Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these patients?
Adrenal Gland Neoplasms
/ mortality
Cause of Death
Cohort Studies
Early Detection of Cancer
/ methods
Female
Follow-Up Studies
Gastrinoma
/ mortality
Humans
Lymphoma, T-Cell, Cutaneous
/ mortality
Male
Middle Aged
Multiple Endocrine Neoplasia Type 1
/ diagnosis
Neoplasm Staging
Neuroendocrine Tumors
/ mortality
Prognosis
Spain
/ epidemiology
Thoracic Neoplasms
/ mortality
MEN 1
Mortality
Pancreatic neuroendocrine tumors
Prognosis
Uncommon tumors
Journal
Journal of endocrinological investigation
ISSN: 1720-8386
Titre abrégé: J Endocrinol Invest
Pays: Italy
ID NLM: 7806594
Informations de publication
Date de publication:
Jun 2021
Jun 2021
Historique:
received:
27
05
2020
accepted:
31
08
2020
pubmed:
11
9
2020
medline:
26
11
2021
entrez:
10
9
2020
Statut:
ppublish
Résumé
The prognosis of MEN 1 patients is not only determined by pancreatic disease; it is also related to other uncommon tumors. The objective of this study is to analyze the tumors associated with MEN 1 outside the classic triad and to investigate their relationship with mortality. One hundred and five MEN 1 patients were studied in a tertiary referral hospital (1980-2019). With a follow-up of 11 ± 4 years, seven patients died (8%), four as a consequence MEN syndrome. Thirty-three percent had adrenal gland tumors. One patient died of adrenal cancer. Eight percent presented with a neuroendocrine thoracic neoplasm, and one patient died. Another patient died due to cutaneous T-cell lymphoma. A further patient died because of a gastrinoma with liver metastasis. To conclude, 75% of MEN-related deaths were the result of an uncommon pathology, and we, therefore, recommend that these tumors should be taken into account in the screening and follow-up of these patients.
Identifiants
pubmed: 32909176
doi: 10.1007/s40618-020-01414-2
pii: 10.1007/s40618-020-01414-2
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
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