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


METHODS
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.
Department of Medical Genetics, Shinshu University School of Medicine, Matsumoto, Japan. aksakura@shinshu-u.ac.jp
Akihiro
A
Sakurai
Tsuneo
T
Imai
Toyone
T
Kikumori
Kiyomi
K
Horiuchi
Takahiro
T
Okamoto
Shinya
S
Uchino
Shinji
S
Kosugi
Shinichi
S
Suzuki
Keiko
K
Suyama
Masanori
M
Yamazaki
Ai
A
Sato
D000328:Adult; D000368:Aged; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D009154:Mutation; D018358:Neuroendocrine Tumors / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D012737:Sex Factors; D013953:Thymus Neoplasms / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*