Botryoid rhabdomyosarcoma arising in choledochal malformation: case report, robotic-assisted excision, lessons learnt and literature review.

Vecchione, S.; Johnson R.; Matcovici M.; Stahlschmidt J.; Dawrant M.J.; Alizai N.K.

Botryoid rhabdomyosarcoma arising in choledochal malformation: case report, robotic-assisted excision, lessons learnt and literature review.

All Authors

Vecchione, S.

Johnson R.

Matcovici M.

Stahlschmidt J.

Dawrant M.J.

Alizai N.K.

LTHT Author

Vecchione, Sarah
Johnson, Rhys
Matcovici, Melania
Dawrant, Michael
Alizai, Naved
Stahlschmidt, Jens

LTHT Department

Leeds Children's Hospital
Children's Liver Unit
Paediatric Histopathology
Doctors' Rotation
Pathology
Histopathology

Publication Date

2024

Item Type

Article

Abstract

Purpose: Malignant change is a well-recognised risk of choledochal malformation (CM). Rhabdomyosarcoma (RMS) has been reported as a primary malignancy in children, which can cause biliary obstruction, mimicking CM. There are no reports describing RMS arising from a CM at a young age treated with minimally invasive approach (MIS). We describe the first case of this kind. The patient underwent robotic-assisted excision and hepaticojejunostomy. Method(s): Patient records were reviewed. Literature search of MEDLINE, Web of science and CENTRAL was performed. Result(s): A 3-year-old female patient presented with obstructive jaundice and fever. Ultrasound and MRCP appearances suggested type 4a CM (Todani Classification), with ectatic intrahepatic ducts. There were unusual CM features, with the presence of sludge in the upper parts of the extra-hepatic biliary system, unresponsiveness to ursodeoxycholic acid and a non-distended gallbladder. Patient was scheduled for robotic excision and hepaticojejunostomy. Intraoperative frozen sections confirmed malignancy. Excision was performed robotically. Histology confirmed embryonal botryoid RMS near the origin of the cystic duct. Patient received further adjuvant chemotherapy and proton beam therapy as per oncology protocol. The patient remains recurrence-free 8 years later. Conclusion(s): To authors knowledge, this represents the first case report of a robotic excision of biliary tract RMS arising in a CM. It demonstrates the importance of a high index of suspicion in the presence of unusual features. Minimal-invasive approach can safely be considered in selected oncological cases.