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胃嗜铬样细胞在 1 型多发性内分泌肿瘤中的变化。

Gastric enterochromaffin-like cell changes in multiple endocrine neoplasia type 1.

机构信息

Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany.

Institute of Pathology and Cytology, Wetzlar, Germany.

出版信息

Clin Endocrinol (Oxf). 2021 Sep;95(3):439-446. doi: 10.1111/cen.14430. Epub 2021 Feb 7.

Abstract

BACKGROUND

Gastric enterochromaffin-like cell (ECL) tumours can occur in patients with multiple endocrine neoplasia type 1 (MEN1), especially in those affected by Zollinger Ellison syndrome (ZES). Since the prevalence of ECL lesions is not well defined yet, the present study evaluated the presence and extent of ECL lesions in MEN1 patients with and without ZES.

METHODS

Multiple endocrine neoplasia type 1 patients being part of a regular screening program (2014-2018) underwent gastroduodenoscopies with biopsies of the stomach and determination of serum gastrin and chromogranin A levels. Haematoxylin- and immunostaining with chromogranin A, gastrin and VMAT I and II (vesicular monoamine transporter I and II) of the biopsies were performed.

RESULTS

Thirty-eight MEN1 patients, of whom 16 (42%) were diagnosed and treated earlier for ZES, were analysed. In ten of 16 (62.5%) ZES patients, a locally scattered, mixed image of diffuse, linear and micronodular mild hyperplasia was present. In addition, two of these patients (13%) showed small (max 1.5 mm in size) intramucosal ECL tumours. Neither ECL changes, nor tumours were found in MEN1 patients without ZES (n = 22). In MEN1/ZES patients, the median serum gastrin level was significantly elevated compared to MEN1 patients without ZES (206 pg/ml vs. 30.5 pg/ml, p < .001). A subgroup analysis of the serum gastrin and chromogranin A levels of MEN1/ZES patients with or without ECL hyperplasia did not show significant differences (gastrin level: p = .302, chromogranin A: p = .464).

CONCLUSION

Enterochromaffin-like cell hyperplasia and gastric carcinoids occur only in MEN1 patients with ZES, but less frequently than reported.

摘要

背景

胃嗜铬样细胞(ECL)肿瘤可发生在多发性内分泌肿瘤 1 型(MEN1)患者中,尤其是在患有卓-艾综合征(ZES)的患者中。由于 ECL 病变的患病率尚未明确,因此本研究评估了伴有和不伴有 ZES 的 MEN1 患者中 ECL 病变的存在和程度。

方法

作为常规筛查计划的一部分(2014-2018 年),MEN1 患者接受了胃十二指肠镜检查,并对胃进行了活检,同时测定了血清胃泌素和嗜铬粒蛋白 A 水平。对活检组织进行了苏木精和免疫染色,免疫染色使用的抗体包括嗜铬粒蛋白 A、胃泌素和 VMAT I 和 II(囊泡单胺转运体 I 和 II)。

结果

分析了 38 名 MEN1 患者,其中 16 名(42%)患者被诊断并更早地治疗 ZES。在 16 名 ZES 患者中的 10 名(62.5%)中,存在局部散在的、弥漫性、线性和微结节性轻度增生的混合图像。此外,其中 2 名患者(13%)存在小的(最大直径为 1.5 毫米)黏膜内 ECL 肿瘤。在无 ZES 的 MEN1 患者(n=22)中,未发现 ECL 变化或肿瘤。在 MEN1/ZES 患者中,血清胃泌素水平明显高于无 ZES 的 MEN1 患者(206pg/ml 比 30.5pg/ml,p<.001)。对伴有或不伴有 ECL 增生的 MEN1/ZES 患者的血清胃泌素和嗜铬粒蛋白 A 水平进行亚组分析,未发现显著差异(胃泌素水平:p=0.302,嗜铬粒蛋白 A:p=0.464)。

结论

嗜铬样细胞增生和胃类癌仅发生在伴有 ZES 的 MEN1 患者中,但比报道的要少见。

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