Titre
ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: clinicopathologic study of 11 cases and review of the literature.
Type
étude de cas
Institution
Externe
Périodique
Auteur(s)
Maragliano, R.
Auteure/Auteur
Vanoli, A.
Auteure/Auteur
Albarello, L.
Auteure/Auteur
Milione, M.
Auteure/Auteur
Basturk, O.
Auteure/Auteur
Klimstra, D.S.
Auteure/Auteur
Wachtel, A.
Auteure/Auteur
Uccella, S.
Auteure/Auteur
Vicari, E.
Auteure/Auteur
Milesi, M.
Auteure/Auteur
Davì, M.V.
Auteure/Auteur
Scarpa, A.
Auteure/Auteur
Sessa, F.
Auteure/Auteur
Capella, C.
Auteure/Auteur
La Rosa, S.
Auteure/Auteur
Liens vers les personnes
ISSN
1532-0979
Statut éditorial
Publié
Date de publication
2015
Volume
39
Numéro
3
Première page
374
Dernière page/numéro d’article
382
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Multicenter Study ; ReviewPublication Status: ppublish
Résumé
Adrenocorticotropic hormone (ACTH)-secreting pancreatic neuroendocrine tumors (PanNETs), although rare, are responsible for about 15% of ectopic Cushing syndrome (CS). They represent a challenging entity because their preoperatory diagnosis is frequently difficult, and clear-cut morphologic criteria useful to differentiate them from other types of PanNETs have not been defined. Ectopic ACTH secretion associated with CS can also be rarely due to pancreatic acinar cell carcinoma (ACC) and pancreatoblastoma, rare tumor types with morphologic features sometimes overlapping those of PanNETs and, for this reason, representing a diagnostic challenge for pathologists. We herein describe the clinicopathologic and immunohistochemical features of 10 PanNETs and 1 ACC secreting ACTH and associated with CS together with an extensive review of the literature to give the reader a comprehensive overview on ACTH-producing pancreatic neoplasms. ACTH-secreting PanNETs are aggressive neoplasms with an immunohistochemical profile that partially overlaps that of pituitary corticotroph adenomas. They are generally large and well-differentiated neoplasms without distinctive histologic features but with signs of aggressiveness including vascular and perineural invasion. They are more frequent in female individuals with a mean age of 42 years. At 5 and 10 years after diagnosis, 35% and 16.2% of patients, respectively, were alive. ACTH-secreting ACCs and pancreatoblastomas are very aggressive pediatric tumors with a poor prognosis. Using an appropriate immunohistochemical panel including ACTH, β-endorphin, trypsin, and BCL10 it is possible to recognize ACTH-secreting PanNETs and to distinguish them from the very aggressive ACTH-secreting ACCs.
PID Serval
serval:BIB_6F6D34DD0AE8
PMID
Date de création
2016-09-06T10:56:20.795Z
Date de création dans IRIS
2025-05-20T21:59:18Z