Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1:A single-centre
Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1:A single-centre
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DOI：
10.1111/cen.13480
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Wang Weiqing
中科院分区：
文献类型：
--
作者：
Ye Lei;Wang Weixi;Ospina Naykky Singh;Jiang Lei;Christakis Ioannis;Lu Jieli;Zhou Yulin;Zhu Wei;Cao Yanan;Wang Shu;Perrier Nancy D;William F. Young Jr;Ning Guang;Wang Weiqing
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.
