Rhabdomyosarcoma arising within the biliary tract mimicking a choledochal cyst: A case report

Abstract

Rhabdomyosarcoma (RMS) involving the biliary tree and choledochal cysts presents with jaundice and abdominal pain. Imaging studies including ultrasound (US), computerized tomography (CT) scan, and magnetic resonance cholangiopancreatography (MRCP) are useful in the diagnosis and evaluation of biliary tree anatomy. We present a patient with biliary RMS initially thought to be a choledochal cyst with intraluminal cystic debris (biliary sludge) that was diagnosed by frozen section examination during cyst resection and subsequently treated with post-operative chemotherapy and radiation.

Pencil Beam Scanning Proton Therapy for Rhabdomyosarcoma of the Biliary Tract

“…This case illustrates the benefit of early input from all subspecialties in the treatment of this patient population. Historically, most of these cases were treated with surgical resection for primary site control [ 3 , 8 - 10 ]. Proton radiotherapy proved advantageous as compared to photon radiotherapy due to substantially improved liver health and contra-lateral kidney sparring (see Figure 6a and Figure 6b ).…”

“…Hepatobiliary RMS is often diagnosed under the context of hepatitis and frequently misdiagnosed as a benign process or a benign mass such as a choledochal cyst [ 3 ]. Its location provides for clinically challenging approaches given relative inaccessibility to achieve gross resection and the functional complications presented by biliary obstruction.…”