ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: clinicopathologic study of 11 cases and review of the literature.

Details

Serval ID
serval:BIB_6F6D34DD0AE8
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: clinicopathologic study of 11 cases and review of the literature.
Journal
American Journal of Surgical Pathology
Author(s)
Maragliano R., Vanoli A., Albarello L., Milione M., Basturk O., Klimstra D.S., Wachtel A., Uccella S., Vicari E., Milesi M., Davì M.V., Scarpa A., Sessa F., Capella C., La Rosa S.
ISSN
1532-0979 (Electronic)
ISSN-L
0147-5185
Publication state
Published
Issued date
2015
Peer-reviewed
Oui
Volume
39
Number
3
Pages
374-382
Language
english
Notes
Publication types: Journal Article ; Multicenter Study ; ReviewPublication Status: ppublish
Abstract
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.
Keywords
ACTH Syndrome, Ectopic/blood, ACTH Syndrome, Ectopic/diagnosis, ACTH Syndrome, Ectopic
Pubmed
Web of science
Create date
06/09/2016 11:56
Last modification date
20/08/2019 14:28
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