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Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst
Bhushan Nemade1, Kaustav Talapatra, Tanuja Shet
1Department of Radiation Oncology, Tata Memorial Hospital, Mumbai, India.
Insights
Embryonal rhabdomyosarcoma (ERMS) of the biliary tree is a rare cancer. This case study details a child treated with surgery, chemotherapy, and radiotherapy for this rare pediatric biliary tumor.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Imaging
Background:
- Embryonal rhabdomyosarcoma (ERMS) is a rare mesenchymal neoplasm.
- ERMS of the biliary tree is exceptionally rare, often diagnosed during surgery or via liver biopsy.
- Accurate diagnosis is crucial for effective treatment planning.
Observation:
- A case of a one-year-eight-month-old child presenting with symptoms mimicking a choledochal cyst is described.
- The patient was diagnosed with embryonal rhabdomyosarcoma of the biliary tree.
- The initial presentation posed a diagnostic challenge due to its resemblance to other pediatric liver conditions.
Findings:
- The child underwent surgical intervention for the biliary tumor.
- Treatment included chemotherapy using the IRS IV protocol.
- Adjuvant radiotherapy was administered to the surgical bed using 6 MV photons, totaling 5040 cGy in 28 fractions.
Implications:
- This case highlights the importance of considering rare diagnoses like ERMS in pediatric patients with biliary abnormalities.
- Multimodal treatment involving surgery, chemotherapy, and radiotherapy can be effective for biliary embryonal rhabdomyosarcoma.
- Further research into the optimal management of pediatric biliary sarcomas is warranted.
Abstract:
Embryonal rhabdomyosarcoma (ERMS) of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040 cGy in 28 fractions.
