Robotic-Assisted Surgery Approach in a Biliary Rhabdomyosarcoma Misdiagnosed as Choledochal Cyst

Abstract

Rhabdomyosarcoma is a soft tissue malignant tumor affecting 1% of children from 0 to 14 years. Preoperative imaging may not always be diagnostic for hepatobiliary rhabdomyosarcoma and differential diagnosis with choledochal cyst (CC) could be difficult. We report a case of 2-years-old girl with a strange CC pattern of presentation. A grapelike lesion involving the choledochal and biliary ducts was easily and completely resected by robotic assisted surgery. Since no previous reports were available about oncologic safety of robotic approach, the porto-enterostomy was performed in open surgery. On histologic examination, the specimen revealed a botryoidembryonal rhabdomyosarcoma affecting both the common bile duct and the common hepatic duct. One year postoperatively the child is safe of tumor relapse. Robotic approach seems to be safe and advantageous to obtain a radical excision of the tumor at the porta hepatis, even in case of misdiagnosed malignant lesion mimicking a CC.

Laparoscopic management of Rhabdomyosarcoma of common Bile duct, Case report

“…Around one hundred cases of CBD RMS reported in literature [ [2] , [3] , [4] , 6 , [13] , [14] , [15] ], the largest series was 25 cases over 25 years [ 15 ]. Most of the reported cases were difficult to diagnose and were misdiagnosed as a Choledochal Cyst [ [2] , [3] , [4] , [5] , 15 ], especially in confined tumor with no invasion to the surrounding structures [ 4 ].…”

“…Rhabdomyosarcoma (RMS) is the most common soft tissue tumor in children. However, they are rare as they constitute about 3% of all malignancies affecting pediatrics [ 1 ], with median age of 3.5 years and slight male predominance [ [2] , [3] , [4] , [5] ]. RMS is divided into five major subtypes; Embryonal subtype is considered the most common one [ 6 ].…”

“…ERMS looks like the radiological and clinical features of Choledochal Cyst (CC); diagnosis is usually made at surgery [ [2] , [3] , [4] , [5] ]. The management of RMS depends on risk stratification and involves one or a combination of surgical resection, chemotherapy and radiotherapy [ 9 ].…”

“…Minimally invasive surgery (MIS) in pediatric age group has been expanded to the extent that complex procedures like laparoscopic choledochal cyst surgery can be conducted with safety and outcomes similar to open approach and with the advantage of minimal scarring [ 10 , 11 ]. However, surgical approaches reported for biliary RMS were laparotomies except one case, which resection was done by robot and converted to open approach for Roux-en-Y Hepaticojejunostomy (RYHJ) reconstruction [ 2 ].…”

Pediatric Choledochal Cysts: Unknowns are Decreasing

“…It is still important to differentiate biliary rhabdomyosarcoma, a rare soft tissue tumor that affects only 1% of children, from CC [106,107]. In the presence of a mass or intraductal growth that causes obstructive jaundice, the possible diagnosis should return in favor of rhabdomyosarcoma in children and prompt evaluation is necessary [107].…”