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胆管胚胎性横纹肌肉瘤:儿童梗阻性黄疸的罕见病因,可酷似胆总管囊肿。

Embryonal rhabdomyosarcoma of the biliary tree: A rare cause of obstructive jaundice in children which can mimic choledochal cysts.

作者信息

Kinariwala Dhara J, Wang Andrew Y, Melmer Patrick D, McCullough William P

机构信息

Division of Pediatric Imaging, Department of Radiology and Medical Imaging, Charlottesville, Virginia, USA.

Division of Gastroenterology and Hepatology, Department of Medicine, University of Virginia Health System, Charlottesville, Virginia, USA.

出版信息

Indian J Radiol Imaging. 2017 Jul-Sep;27(3):306-309. doi: 10.4103/ijri.IJRI_460_16.

DOI:10.4103/ijri.IJRI_460_16
PMID:29089679
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5644324/
Abstract

Jaundice in children is more often due to hepatic disease than obstruction. Differential considerations for obstructive jaundice in children include choledocholithiasis, choledochal cysts and rare neoplasms. Rhabdomyosarcoma, the most common soft tissue sarcoma in pediatric patients, typically involves the head and neck, genitourinary system and extremities. Embryonal rhabdomyosarcoma of the biliary tree is a rare entity. We present a 3-year-old boy with abrupt onset obstructive jaundice. Although initial imaging suggested a dilated biliary system with fusiform common bile duct, sludge, and possible cholelithiasis, endoscopic retrograde cholangiopancreatogram (ERCP) diagnosed a common bile duct embryonal rhabdomyosarcoma and further imaging showed involvement of the cystic duct. This case illustrates the importance of considering malignant etiologies in cases of obstructive jaundice, particularly when imaging is not classic for common causes.

摘要

儿童黄疸更常见的病因是肝脏疾病而非梗阻。儿童梗阻性黄疸的鉴别诊断包括胆总管结石、胆总管囊肿和罕见肿瘤。横纹肌肉瘤是儿科患者中最常见的软组织肉瘤,通常累及头颈部、泌尿生殖系统和四肢。胆管胚胎性横纹肌肉瘤是一种罕见的疾病。我们报告一名3岁突发梗阻性黄疸的男孩。尽管初始影像学检查提示胆管系统扩张,胆总管呈梭形,有胆泥及可能的胆结石,但内镜逆行胰胆管造影(ERCP)诊断为胆总管胚胎性横纹肌肉瘤,进一步影像学检查显示胆囊管也受累。该病例说明了在梗阻性黄疸病例中考虑恶性病因的重要性,尤其是当影像学表现不符合常见病因的典型表现时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/cd9b9c287f58/IJRI-27-306-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/cb123cc8389b/IJRI-27-306-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/fedb555be8b2/IJRI-27-306-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/47743b9a2475/IJRI-27-306-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/2084d054563f/IJRI-27-306-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/39e5eb459284/IJRI-27-306-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/cd9b9c287f58/IJRI-27-306-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/cb123cc8389b/IJRI-27-306-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/fedb555be8b2/IJRI-27-306-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/47743b9a2475/IJRI-27-306-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/2084d054563f/IJRI-27-306-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/39e5eb459284/IJRI-27-306-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d686/5644324/cd9b9c287f58/IJRI-27-306-g006.jpg

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