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Clinical Endocrinology

Thymic neuroendocrine tumour in multiple endocrine neoplasia type 1: female patients are not rare exceptions

Authors


Correspondence: Akihiro Sakurai, Department of Medical Genetics, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan. Tel.: +81 263 372618; Fax: +81 263 372619; E-mail: aksakura@shinshu-u.ac.jp

Summary

Objective

Thymic neuroendocrine tumour (Th-NET) occurs in 2–5% of patients with MEN1 and has high malignant potency accompanying recurrence and distant metastasis. While Th-NET is recognized to develop predominantly in men and heavy smokers, a number of female patients have been reported in the literature. The objective of this study is to clarify the clinical features of MEN1 patients with Th-NET using database analysis.

Design/Patients

Clinical data of patients with Th-NET were extracted and analysed from a recently constructed database of Japanese MEN1 patients.

Results

Among 560 registered cases, Th-NET was seen in 28 (5·0%) patients. Of note, 36% of patients (10/28) were women; only one patient among those was a smoker and another six patients were non-smokers. Age at diagnosis of Th-NET and MEN1, tumour size, prevalence of other MEN1-related tumours did not differ between male and female patients, and 10-year survival probability was 0·271 ± 0·106.

Conclusions

Although the prevalence of Th-NET in women (3·2%) is significantly lower than that in men (7·6%), a considerable proportion of female patients develop Th-NET. Given that Th-NET is a major determinant of life expectancy of patients, our results alert clinicians who treat patients with MEN1 that surveillance of Th-NET is essential even for female patients without a smoking habit.

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