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Objective MEN1 is associated with an increased risk of developing tumors in different endocrine organs. Neuroendocrine tumors of the thymus (TNETs) are very rare but often have an aggressive nature. We evaluated patients with MEN1 and TNET in three university hospitals in Finland. Design/Methods We evaluated patient records of 183 MEN1-patients from three university hospitals between the years 1985–2019 with TNETs. Thymus tumor specimens were classified according to the new WHO 2021 classification of TNET. We collected data on treatments and outcomes of these patients. Results There were six patients (3.3%) with MEN1 and TNET. Five of them had the same common gene mutation occurring in Finland. They originated from common ancestors encompassing two pairs of brothers from sequential generations. The mean age at presentation of TNET was 44.7 ± 11.9 years. TNET was classified as atypical carcinoid (AC) in five out of six patients. One patient had a largely necrotic main tumor with very few mitoses and another nodule with 25 mitoses per 2 mm2, qualifying for the 2021 WHO diagnosis of large cell neuroendocrine carcinoma (LCNEC). In our patients, the 5-year survival of the TNET patients was 62.5% and 10-year survival 31.3%. Conclusion In this study, TNETs were observed in one large MEN1 founder pedigree, where an anticipation-like earlier disease onset was observed in the most recent generation. TNET in MEN1 patients is an aggressive disease. The prognosis can be better by systematic screening. We also show that LCNEC can be associated with TNET in MEN1 patients.

Background Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-year survival rates of 20 Hounsfield Units (HU) for all tumours (median 34 (21–45)), median size 92 mm (20–196), Ki67 17% (1–40%), Weiss score 7 (4–9) and Helsinki score 24 (4–48). ACC was more often found in the left than the right adrenal (p 5 to > 10 years was achieved after repeated surgery of metastases. Overall 5-year survival was 67%, and 96% vs. 26% for ENSAT stage I–II vs. III–IV (p 20 on nonenhanced CT but variable tumour size (20–196 mm). Malignancy cannot be ruled out by small tumour size only. The 5-year survival of 96% in ENSAT stage I–III compares favourably to previous studies.

Aim To perform a baseline survey on condition-specific information access among patients/parents/caregivers with rare endocrine disorders (RD) in Europe. Methods Electronic invitation to participate in a survey (19 questions) was sent to 120 patient advocacy groups (PAGs), and further distributed to 32 European countries. Results A total of 1138 respondents from 22 countries (74% women), aged between 1 year (parents) and 70 years, participated. The Netherlands, France, Germany, Italy and France had highest participation rates. All Main Thematic Groups (MTGs) were represented; the adrenal (32%), pituitary (26%) and thyroid (22%) were the most common. The majority of the respondents got information from their endocrinologist (75%), PAGs (37%) and expert reference centre (22%); 95% received information in their mother tongue. Leaflets (70%), infographics (65%), webinars (60%) and Internet films (55%) were preferred ways of learning. Respondents relied mostly on materials by PAGs and alliances (79%), rather than from specific international RD sites (15%). Fifty-six percent used Facebook, and 37% other social media, with a significant age difference (40 years) among non-users, 19% vs. 36%, p 40 years) in the willingness to help create new educational materials; 49% vs. 34%, p Conclusions Our current patient information access survey provides a sound basis for further planning and execution of educational and teaching activities by Endo-ERN.