Tripathy Tara Prasad, Patidar Yashwant, Chandel Karamvir, Varadarajan Annapoorani, Sood Vikrant, Laroia Shalini Thapar
Department of Radiodiagnosis, AIIMS, Bhubaneswar, India.
Department of Interventional Radiology, Institute of Liver & Biliary Sciences.
Acta Med Litu. 2022;29(1):112-117. doi: 10.15388/Amed.2021.29.1.2. Epub 2022 Jan 24.
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.
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。