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Endocrine Abstracts (2021) 73 OC9.1 | DOI: 10.1530/endoabs.73.OC9.1


1Vall d’Hebron Universitary Hospital, Endocrinology, Barcelona, Spain; 2Hospital of Cabueñes, Endocrinology, Gijón, Spain; 3Hospital General Universitario de Albacete, Endocrinology, Albacete, Spain; 4Hospital Universitario Cruces, Endocrinology, Barakaldo, Spain; 5Basurto University Hospital, Endocrinology, Bilbao, Spain; 6Virgen del Rocío University Hospital, Endocrinology, Sevilla, Spain; 7Hospital Universitario La Paz, Endocrinology, Madrid, Spain; 8University Hospital 12 de Octubre, Endocrinology, Madrid, Spain; 9Hospital Clínico Universitario Virgen de la Arrixaca, El Palmar, Spain; 10Hospital Clínic de Barcelona, Biochemistry and Molecular Genetics, Barcelona, Spain; 11Sociedad Española de Endocrinología y Nutrición (SEEN), Madrid, Spain


Background

Thymic neuroendocrine tumor (THY-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Diagnosis at early tumor stage is associated with improved survival.

Objective

To study the prevalence, clinical features and prognosis of THY-NET in MEN 1. To describe the overall causes of death among the registered MEN1 patients. To compare and discuss our results with the literature, identifying risk factors for the development and prognostic factors of survival of THY-NET.

Design and patients

Retrospective analysis of the 201-patient MEN1 cohort from the Spanish registry (REGMEN).

Results

THY-NET was detected in seven (3.5%) of 201 MEN1 patients, six (85.7%) were men and the mean age at diagnosis was 40.7 ± 10.5 years [range: 29–53]. Three were index cases and none had a prophylactic thymectomy performed. No particular genetic pathological variation was found to be associated with THY-NET. At presentation six were asymptomatic, and only one had chest pain; two developed Cushing’s syndrome later on (10 and 12 y). All the patients were operated on with a mean tumor size of 6.1 ± 2.9 cm, corresponding to five intermediate (atypical) and two high grade NET. Six patients received SSA and further approaches on the progression were reoperation (a second time in 2, three times in 2 patients), radiotherapy (1), everolimus (4), INFalpha (1), temozolomide (2), cisplatin-etoposide (1). After a mean follow up of 9 ± 3.2 years only one patient is free of disease (3 cm, stage I) at 64 months. Two are alive with metastatic disease after 55 and 146 months of follow-up, and four have died after 10.3 ± 2 years (mortality 57.1%), at a mean age of 46.5 ± 8.9 y. The causes of death were cardiac failure secondary to local metastatic disease, pneumonia in a patient with Cushing’s syndrome, Cushing’s syndrome, and postsurgical complication of a lung metastasis. Among the whole MEN1 cohort, 17 deaths have been reported (8.5%). THY-NET accounted for 23.5% of the mortality. Other causes of death were: duodenopancreatic tumors (4), adrenocortical cancer (1), pituitary macroadenoma (1), and others (mesothelioma, hepatocellular-carcinoma, adenocarcinoma (2), endocarditis, pancreatitis, covid-19). Age of death was 12 years younger in THY-NET patients (58 ± 14.1 vs 46.5 ± 9 y).

Conclusion

We found a similar prevalence, male predominance and prognosis of THY-NET in Spanish MEN 1 to other European/American series. Advanced stage and ectopic Cushing’s syndrome conferred worse prognosis. THY-NET is a rare but fatal component of MEN1. Screening and earlier detection of THY-NET is mandatory.

Volume 73

European Congress of Endocrinology 2021

Online
22 May 2021 - 26 May 2021

European Society of Endocrinology 

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