and 5 (55.6%) were women. Seven additional studies were identified through a systematic review of the literature. The pool estimate of TH-NET prevalence was 3.7% (n = 99) in MEN1 (n = 2710), sex ratio was 79:20 (male vs female), and the median age at diagnosis was 43.0 years (range, 16.0-72.0 years). Forty-three patients died with a median survival time of 8.4 years. Older age at diagnosis (HR = 1.4, 95% CI = 1.0-1.8, P = .03), maximum tumour diameter (HR = 1.5, 95% CI = 1.0-2.3, P = .04) and presence of metastasis (HR = 1.6, 95% CI = 1.0-2.5, P = .04) were associated with worse outcome. A male predominance (91.9% vs 59.5%, P < .001) and history of smoking (59.0% vs 23.5%, P = .015) were more common in American/European series compared to Asian reports. Conclusion: TH-NET is a rare but fatal component of MEN1. Earlier detection of TH-NET in patients with MEN1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.

Original languageEnglish (US)
JournalClinical Endocrinology
DOIs
StateAccepted/In press - 2017

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Thymus Neoplasms
Multiple Endocrine Neoplasia Type 1
Neuroendocrine Tumors
Meta-Analysis
Sex Ratio
Neoplasms
Smoking
Neoplasm Metastasis
Survival
Mortality

Keywords

  • Meta-analysis
  • Multiple endocrine neoplasia type 1 (MEN1)
  • Thymic neuroendocrine tumour (TH-NET)

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

Cite this

Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1 : A single-centre study, systematic review and meta-analysis. / Ye, Lei; Wang, Weixi; Ospina, Naykky Singh; Jiang, Lei; Christakis, Ioannis; Lu, Jieli; Zhou, Yulin; Zhu, Wei; Cao, Yanan; Wang, Shu; Perrier, Nancy D.; Young, William Francis; Ning, Guang; Wang, Weiqing.

In: Clinical Endocrinology, 2017.

Research output: Contribution to journalArticle

Ye, Lei ; Wang, Weixi ; Ospina, Naykky Singh ; Jiang, Lei ; Christakis, Ioannis ; Lu, Jieli ; Zhou, Yulin ; Zhu, Wei ; Cao, Yanan ; Wang, Shu ; Perrier, Nancy D. ; Young, William Francis ; Ning, Guang ; Wang, Weiqing. / Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1 : A single-centre study, systematic review and meta-analysis. In: Clinical Endocrinology. 2017.
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abstract = "Objective: Thymic neuroendocrine tumour (TH-NET) accounts for almost 20{\%} of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumours and prognostic factors for clinical outcomes will be helpful in clinical practice. Design and Patients: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features and prognosis. Results: TH-NET was detected in 9 (7.4{\%}) of 121 patients with MEN1 seen in our institution, and 5 (55.6{\%}) were women. Seven additional studies were identified through a systematic review of the literature. The pool estimate of TH-NET prevalence was 3.7{\%} (n = 99) in MEN1 (n = 2710), sex ratio was 79:20 (male vs female), and the median age at diagnosis was 43.0 years (range, 16.0-72.0 years). Forty-three patients died with a median survival time of 8.4 years. Older age at diagnosis (HR = 1.4, 95{\%} CI = 1.0-1.8, P = .03), maximum tumour diameter (HR = 1.5, 95{\%} CI = 1.0-2.3, P = .04) and presence of metastasis (HR = 1.6, 95{\%} CI = 1.0-2.5, P = .04) were associated with worse outcome. A male predominance (91.9{\%} vs 59.5{\%}, P < .001) and history of smoking (59.0{\%} vs 23.5{\%}, P = .015) were more common in American/European series compared to Asian reports. Conclusion: TH-NET is a rare but fatal component of MEN1. Earlier detection of TH-NET in patients with MEN1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.",
keywords = "Meta-analysis, Multiple endocrine neoplasia type 1 (MEN1), Thymic neuroendocrine tumour (TH-NET)",
author = "Lei Ye and Weixi Wang and Ospina, {Naykky Singh} and Lei Jiang and Ioannis Christakis and Jieli Lu and Yulin Zhou and Wei Zhu and Yanan Cao and Shu Wang and Perrier, {Nancy D.} and Young, {William Francis} and Guang Ning and Weiqing Wang",
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T1 - Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1

T2 - A single-centre study, systematic review and meta-analysis

AU - Ye, Lei

AU - Wang, Weixi

AU - Ospina, Naykky Singh

AU - Jiang, Lei

AU - Christakis, Ioannis

AU - Lu, Jieli

AU - Zhou, Yulin

AU - Zhu, Wei

AU - Cao, Yanan

AU - Wang, Shu

AU - Perrier, Nancy D.

AU - Young, William Francis

AU - Ning, Guang

AU - Wang, Weiqing

PY - 2017

Y1 - 2017

N2 - Objective: Thymic neuroendocrine tumour (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumours and prognostic factors for clinical outcomes will be helpful in clinical practice. Design and Patients: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features and prognosis. Results: TH-NET was detected in 9 (7.4%) of 121 patients with MEN1 seen in our institution, and 5 (55.6%) were women. Seven additional studies were identified through a systematic review of the literature. The pool estimate of TH-NET prevalence was 3.7% (n = 99) in MEN1 (n = 2710), sex ratio was 79:20 (male vs female), and the median age at diagnosis was 43.0 years (range, 16.0-72.0 years). Forty-three patients died with a median survival time of 8.4 years. Older age at diagnosis (HR = 1.4, 95% CI = 1.0-1.8, P = .03), maximum tumour diameter (HR = 1.5, 95% CI = 1.0-2.3, P = .04) and presence of metastasis (HR = 1.6, 95% CI = 1.0-2.5, P = .04) were associated with worse outcome. A male predominance (91.9% vs 59.5%, P < .001) and history of smoking (59.0% vs 23.5%, P = .015) were more common in American/European series compared to Asian reports. Conclusion: TH-NET is a rare but fatal component of MEN1. Earlier detection of TH-NET in patients with MEN1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.

AB - Objective: Thymic neuroendocrine tumour (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumours and prognostic factors for clinical outcomes will be helpful in clinical practice. Design and Patients: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features and prognosis. Results: TH-NET was detected in 9 (7.4%) of 121 patients with MEN1 seen in our institution, and 5 (55.6%) were women. Seven additional studies were identified through a systematic review of the literature. The pool estimate of TH-NET prevalence was 3.7% (n = 99) in MEN1 (n = 2710), sex ratio was 79:20 (male vs female), and the median age at diagnosis was 43.0 years (range, 16.0-72.0 years). Forty-three patients died with a median survival time of 8.4 years. Older age at diagnosis (HR = 1.4, 95% CI = 1.0-1.8, P = .03), maximum tumour diameter (HR = 1.5, 95% CI = 1.0-2.3, P = .04) and presence of metastasis (HR = 1.6, 95% CI = 1.0-2.5, P = .04) were associated with worse outcome. A male predominance (91.9% vs 59.5%, P < .001) and history of smoking (59.0% vs 23.5%, P = .015) were more common in American/European series compared to Asian reports. Conclusion: TH-NET is a rare but fatal component of MEN1. Earlier detection of TH-NET in patients with MEN1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.

KW - Meta-analysis

KW - Multiple endocrine neoplasia type 1 (MEN1)

KW - Thymic neuroendocrine tumour (TH-NET)

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