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胰腺卡波西样血管内皮瘤合并卡萨巴赫-梅里特现象:一种罕见病例。

Pancreatic kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: A rare entity.

作者信息

Mathew Denny, Mahomed Nasreen

机构信息

Department of Diagnostic Radiology, University of the Witwatersrand, Johannesburg, South Africa.

Department of Radiology, Rahima Moosa Mother and Child Hospital, University of the Witwatersrand, Johannesburg, South Africa.

出版信息

SA J Radiol. 2019 Aug 19;23(1):1760. doi: 10.4102/sajr.v23i1.1760. eCollection 2019.

Abstract

Primary pancreatic tumours are a rare and unusual entity in children. In this article, we present the case of an 8-month-old girl who presented with obstructive jaundice. The differential diagnosis based on imaging studies was that of a pancreatic vascular neoplasm; however, with the laboratory evidence of Kasabach-Merritt phenomenon (KMP), this prompted the diagnosis of pancreatic kaposiform hemangioendothelioma. A core biopsy of the pancreatic mass was taken at laparotomy and confirmed this diagnosis. The pancreas is an exceedingly rare site of occurrence for this tumour, with only nine cases being published to date. The clinical, biochemical, imaging and pathological findings are discussed to highlight a rare and potentially life-threatening vascular tumour.

摘要

原发性胰腺肿瘤在儿童中是一种罕见且不常见的疾病。在本文中,我们报告了一名8个月大患阻塞性黄疸女童的病例。基于影像学研究的鉴别诊断为胰腺血管肿瘤;然而,由于存在卡萨巴赫-梅里特现象(KMP)的实验室证据,促使诊断为胰腺卡波西样血管内皮瘤。在剖腹手术时对胰腺肿块进行了核心活检,证实了这一诊断。胰腺是该肿瘤极为罕见的发生部位,迄今为止仅有9例病例报道。本文讨论了其临床、生化、影像学和病理学表现,以突出这种罕见且可能危及生命的血管肿瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2dd8/6837828/947aa111c789/SAJR-23-1760-g001.jpg

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