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 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. 

译文

促肾上腺皮质激素 (ACTH) 分泌的胰腺神经内分泌肿瘤 (PanNETs) 虽然罕见，但却导致了约 15% 的异位库欣综合征 (CS)。它们代表了一个具有挑战性的实体，因为它们的术前诊断经常是困难的，并且明确的形态学标准有助于将它们与其他类型的 PanNETs 区分开来还没有被定义。与 CS 相关的异位 ACTH 分泌也很少是由于胰腺腺泡细胞癌 (ACC) 和胰腺母细胞瘤，这些罕见的肿瘤类型具有形态特征，有时与 PanNETs 重叠，因此, 代表病理学家的诊断挑战。我们在此描述了 10 个 PanNETs 和 1 个 ACC 分泌 ACTH 并与 CS 相关的临床病理和免疫组织化学特征，并对文献进行了广泛的综述，让读者对产生 ACTH 的胰腺有一个全面的概述。肿瘤。分泌 ACTH 的 PanNETs 是侵袭性肿瘤，其免疫组织化学特征部分与垂体皮质 troph 腺瘤重叠。它们通常是大而分化良好的肿瘤，没有独特的组织学特征，但有攻击性的迹象，包括血管和神经周围的侵犯。它们在平均年龄为 42 岁的女性中更常见。在诊断后的 5 年和 10 年，分别有 35% 和 16.2% 的患者存活。分泌 ACTH 的 ACCs 和胰腺母细胞瘤是非常具有侵袭性的儿童肿瘤，预后不良。使用适当的免疫组织化学小组，包括 ACTH 、 β-内啡肽、胰蛋白酶和 BCL10，可以识别分泌 ACTH 的 PanNETs 并将其与非常具有攻击性的分泌 ACTH 的 ACCs 区分开来。