Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct

Mixed adenoneuroendocrine carcinomas, spindle cell carcinomas, and clear cell carcinomas are all rare tumors in the biliary tract. We present the first case, to our knowledge, of an extrahepatic bile duct carcinoma composed of all three types. A 65-year-old man with prior cholecystectomy presented w...

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Main Authors: John Wysocki, Rishi Agarwal, Laura Bratton, Jeremy Nguyen, Mandy Crause Weidenhaft, Nathan Shores, Hillary Z. Kimbrell
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2014/347949
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author John Wysocki
Rishi Agarwal
Laura Bratton
Jeremy Nguyen
Mandy Crause Weidenhaft
Nathan Shores
Hillary Z. Kimbrell
author_facet John Wysocki
Rishi Agarwal
Laura Bratton
Jeremy Nguyen
Mandy Crause Weidenhaft
Nathan Shores
Hillary Z. Kimbrell
author_sort John Wysocki
collection DOAJ
description Mixed adenoneuroendocrine carcinomas, spindle cell carcinomas, and clear cell carcinomas are all rare tumors in the biliary tract. We present the first case, to our knowledge, of an extrahepatic bile duct carcinoma composed of all three types. A 65-year-old man with prior cholecystectomy presented with painless jaundice, vomiting, and weight loss. CA19-9 and alpha-fetoprotein (AFP) were elevated. Cholangioscopy revealed a friable mass extending from the middle of the common bile duct to the common hepatic duct. A bile duct excision was performed. Gross examination revealed a 3.6 cm intraluminal polypoid tumor. Microscopically, the tumor had foci of conventional adenocarcinoma (CK7-positive and CA19-9-postive) surrounded by malignant-appearing spindle cells that were positive for cytokeratins and vimentin. Additionally, there were separate areas of large cell neuroendocrine carcinoma (LCNEC). Foci of clear cell carcinoma merged into both the LCNEC and the adenocarcinoma. Tumor invaded through the bile duct wall with extensive perineural and vascular invasion. Circumferential margins were positive. The patient’s poor performance status precluded adjuvant therapy and he died with recurrent and metastatic disease 5 months after surgery. This is consistent with the reported poor survival rates of biliary mixed adenoneuroendocrine carcinomas.
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spelling doaj-art-1bb3afc68e144ea9a9176d33c8e6047c2025-02-03T05:53:56ZengWileyCase Reports in Pathology2090-67812090-679X2014-01-01201410.1155/2014/347949347949Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile DuctJohn Wysocki0Rishi Agarwal1Laura Bratton2Jeremy Nguyen3Mandy Crause Weidenhaft4Nathan Shores5Hillary Z. Kimbrell6Department of Gastroenterology and Hepatology, Tulane University, 1430 Tulane Avenue, New Orleans, LA 70112, USADepartment of Gastroenterology and Hepatology, Tulane University, 1430 Tulane Avenue, New Orleans, LA 70112, USADepartment of Pathology and Laboratory Medicine, Tulane University, 1430 Tulane Avenue, SL-79, New Orleans, LA 70112, USADepartment of Radiology, Tulane University, 1430 Tulane Avenue, New Orleans, LA 70112, USADepartment of Radiology, Tulane University, 1430 Tulane Avenue, New Orleans, LA 70112, USADepartment of Gastroenterology and Hepatology, Tulane University, 1430 Tulane Avenue, New Orleans, LA 70112, USADepartment of Pathology and Laboratory Medicine, Tulane University, 1430 Tulane Avenue, SL-79, New Orleans, LA 70112, USAMixed adenoneuroendocrine carcinomas, spindle cell carcinomas, and clear cell carcinomas are all rare tumors in the biliary tract. We present the first case, to our knowledge, of an extrahepatic bile duct carcinoma composed of all three types. A 65-year-old man with prior cholecystectomy presented with painless jaundice, vomiting, and weight loss. CA19-9 and alpha-fetoprotein (AFP) were elevated. Cholangioscopy revealed a friable mass extending from the middle of the common bile duct to the common hepatic duct. A bile duct excision was performed. Gross examination revealed a 3.6 cm intraluminal polypoid tumor. Microscopically, the tumor had foci of conventional adenocarcinoma (CK7-positive and CA19-9-postive) surrounded by malignant-appearing spindle cells that were positive for cytokeratins and vimentin. Additionally, there were separate areas of large cell neuroendocrine carcinoma (LCNEC). Foci of clear cell carcinoma merged into both the LCNEC and the adenocarcinoma. Tumor invaded through the bile duct wall with extensive perineural and vascular invasion. Circumferential margins were positive. The patient’s poor performance status precluded adjuvant therapy and he died with recurrent and metastatic disease 5 months after surgery. This is consistent with the reported poor survival rates of biliary mixed adenoneuroendocrine carcinomas.http://dx.doi.org/10.1155/2014/347949
spellingShingle John Wysocki
Rishi Agarwal
Laura Bratton
Jeremy Nguyen
Mandy Crause Weidenhaft
Nathan Shores
Hillary Z. Kimbrell
Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
Case Reports in Pathology
title Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
title_full Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
title_fullStr Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
title_full_unstemmed Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
title_short Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct
title_sort mixed large cell neuroendocrine carcinoma and adenocarcinoma with spindle cell and clear cell features in the extrahepatic bile duct
url http://dx.doi.org/10.1155/2014/347949
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