Talal Alkayali, MBBS1, Ahmed I. Salem, MD1, Juan Ricardo, MD1, Bahaa Amer, MD1, Justin George, MD1, Kinan Rahal, MD2
1Florida State University / Sarasota Memorial Hospital, Sarasota, FL; 2Intercoastal Medical Group, Sarasota, FL
Introduction: Plexiform neurofibroma (PN) is a benign tumor that originates from nerves. This tumor is usually associated with neurofibromatosis type1 (NF1) and found in young patients. Common places of growth include head, neck and maxillofacial area. We report a rare case of common bile duct plexiform neurofibroma.
Case Description/Methods: A 69 year old female with history of cholecystectomy had incidental finding of dilated pancreatic duct (PD) on chest CT during pneumonia work up. Patient denied abdominal pain, weight loss, change in bowel habits, jaundice or itching. Physical examination was normal. Laboratory tests including CBC, LFT, CA 19-9 were unremarkable. MRCP showed extrahepatic biliary ductal dilation measuring 8mm, minor intrahepatic ductal dilation involving left lobe, diffuse PD dilation from the ampulla to the body, involving the major and minor ducts with acinarization, and abrupt change in the main PD at the junction between the body and tail of pancreas, no masses were seen. EUS showed a CBD of 8mm and a hypoechoic mass measuring 8.8x5.6mm in the common hepatic duct (CHD) with mild dilation of the left hepatic duct measuring 6mm. PD was dilated between the pancreatic head, and body measuring 8mm without mural nodules, and 1.5mm in the distal body and tail of pancreas. No obvious abnormality was visualized at the transition point. Previous findings were concerning for segmental main duct IPMN. ERCP performed, ampulla appeared normal without “fish mouth” appearance, and no filling defect in PD. Cholangiogram and biliary ductosccopy using Spyglass were performed and showed low-grade stricture in the CHD. Biliary epithelium was endoscopically normal in the strictured area. Biopsies obtained using Spybite. EUS and FNA of the hypoechoic area in the CHD was performed after the ERCP. Biopsies obtained with Spybite showed benign appearing ductal epithelium with fibrotic tissue. However, FNA cytology showed rare cluster of atypical glandular cells. Patient was referred to surgery. Pathology of the common bile duct showed a 7mm plexiform neurofibroma with clear border resection without evidence of malignancy.
Discussion: PN is a rare benign tumor that develops from schwann cells in a bundle of nerves involving cutaneous nerves or internal nerve fibers. PN has substantial risk to transform into malignant peripheral nerve sheath tumors, therefore surgical resection is advised. PN is usually associated with NF1. However, few cases without clear etiology were reported with no link to the syndrome.
Citation: Talal Alkayali, MBBS; Ahmed I. Salem, MD; Juan Ricardo, MD; Bahaa Amer, MD; Justin George, MD; Kinan Rahal, MD. P1907 - A RARE TUMOR IN THE COMMON BILE DUCT: A CASE REPORT OF PLEXIFORM NEUROFIBROMA IN THE COMMON BILE DUCT. Program No. P1907. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.