Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct

Abstract

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT. (Korean J Gastroenterol 2018;72:318-321)

Acute Cholangitis Masquerading Biliary Neuroendocrine Carcinoma: A Rare Twist to a Typical Presentation

“…Only a few cases of BNEC have been reviewed in the literature with most patients presenting with complaints of jaundice and right upper quadrant pain. [2][3][4][5] Similar to our patient, an 80-year-old man presented with jaundice, and the workup with ERCP showed complete obstruction of the CBD by a 1.5 cm mass. Interestingly, there were no malignant cells in the biliary brush cytology; however, the patient underwent extrahepatic bile duct resection because of suspicions of cholangiocarcinoma.…”

“…One other case presented a 59-year-old man with a complaint of jaundice, and CT imaging showed a 4.5 cm mass involving the CBD. 4 He underwent radical CBD and Roux-en-Y hepaticojejunostomy, and histology confirmed large-cell neuroendocrine carcinoma. This patient also received adjuvant chemotherapy and radiotherapy because of tumor invasion into proximal resection margins.…”

“…No recurrence was detected on CT imaging at a 10-month follow-up. 4 Surgical resection is the mainstay of treatment for BNEC, but the extent of surgery depends on the location and size of the tumor. For more advanced tumors, adjuvant chemotherapy and radiation may be necessary to improve chances of long-term survival.…”

Neuroendocrine carcinoma of the common hepatic duct coexisting with distal cholangiocarcinoma: A case report and review of literature

“…To date, adjuvant therapy for EBNEC lacks standardization, and the roles of chemotherapy, radiotherapy and immunotherapy are still unclear. Commonly used chemotherapy regimens include cisplatin and etoposide[ 17 , 22 , 26 , 38 , 44 ] and cisplatin and irinotecan[ 37 , 39 , 50 ]. As neoadjuvant chemotherapy regimens, these two regimens can successfully achieve conversion.…”

Bile Duct Mixed Neuroendocrine-nonendocrine Neoplasm with a Complete Response to Chemoradiotherapy

“…As mentioned earlier, the frequency of neuroendocrine carcinomas arising from the bile duct is very low, and only a few reports have discussed the use of chemoradiotherapy for treatment ( 5 , 6 ). To our knowledge, no cases of curative chemoradiotherapy for neuroendocrine extrahepatic bile duct carcinomas have yet been reported.…”