Nazmul Hassan, Rafiqul Islam, Kaniz Fathema, Maimuna Sayeed, Md Wahiduzzaman Mazumder, Asm Bazlul Karim, Bishnu Pada Dey
Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.
Department of Pathology, Faculty of Basic Science and Paraclinical Science, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.
Zhongguo Dang Dai Er Ke Za Zhi. 2020 Dec;22(12):1338-1343. doi: 10.7499/j.issn.1008-8830.2007118.
Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.
肝外胆道肿瘤较为罕见,其中横纹肌肉瘤最为常见。横纹肌肉瘤是一种软组织恶性肌肉骨骼肿瘤,是儿童胆总管非常罕见的恶性肿瘤。它通常表现为梗阻性黄疸和/或瘙痒。如果没有局部侵犯相邻组织,肿瘤病变的影像学表现类似于胆总管囊肿。因此,诊断通常在手术时或术前活检时做出。早期诊断并将其与胆总管囊肿区分开来,并尽早开始治疗对于患者的长期生存很重要。本病例报告介绍了一名10岁男孩,术前反复出现梗阻性黄疸和发热,术前诊断为胆总管囊肿,术后诊断为胆总管胚胎性横纹肌肉瘤。经过手术切除和术后化疗,患儿预后良好。因此,重要的是要知道这种罕见肿瘤可模仿先天性胆总管囊肿,在儿童梗阻性黄疸的鉴别诊断中应予以考虑。