Neuroendocrine Tumor of the Common Bile Duct: Case Report

Abstract

Carcinoma of the extrahepatic biliary tract accounts for <2% of all cancers. Neuroendocrine tumor of the extrahepatic bile duct is very rare, and there are <200 cases reported since 1959. The preoperative diagnosis is infrequent (5.12%). The definite diagnosis relies on postoperative pathology which utilized immunohistochemistry study on many biomarkers to diagnose the histological subtypes of neuroendocrine neoplasms, such as chromogranin A, synaptophysin, and neuron-specific enolase. When the primary tumor has no metastases, radical removal of the lesion appears as curative treatment. The treatment of the carcinoid syndrome or other functioning syndrome is the first priority. We report a case of a 12-year-old Mexican woman with neuroendocrine tumor of the extrahepatic bile duct (common bile duct neuroendocrine tumor) seen in our hospital. Resection of the common bile duct, cholecystectomy, end to side Roux-en-y hepaticojejunostomy, and portal lymphadenectomy was performed. A review of the pertinent literature was performed. Given the rarity of the disease, treatment principles are based mainly on retrospective series and case reports. We present the eighth case in adolescence in the literature.

Keywords: Extrahepatic bile duct; Immunohistochemical analysis; Kulchitsky cells; Neuroendocrine tumors; Surgical resection.

Fig. 1

The bile duct ultrasound shows dilation in the common bile duct (a), endoscopic retrograde cholangiopancreatography reported a polyp that conditioned an increase in the volume of 50% of the intra- and extrahepatic bile duct (b), prosthesis was placed (c), and magnetic cholangioresonance was performed, in which dilation of the bile duct was reported (d).

Fig. 2

The lesion corresponds to a polyp with a broad base measuring 1.9 × 1.8 × 1.6 cm, which was exophytic and was still firmly attached to the wall, and the cut was solid (a). The microscopic study showed that the tumor had multiple growth patterns. It had areas made up of solid sheets of cells, areas with growth of papillary architecture, and areas with trabecular growth (b). The cells were medium in size with round nuclei with an eosinophilic cytoplasm, and the chromatin was distinguished by a granular appearance (c).

Fig. 3

Immunohistochemical stains were performed in which the neuroendocrine markers were intensely positive for chromogranin (a), synaptophysin (b), and CD56 (c).