Metastatic rhabdomyosarcoma of the bile duct diagnosed by endoscopic retrograde cholangiopancreatography (ERCP)

Abstract

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric patients, although its location in the biliary tree in childhood is very rare. We present a five-year-old male patient who was diagnosed with hepatic rhabdomyosarcoma two years ago based on palpation of a large abdominal mass measuring about 20 cm. The patient received cytoreductive radiotherapy, which determined his dependence on the right part of the caudate lobe of the liver, with a 2 cm pedicle. He subsequently underwent surgery, with complete removal of the lesion, followed by radio and chemotherapy. Eight months later he presented symptoms of obstructive jaundice with cholangitis and diffuse abdominal pain. Endoscopic retrograde cholangiopancreatography (ERCP) was performed, which showed malignant-looking stenosis of the main bile duct below the confluence of the hepatic ducts and a dilated biliary tree above. A sphincterotomy was performed and a simple 7-fr, 5-cm-long pig-tail endoprosthesis was placed. A cytological sample was taken by brushing, and the histological study confirmed the diagnosis of rhabdomyosarcoma. Although improvement in the jaundiced condition was achieved and oncological treatment was indicated according to the established protocol, the patient died 3 months later. Tumor recurrence in the biliary tree was the main factor present in this patient that was related to low survival, despite the chemosensitivity of the tumor. ERCP, with its related techniques, proved to be a useful procedure in a patient who is difficult to manage due to his age and diagnostic and surgical complexity.