Background: Schwannomas are benign nerve sheath tumors that are extremely rare in the biliary tract. A comprehensive review of literature enumerated approximately 30 case reports of schwannoma in the biliary tract tree and porta hepatis region.

Case Presentation: We report a case of a 40-year-old female who presented with abdominal pain. Imaging revealed a mass at the porta hepatis extending from the portal bifurcation till the hilum encasing the main portal vein and abutting the right portal vein. Differentials of carcinoma, lymphoma, and mesenchymal tumor were kept. Ultrasound-guided biopsy of the mass showed a benign nerve sheath tumor, immunopositive for S100. The histopathological evaluation of the excised mass confirmed the origin of mass in the common bile duct.

Conclusions: Our case highlights that schwannomas, though benign, can mimic a carcinoma or lymphoma if present at a rare site such as bile ducts. An exhaustive clinical and radiological workup with diligent histopathological evaluation is mandatory in dealing with such rare cases as radical surgery and chemotherapy can be avoided in such patients.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11216546PMC
http://dx.doi.org/10.1159/000534300DOI Listing

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