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Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1: A single‐centre study, systematic review and meta‐analysis
Author(s) -
Ye Lei,
Wang Weixi,
Ospiykky Singh,
Jiang Lei,
Christakis Ioannis,
Lu Jieli,
Zhou Yulin,
Zhu Wei,
Cao Yanan,
Wang Shu,
Perrier Nancy D.,
Young William F.,
Ning Guang,
Wang Weiqing
Publication year - 2017
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.13480
Subject(s) - medicine , meta analysis , neuroendocrine tumors , multiple endocrine neoplasia , gastroenterology , biology , biochemistry , gene
Summary Objective Thymic neuroendocrine tumour ( TH ‐ NET ) accounts for almost 20% of multiple endocrine neoplasia type 1 ( MEN 1)‐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 MEN 1 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 MEN 1 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 MEN 1 (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 MEN 1. Earlier detection of TH ‐ NET in patients with MEN 1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.