Korean J Gastroenterol.  2023 Mar;81(3):121-124. 10.4166/kjg.2023.005.

Small-cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct: A Rare Case Report

• Han B
• Seo YJ
• Oh GH
• You GR
• Ma KY
• Kim KH
• Noh MG
• Joo YE

• KMID: 2540954
• DOI: http://doi.org/10.4166/kjg.2023.005

Abstract

Neuroendocrine carcinoma (NEC) arising from the extrahepatic bile duct is extremely rare and commonly mistaken for cholangiocarcinoma. Therefore, NEC of the bile duct is difficult to diagnose preoperatively. Previously reported cases were resected with a diagnosis of cholangiocarcinoma and diagnosed with NEC after surgery. This paper reports an 84-year-old female with small-cell NEC of the extrahepatic bile duct, confirmed by a biopsy from an ERCP, with a review of the relevant literature. Contrast-enhanced abdomen computed tomography and magnetic resonance cholangiopancreatography revealed an approximately 1.7 cm enhancing intraductal mass in the proximal common bile duct with dilatation of the upstream bile duct. ERCP showed a long strictured segment in the proximal common bile duct with bile duct dilatation. A biopsy was performed at the site of the stricture. Histological examinations and hematoxylin–eosin staining showed the solid proliferation of small tumor cells with irregularly shaped hyperchromatic nuclei. Immunohistochemical examinations showed that the tumor cells were positive for CD56 and synaptophysin. Small-cell NEC of the extrahepatic bile duct was confirmed based on the histology and immunohistochemistry findings. The patient and their family denied treatment because of the patient’s old age.

Keyword

Neuroendocrine carcinoma; Bile duct

Figure

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  Fig. 1 (A, B) Contrast-enhanced abdomen and pelvis CT revealed an approximately 1.7 cm enhancing intraductal mass in the proximal common bile duct with dilatation of the upstream bile duct (square), and a 3.1 cm irregular enhancing mass along common hepatic chain suggesting metastatic lymph node (circle). (C) MRCP revealed an intraductal mass in the proximal common bile duct.

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  Fig. 2 (A) ERCP revealed a long strictured segment in the proximal portion of the common bile duct with bile duct dilatation. (B) Biliary drainage was performed with a plastic stent (7 Fr, 7 cm).

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  Fig. 3 (A) Histopathological examination shows solid and cord-structured proliferation of small tumor cells with of small tumor cells with irregularly shaped hyperchromatic nuclei (H&E, ×100). Immunohistochemical examinations show that the cells are positive for CD56 (B), synaptophysin (C), and a Ki-67 index of 85% (D) (IHC, ×100).

Reference

1. Luchini C, Pelosi G, Scarpa A, et al. 2021; Neuroendocrine neoplasms of the biliary tree, liver and pancreas: a pathological approach. Pathologica. 113:28–38. DOI: 10.32074/1591-951X-231. PMID: 33686308. PMCID: PMC8138696.

2. Hong N, Kim HJ, Byun JH, et al. 2015; Neuroendocrine neoplasms of the extrahepatic bile duct: radiologic and clinical characteristics. Abdom Imaging. 40:181–191. DOI: 10.1007/s00261-014-0191-0. PMID: 25008023.

3. Michalopoulos N, Papavramidis TS, Karayannopoulou G, Pliakos I, Papavramidis ST, Kanellos I. 2014; Neuroendocrine tumors of extrahepatic biliary tract. Pathol Oncol Res. 20:765–775. DOI: 10.1007/s12253-014-9808-4. PMID: 24917351.

4. Ishida M, Okano K, Sandoh K, et al. 2020; Neuroendocrine carcinoma diagnosis from bile duct cytological specimens: A retrospective single-center study. Diagn Cytopathol. 48:154–158. DOI: 10.1002/dc.24334. PMID: 31697402.

5. Zheng Z, Chen C, Li B, et al. 2019; Biliary neuroendocrine neoplasms: clinical profiles, management, and analysis of prognostic factors. Front Oncol. 9:38. DOI: 10.3389/fonc.2019.00038. PMID: 30805307. PMCID: PMC6370735.

6. Nagtegaal ID, Odze RD, Klimstra D, et al. WHO Classification of Tumours Editorial Board. The 2019 WHO classification of tumours of the digestive system. Histopathology. 2020; 76:182–188. DOI: 10.1111/his.13975. PMID: 31433515. PMCID: PMC7003895.

7. Rindi G, Mete O, Uccella S, et al. 2022; Overview of the 2022 WHO classification of neuroendocrine neoplasms. Endocr Pathol. 33:115–154. DOI: 10.1007/s12022-022-09708-2. PMID: 35294740.

8. Fernandes CJ, Leung G, Eads JR, Katona BW. 2022; Gastroenteropancreatic neuroendocrine tumors. Gastroenterol Clin North Am. 51:625–647. DOI: 10.1016/j.gtc.2022.06.002. PMID: 36153114.

9. Shim JR, Kim JR, Park Y, Seo HI. 2020; Small-cell neuroendocrine carcinoma arising from an extra-hepatic bile duct: a case report. Gastroenterol Rep (Oxf). 9:380–382. DOI: 10.1093/gastro/goaa051. PMID: 34567573. PMCID: PMC8460092.

10. Kamiya M, Yamamoto N, Kamioka Y, et al. 2020; Rapidly progressed neuroendocrine carcinoma in the extrahepatic bile duct: a case report and review of the literature. Surg Case Rep. 6:191. DOI: 10.1186/s40792-020-00945-3. PMID: 32748005. PMCID: PMC7399003.

11. Umezaki N, Hashimoto D, Yamashita YI, et al. 2019; Neuroendocrine tumor of the hilar bile duct. Anticancer Res. 39:903–907. DOI: 10.21873/anticanres.13192. PMID: 30711974.

12. Hazama K, Suzuki Y, Takahashi M, et al. 2003; Primary small cell carcinoma of the common bile duct, in which surgical treatment was performed after neoadjuvant chemotherapy: report of a case. Surg Today. 33:870–872. DOI: 10.1007/s00595-003-2594-3. PMID: 14605962.

13. Okamura Y, Maeda A, Matsunaga K, et al. 2009; Small-cell carcinoma in the common bile duct treated with multidisciplinary management. J Hepatobiliary Pancreat Surg. 16:575–578. DOI: 10.1007/s00534-009-0051-4. PMID: 19288048.

14. Aigner B, Kornprat P, Schöllnast H, Kasparek AK, Mischinger HJ, Haybaeck J. 2015; A case of focal small-cell neuroendocrine carcinoma in the vicinity of the extrahepatic bile duct, adjacent to an extensive biliary intraepithelial neoplasm: a diagnostic challenge with major clinical implications. Anticancer Res. 35:4821–4828.

15. Jeon WJ, Chae HB, Park SM, Youn SJ, Choi JW, Kim SH. 2006; [A case of primary small cell carcinoma arising from the common bile duct]. Korean J Gastroenterol. 48:438–442. Korean.

16. Kwon YD, Kim CD, Kim YS, et al. 2009; A case of synchronous primary cancer: small cell carcinoma in the common bile duct and adenocarcinoma in the stomach. Korean J Gastrointest Endosc. 38:303–308.

17. Kim BC, Song TJ, Lee H, et al. 2013; [A case of small cell neuroendocrine tumor occurring at hilar bile duct]. Korean J Gastroenterol. 62:301–305. Korean. DOI: 10.4166/kjg.2013.62.5.301. PMID: 24262597.

18. Sugita H, Maeda K, Nishikawa S, Doden K, Hashizume Y. 2022; Case of resected small-cell neuroendocrine carcinoma of the extrahepatic bile duct. J Surg Case Rep. 2022:rjac020. DOI: 10.1093/jscr/rjac020. PMID: 35154639. PMCID: PMC8828790.

19. Garcia-Carbonero R, Sorbye H, Baudin E, et al. Vienna Consensus Conference participants. ENETS consensus guidelines for high-grade gastroenteropancreatic neuroendocrine tumors and neuroendocrine carcinomas. Neuroendocrinology. 2016; 103:186–194. DOI: 10.1159/000443172. PMID: 26731334.

20. Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC. 2014; Gastroenteropancreatic high-grade neuroendocrine carcinoma. Cancer. 120:2814–2823. DOI: 10.1002/cncr.28721. PMID: 24771552.