ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome Clinicopathologic Study of 11 Cases and Review of the Literature

被引:1
|
作者
Maragliano, Roberta [1 ]
Vanoli, Alessandro [3 ]
Albarello, Luca [4 ]
Milione, Massimo [5 ]
Basturk, Olca [10 ]
Klimstra, David S. [10 ]
Wachtel, Antonio [11 ]
Uccella, Silvia [1 ]
Vicari, Emanuela [1 ]
Milesi, Marina [6 ]
Davi, Maria Vittoria [7 ]
Scarpa, Aldo [8 ,9 ]
Sessa, Fausto [1 ]
Capella, Carlo [1 ]
La Rosa, Stefano [2 ]
机构
[1] Univ Insubria, Dept Surg & Morphol Sci, Varese, Italy
[2] Osped Circolo Varese, Dept Pathol, I-21100 Varese, Italy
[3] Univ Pavia, Dept Mol Med, I-27100 Pavia, Italy
[4] Hosp San Raffaele, Dept Pathol, I-20132 Milan, Italy
[5] Natl Canc Inst, Dept Pathol, I-20133 Milan, Italy
[6] Multimedica, Dept Pathol, Milan, Italy
[7] GB Rossi Univ Hosp, Dept Med, Verona, Italy
[8] Univ Verona, ARC NET Res Ctr, I-37100 Verona, Italy
[9] Univ Verona, Dept Pathol & Diagnost, I-37100 Verona, Italy
[10] Mem Sloan Kettering Canc Ctr, Dept Pathol, New York, NY 10021 USA
[11] Inst Nacl Enfermedades Neoplasicas, Lima, Peru
关键词
Cushing syndrome; ACTH; neuroendocrine tumor; acinar cell carcinoma; pancreas; CARBOXYL ESTER LIPASE; CELL-DIFFERENTIATION; ENDOCRINE TUMORS; ADENOMAS; MARKER; IMMUNOPHENOTYPE; CARCINOMAS; EXPRESSION; SURVIVAL; INSIGHTS;
D O I
暂无
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
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 pre-operatory 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, beta-endorphin, trypsin, and BCL10 it is possible to recognize ACTH-secreting PanNETs and to distinguish them from the very aggressive ACTH-secreting ACCs.
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页码:374 / 382
页数:9
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