Isolated neurofibromas of the gallbladder (GB) and common bile duct (CBD) are exceptionally rare benign tumors, often mimicking malignancies and posing diagnostic challenges. This paper reports the unique case of a 32-year-old male presenting with right upper quadrant pain, jaundice, and clay-colored stools. Imaging revealed a septate GB with mural thickening, a gallstone, and distal CBD annular thickening, indicating a dual malignancy (GB carcinoma and cholangiocarcinoma). A hepato-pancreatic-duodenectomy was performed because of a suspected malignancy. The histopathology examination unexpectedly revealed spindle cell lesions positive for S100 and neurofilament protein, confirming a primary neurofibroma of the GB and CBD. This first reported case of simultaneous dual-site involvement highlights the diagnostic difficulty because of malignancy mimicry, advocating for intraoperative frozen sections to guide surgical management and avoid overtreatment.
Harsh et al. (Fri,) studied this question.