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胰腺多囊性腺瘤样错构瘤:一例发生于一名3岁男孩的此前未被描述过的胰腺肿瘤。

Multicystic adenomatoid hamartoma of the pancreas: a hitherto undescribed pancreatic tumor occurring in a 3-year-old boy.

作者信息

Thrall Michael, Jessurun Jose, Stelow Edward B, Adsay N Volkan, Vickers Selwyn M, Whitson Amy K, Saltzman Daniel A, Pambuccian Stefan E

机构信息

Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, MN, USA.

出版信息

Pediatr Dev Pathol. 2008 Jul-Aug;11(4):314-20. doi: 10.2350/07-04-0260.1. Epub 2007 Jul 17.

DOI:10.2350/07-04-0260.1
PMID:17990924
Abstract

This report describes an unusual pancreatic tumor in a 3-year-old boy. He presented with abdominal pain secondary to pancreatitis and was found to have a complex, multicystic lesion within the head of the pancreas. He subsequently underwent a pancreatoduodenectomy. Grossly, the mass was 3 cm, multicystic, and well demarcated. Histologically, there were numerous variably sized, dilated ducts lined by tall columnar mucinous cells surrounded by a stroma that exhibited foci of cellular condensations resembling primitive pancreatic mesenchyme. Acinar and endocrine cells were often seen budding into the ducts forming "ductulo-insular bodies." As a result of its superficial resemblance to Stocker type 2 cystic adenomatoid malformation of the lung, we are proposing the name "multicystic adenomatoid hamartoma" for this lesion.

摘要

本报告描述了一名3岁男孩身上的一种罕见胰腺肿瘤。他因胰腺炎继发腹痛就诊,检查发现胰腺头部有一个复杂的多囊肿块。随后他接受了胰十二指肠切除术。大体上,肿块为3厘米,多囊肿性,边界清晰。组织学检查显示,有许多大小不一、扩张的导管,内衬高柱状黏液细胞,周围间质有类似原始胰腺间充质的细胞凝聚灶。常可见腺泡和内分泌细胞向导管内出芽,形成“导管岛状结构”。由于其外观与肺的斯托克2型囊性腺瘤样畸形相似,我们建议将此病变命名为“多囊肿性腺瘤样错构瘤”。

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