In:
Clinical Endocrinology, Wiley, Vol. 87, No. 6 ( 2017-12), p. 706-716
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
0300-0664
,
1365-2265
DOI:
10.1111/cen.2017.87.issue-6
Language:
English
Publisher:
Wiley
Publication Date:
2017
detail.hit.zdb_id:
2004597-9
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