2006
DOI: 10.1111/j.1365-2354.2006.00683.x
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Botryoid rhabdomyosarcoma of the biliary tract in children: a unique case report

Abstract: Rhabdomyosarcoma (RMS) is the most common tumour of the biliary tree in childhood. In children, it is a rare lesion, accounting for about 1% of all RMS. Hepatobiliary botryoid RMS is a disease affecting young children at a median age of about 3 years. In literature, the radiological findings of hepatobiliary RMS have been described in small series and some case reports. In this case report, we present a rare case of RMS of the extrahepatic biliary tree initially diagnosed as a choledochal cyst.

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Cited by 31 publications
(20 citation statements)
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“…Median age at presentation is 3 years, but it should be included in the differential diagnosis of an infant presenting with jaundice and a mass in the porta hepatis. Other diagnostic possibilities include undifferentiated sarcoma of the liver, pancreateoblastoma, papillary cystic tumor of the pancreas, metastatic lesions and more rarely, hepatoblastoma [50,51] . Additionally, a RMS in this location can mimic the radiological appearance of a choledochal cyst because of its combined cystic and solid component [52] .…”
Section: Biliary Tract Rhabdomyosarcomamentioning
confidence: 99%
“…Median age at presentation is 3 years, but it should be included in the differential diagnosis of an infant presenting with jaundice and a mass in the porta hepatis. Other diagnostic possibilities include undifferentiated sarcoma of the liver, pancreateoblastoma, papillary cystic tumor of the pancreas, metastatic lesions and more rarely, hepatoblastoma [50,51] . Additionally, a RMS in this location can mimic the radiological appearance of a choledochal cyst because of its combined cystic and solid component [52] .…”
Section: Biliary Tract Rhabdomyosarcomamentioning
confidence: 99%
“…It is a disease of young children (average age 3 years) with a slight male preponderance. 3,4 This tumor that mainly arises from the CBD may occur in any part of the biliary tree, resulting in symptoms of obstructive jaundice which is observed in 60%-80% of the cases. Clinical presentation may include other symptoms such as abdominal pain, emesis, fever, acholic stools, and decreased appetite.…”
Section: Discussionmentioning
confidence: 99%
“…1 Because of the scarcity of this disease, the majority of the pediatric literature consists of isolated case reports. 2,3 Here, we present a case of a male child with embryonal RMS originating in the common bile duct.…”
Section: Introductionmentioning
confidence: 99%
“…Although it is the most common biliary tumor in children, BRMS comprises only about 1% of all rhabdomyosarcomas [1][2][3] . BRMS usually arises in the CBD, but may originate from anywhere along the biliary tree.…”
Section: Discussionmentioning
confidence: 99%