Resection of perihilar biliary schwannoma

Schwannomas are usually benign nerve sheath tumors, which typically arise in the head, neck, spinal cord and extremities. Schwannoma of the biliary tract is an extremely rare finding. Patients generally lack symptoms and seek medical attention when tumor growth causes obstructive jaundice. Preoperative diagnosis is difficult and resection is the treatment of choice.

A 54 year-old female with history of back and right labia minor melanoma for which she underwent complete excision and right inguinal lymph node dissection more than 10 years ago, was evaluated for new onset gastroesophageal reflux symptoms and found to have markedly abnormal liver enzymes. Imagining studies revealed intrahepatic ductal dilatation and a 5.2 cm mass in the porta hepatis that was not consistent with cholangiocarcinoma or hepatocellular carcinoma. Multiple percutaneous biopsies of the mass failed to provide a definitive diagnosis. With a high clinical suspicion of metastatic melanoma and no other evident sites of disease, operative intervention was undertaken for diagnosis and definitive treatment.

Diagnostic laparoscopy was performed initially, but access to the mass was difficult, given its location. Subsequently, the patient underwent laparotomy, with tumor excision, common bile duct resection and hepato-jejunostomy. Pathologic examination and analysis were consistent with cellular schwannoma. Postoperatively, the patient recovered uneventfully, and liver function studies returned to normal.

Schwannomas are uncommon tumors, which very rarely arise from the biliary tract and cause biliary obstruction. Exploration is indicated in order to establish the diagnosis and to render definitive treatment.