神经内分泌肿瘤嗜铬粒蛋白合成生长抑素类似物(兰瑞肽)后的反应:从一个分离的十二指肠肿瘤中的早期观察。

Neuro endocrinology letters Pub Date : 2023-07-05
E Scott Sills, Samuel H Wood, Seang Lin Tan, Daniel M Ibach
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引用次数: 0

摘要

十二指肠源性神经内分泌肿瘤(NETs)是所有NETs中一个不寻常的亚群,仅占该类肿瘤的3%。一般来说,NETs的特征是生长抑素受体的过度表达,并且具有良好的预后和早期诊断和干预。嗜铬粒蛋白A(CgA)是一种起源于神经元和内分泌细胞分泌囊泡的蛋白质,在NET的诊断和监测中得到了广泛的应用。兰瑞肽是一种具有强效抗增殖作用的合成八肽生长抑素类似物,已被美国食品药品监督管理局和欧洲药品管理局批准用于NET治疗。众所周知,它对生长激素、血清素、CgA和其他标志物具有抑制作用。在这里,我们描述了一名56岁的女性,患有十二指肠源性功能性NET,其血清CgA从3636成功降低到
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Neuroendocrine tumor chromogranin A response following synthetic somatostatin analog (lanreotide): Early observations from an isolated duodenal neoplasm .

Neuroendocrine tumors (NETs) of duodenal origin are an unusual subset among all NETs, comprising only about 3% of this neoplasm class. In general, NETs are characterized by overexpression of somatostatin receptors and carry an excellent prognosis with early diagnosis and intervention. Chromogranin A (CgA), a protein originating in secretory vesicles of neurons and endocrine cells, has gained wide usage in NET diagnosis and surveillance. Lanreotide is a synthetic octapeptide somatostatin analog with potent anti-proliferative action which has been approved by the FDA (U.S.) and EMA (E.U.) for NET treatment. It is known for its inhibitory effects on growth hormone, serotonin, CgA, and other markers. Here we describe a 56yr-old female with functional NET of duodenal origin, where serum CgA was successfully reduced from 3636 to <100 ng/mL after multidose lanreotide within five months. Of note, no metastatic spread was identified on positron emission tomography/computed tomography with 64Cu-labeled somatostatin analog tracer. Surgical resection of distal antrum, pylorus, and proximal duodenum was completed without complication. Histology revealed well-differentiated tumor cells with characteristic neuroendocrine features and clear surgical margins; low proliferation index (2%) was noted on Ki-67 staining. While select laboratory and imaging modalities are available for diagnosis and monitoring of duodenal NET, this is the first reported therapeutic use of lanreotide in this NET setting. The observed serum chromogranin A attenuation, even before surgery, supports its effectiveness in management of primary nonmetastatic duodenal NET after resection.

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