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胆道胚胎性横纹肌肉瘤作为小儿患者出现胆管扩张时的鉴别诊断:并非总是胆总管囊肿。

Embryonal Rhabdomyosarcoma of the Biliary Tree as a Differential in a Paediatric Patient Presenting with Biliary Dilatation: Not Always a Choledochal Cyst.

作者信息

Tripathy Tara Prasad, Patidar Yashwant, Chandel Karamvir, Varadarajan Annapoorani, Sood Vikrant, Laroia Shalini Thapar

机构信息

Department of Radiodiagnosis, AIIMS, Bhubaneswar, India.

Department of Interventional Radiology, Institute of Liver & Biliary Sciences.

出版信息

Acta Med Litu. 2022;29(1):112-117. doi: 10.15388/Amed.2021.29.1.2. Epub 2022 Jan 24.

DOI:10.15388/Amed.2021.29.1.2
PMID:36061928
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9428640/
Abstract

Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumour and is the most prevalent soft-tissue sarcoma in the paediatric population. Although, Embryonal RMS of the biliary tree is a rare entity, however, it is the most common cause of paediatric malignant obstructive jaundice. We present a 4-year-old child who had symptoms of obstructive jaundice and palpable liver. The non-contrast magnetic resonance imaging and magnetic resonance cholangiopancreatography (MRCP) features were consistent with choledochal cyst. However, contrast enhanced computed tomography and PET CT images revealed biliary RMS as the differential diagnosis. Percutaneous biopsy followed by histopathology confirmed the diagnosis of embryonal biliary RMS. Since embryonal rhabdomyosarcoma is uncommonly recorded in the literature and can mimic the appearance of a choledochal cyst, this case report emphasises the necessity of keeping embryonal RMS as a differential in paediatric cases of obstructive jaundice.

摘要

横纹肌肉瘤是一种软组织恶性肌肉骨骼肿瘤,是儿科人群中最常见的软组织肉瘤。虽然胆管胚胎性横纹肌肉瘤是一种罕见的疾病,但它是小儿恶性梗阻性黄疸的最常见原因。我们报告一名4岁儿童,有梗阻性黄疸症状且可触及肝脏。非增强磁共振成像和磁共振胰胆管造影(MRCP)特征与胆总管囊肿一致。然而,增强计算机断层扫描和PET CT图像显示胆管横纹肌肉瘤为鉴别诊断。经皮活检及组织病理学检查确诊为胚胎性胆管横纹肌肉瘤。由于胚胎性横纹肌肉瘤在文献中记载较少,且可模仿胆总管囊肿的表现,本病例报告强调在小儿梗阻性黄疸病例中,将胚胎性横纹肌肉瘤作为鉴别诊断的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/17bff7676031/aml-29-1-112-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/c0a561376173/aml-29-1-112-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/8d9f94363765/aml-29-1-112-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/17bff7676031/aml-29-1-112-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/c0a561376173/aml-29-1-112-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/8d9f94363765/aml-29-1-112-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8301/9428640/17bff7676031/aml-29-1-112-g003.jpg

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引用本文的文献

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Rhabdomyosarcoma of the biliary tract in a child: a case report.儿童胆道横纹肌肉瘤:一例报告
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本文引用的文献

1
Embryonal rhabdomyosarcoma of the biliary tree in a paediatric patient - A rare cause of obstructive jaundice.一名儿科患者发生的胆管胚胎性横纹肌肉瘤——梗阻性黄疸的罕见病因。
SA J Radiol. 2019 Jan 10;23(1):1662. doi: 10.4102/sajr.v23i1.1662. eCollection 2019.
2
Pediatric Anaplastic Embryonal Rhabdomyosarcoma: Targeted Therapy Guided by Genetic Analysis and a Patient-Derived Xenograft Study.小儿间变性胚胎性横纹肌肉瘤:基于基因分析和患者来源异种移植研究的靶向治疗
Front Oncol. 2018 Jan 11;7:327. doi: 10.3389/fonc.2017.00327. eCollection 2017.
3
Embryonal rhabdomyosarcoma of the biliary tree: A rare cause of obstructive jaundice in children which can mimic choledochal cysts.
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。
Indian J Radiol Imaging. 2017 Jul-Sep;27(3):306-309. doi: 10.4103/ijri.IJRI_460_16.
4
The World Health Organization Classification of Skeletal Muscle Tumors in Pediatric Rhabdomyosarcoma: A Report From the Children's Oncology Group.世界卫生组织儿童横纹肌肉瘤骨骼肌肿瘤分类:来自儿童肿瘤学组的报告。
Arch Pathol Lab Med. 2015 Oct;139(10):1281-7. doi: 10.5858/arpa.2014-0475-OA. Epub 2015 May 19.
5
Rhabdomyosarcoma of biliary tract- a diagnostic dilemma.胆道横纹肌肉瘤——诊断难题
Indian J Surg Oncol. 2012 Dec;3(4):314-6. doi: 10.1007/s13193-012-0186-7. Epub 2012 Oct 6.
6
Biliary rhabdomyosarcoma.胆管横纹肌肉瘤
Pediatr Radiol. 2007 Oct;37(10):1059. doi: 10.1007/s00247-007-0531-0. Epub 2007 Jul 27.