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。

Cushing Syndrome

内分泌 糖皮质激素分泌过多 疾病
概述  :  

库欣综合征是由于长期接触糖皮质激素(如皮质醇)引起的体征和症状的集合。症状和体征可能包括高血压,腹部肥胖,但手臂和腿部较细,红色妊娠纹,红色圆脸,肩部脂肪肿块,肌肉无力,骨骼脆弱,痤疮和皮肤愈合不良。女性可能会出现毛发旺盛和月经不调。偶尔可能会出现情绪变化,头痛和疲倦感诊断方法①24h尿游离皮质醇测试;②深夜唾液皮质醇测试;③低剂量地塞米松抑制试验(LDDST);④地塞米松-CRH试验。治疗方法垂体瘤最常见的治疗方法是手术切除肿瘤。使用特殊的显微镜和精细仪器,外科医生通过鼻孔或上唇下方的开口

Cushing   英 /'kuʃiŋ/

       n. 库欣(姓氏)

       Objective To reinvestigate the value of overnight low-dose dexamethasone suppression test in the diagnosis of Cushing syndrome. 目的重新探讨过夜小剂量地塞米松抑制试验对库欣综合征的诊断价值。

 

Syndrome   英 /'sɪndrəʊm/   美 /'sɪndrəm/

       n. [临床] 综合症状;并发症状;校验子;并发位

               n. (Syndrome)人名;(英)辛德罗姆

       The researchers found that some of the people with this syndrome lack a gene for BDNF and have correspondingly low blood levels of the substance. 研究人员发现患有这种综合症的一些人缺少表达BDNF的基因,并且血液中这种物质的水平相对较低。

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