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

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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.

Methods

Patient records were reviewed. Literature search of MEDLINE, Web of science and CENTRAL was performed.

Results

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.

Conclusions

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.

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Data availability

The data that support the findings of this study are available on request from the corresponding author, [NKA]. The data are not publicly available due to privacy restrictions.

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Author information

Authors and Affiliations

  1. Paediatric Liver Unit, Department of Paediatric Surgery, Leeds Children’s Hospital, Leeds, UK

    Sarah Vecchione, Rhys Johnson, Melania Matcovici, Michael J. Dawrant & Naved K. Alizai

  2. Paediatric Histopathology Unit, St James’s University Hospital, Leeds, UK

    Jens Stahlschmidt

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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by SV and RJ. The first draft of the manuscript was written by SV with contribution from RJ and all authors commented on further versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Naved K. Alizai.

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The authors have no competing interests to declare that are relevant to the content of this article. Mr. Alizai is Proctor for Intuitive Surgical Inc.

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Informed consent was obtained from legal guardians and the participant has consented to the submission of the case report to the journal.

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Vecchione, S., Johnson, R., Matcovici, M. et al. Botryoid rhabdomyosarcoma arising in choledochal malformation: case report, robotic-assisted excision, lessons learnt and literature review. J Ped Endosc Surg 6, 77–84 (2024). https://doi.org/10.1007/s42804-024-00224-0

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