Clinical Features and Prognosis of Thymic Neuroendocrine Tumors Associated with Multiple Endocrine Neoplasia Type 1: A Single Center Study, Systematic Review, and Meta-analysis

Summary

Objective

Thymic neuroendocrine tumor (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumors 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 were detected in nine (7.4%) of 121 MEN1 patients seen in our institution and five (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=0.03), maximum tumor diameter (HR=1.5, 95%CI=1.0-2.3, P=0.04), and presence of metastasis (HR=1.6, 95%CI=1.0-2.5, P=0.04) were associated with worse outcome. A male predominance (91.9% vs 59.5%, P<0.001) and history of smoking (59.0% vs 23.5%, P=0.015) 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.

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