PMID- 28940393 OWN - NLM STAT- MEDLINE DCOM- 20180702 LR - 20181202 IS - 1365-2265 (Electronic) IS - 0300-0664 (Linking) VI - 87 IP - 6 DP - 2017 Dec TI - Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1: A single-centre study, systematic review and meta-analysis. PG - 706-716 LID - 10.1111/cen.13480 [doi] 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. CI - (c) 2017 John Wiley & Sons Ltd. FAU - Ye, Lei AU - Ye L AUID- ORCID: 0000-0003-4064-4460 AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Wang, Weixi AU - Wang W AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Ospina, Naykky Singh AU - Ospina NS AD - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA. FAU - Jiang, Lei AU - Jiang L AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Christakis, Ioannis AU - Christakis I AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Lu, Jieli AU - Lu J AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Zhou, Yulin AU - Zhou Y AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Zhu, Wei AU - Zhu W AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Cao, Yanan AU - Cao Y AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Wang, Shu AU - Wang S AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. FAU - Perrier, Nancy D AU - Perrier ND AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Young, William F Jr AU - Young WF Jr AD - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA. FAU - Ning, Guang AU - Ning G AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. AD - Laboratory for Endocrine & Metabolic Diseases of Institute of Health Science, Shanghai JiaoTong University School of Medicine and Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai, China. FAU - Wang, Weiqing AU - Wang W AD - Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. LA - eng PT - Journal Article PT - Meta-Analysis PT - Review PT - Systematic Review DEP - 20171016 PL - England TA - Clin Endocrinol (Oxf) JT - Clinical endocrinology JID - 0346653 SB - IM MH - Female MH - Humans MH - Male MH - Multiple Endocrine Neoplasia Type 1/*pathology MH - Neuroendocrine Tumors/*pathology MH - Prognosis MH - Thymus Neoplasms/*pathology OTO - NOTNLM OT - meta-analysis OT - multiple endocrine neoplasia type 1 (MEN1) OT - thymic neuroendocrine tumour (TH-NET) EDAT- 2017/09/25 06:00 MHDA- 2018/07/03 06:00 CRDT- 2017/09/24 06:00 PHST- 2017/04/26 00:00 [received] PHST- 2017/09/11 00:00 [revised] PHST- 2017/09/07 00:00 [accepted] PHST- 2017/09/25 06:00 [pubmed] PHST- 2018/07/03 06:00 [medline] PHST- 2017/09/24 06:00 [entrez] AID - 10.1111/cen.13480 [doi] PST - ppublish SO - Clin Endocrinol (Oxf). 2017 Dec;87(6):706-716. doi: 10.1111/cen.13480. Epub 2017 Oct 16.