Skip to main content
Top
Published in: BMC Surgery 1/2015

Open Access 01-12-2015 | Case report

A case report of primary neuroendocrine carcinoma of the perihilar bile duct

Authors: Yasuhiro Kihara, Hiroshi Yokomizo, Takahiro Urata, Michiko Nagamine, Toshihiko Hirata

Published in: BMC Surgery | Issue 1/2015

Login to get access

Abstract

Background

Although neuroendocrine tumors are most commonly found in the digestive system, neuroendocrine tumors originating from the bile duct are rare, and neuroendocrine carcinomas derived from the perihilar bile duct are extremely rare. This report presents the clinical course and clinicopathological features of neuroendocrine carcinomas arising from the extrahepatic bile duct.

Case presentation

A 70-year-old Japanese woman was preoperatively diagnosed with perihilar cholangiocarcinoma, and a radical resection with an extended left hepatic lobectomy and a choledochojejunostomy was performed. From the histopathological findings, we diagnosed the tumor as a neuroendocrine carcinoma of the bile duct (small cell type) with lymph node metastasis. The patient was treated with the same adjuvant chemotherapy as that used for small cell carcinoma of the lung. At 10 months after surgery, there was no recurrence of the disease.

Conclusion

Neuroendocrine carcinoma of the extrahepatic biliary tracts is a very rare and highly malignant disease with a poor prognosis. A multidisciplinary approach could improve the prognosis for this neoplasm.
Literature
1.
go back to reference Komminoth P, Arnold R, Capella C, Klimstra DS, Kloppel G, Rindi G, et al. Neuroendocrine neoplasms of the gallbladder and extrahepatic bile ducts. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2010. p. p274–6. Komminoth P, Arnold R, Capella C, Klimstra DS, Kloppel G, Rindi G, et al. Neuroendocrine neoplasms of the gallbladder and extrahepatic bile ducts. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2010. p. p274–6.
2.
go back to reference Chamberlain RS, Blumgart LH. Carcinoid tumors of the extrahepatic bile duct. A rare cause of malignant biliary obstruction. Cancer. 1999;86:1959–65.CrossRefPubMed Chamberlain RS, Blumgart LH. Carcinoid tumors of the extrahepatic bile duct. A rare cause of malignant biliary obstruction. Cancer. 1999;86:1959–65.CrossRefPubMed
3.
go back to reference Rindi G, Arnold R, Bosman FT, Capella C, Klimstra DS, Kloppel G, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2010. p. 13–4. Rindi G, Arnold R, Bosman FT, Capella C, Klimstra DS, Kloppel G, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2010. p. 13–4.
4.
go back to reference Suzuki S, Tanaka S, Hayashi T, Harada N, Suzuki M, Hanyu F, et al. Small-cell neuroendocrine carcinoma of the ampulla of Vater. J Hepatobiliary Pancreat Surg. 2006;13:450–3.CrossRefPubMed Suzuki S, Tanaka S, Hayashi T, Harada N, Suzuki M, Hanyu F, et al. Small-cell neuroendocrine carcinoma of the ampulla of Vater. J Hepatobiliary Pancreat Surg. 2006;13:450–3.CrossRefPubMed
5.
go back to reference Nakamura Y, Tajiri T, Uchida E, Arima Y, Aimoto T, Katsuno A, et al. Changes to levels of serum neuron-specific enolase in a patient with small-cell carcinoma of the pancreas. J Hepatobiliary Pancreat Surg. 2005;12:93–8.CrossRefPubMed Nakamura Y, Tajiri T, Uchida E, Arima Y, Aimoto T, Katsuno A, et al. Changes to levels of serum neuron-specific enolase in a patient with small-cell carcinoma of the pancreas. J Hepatobiliary Pancreat Surg. 2005;12:93–8.CrossRefPubMed
6.
go back to reference Eriguchi N, Aoyagi S, Noritomi T, Imamura M, Sato S, Fujiki K, et al. Adeno-endocrine cell carcinoma of the gallbladder. J Hepatobiliary Pancreat Surg. 2000;7:97–101.CrossRefPubMed Eriguchi N, Aoyagi S, Noritomi T, Imamura M, Sato S, Fujiki K, et al. Adeno-endocrine cell carcinoma of the gallbladder. J Hepatobiliary Pancreat Surg. 2000;7:97–101.CrossRefPubMed
7.
go back to reference Sabanathan S, Hashimi H, Nicholson G, Edwards AS. Primary oat cell carcinoma of the common bile duct. J R Coll Surg Edinb. 1988;33:285–6.PubMed Sabanathan S, Hashimi H, Nicholson G, Edwards AS. Primary oat cell carcinoma of the common bile duct. J R Coll Surg Edinb. 1988;33:285–6.PubMed
8.
go back to reference Van der Wal AC, Van Leeuwen DJ, Walford N. Small cell neuroendocrine (oat cell) tumour of the common bile duct. Histopathology. 1990;16:398–400.CrossRefPubMed Van der Wal AC, Van Leeuwen DJ, Walford N. Small cell neuroendocrine (oat cell) tumour of the common bile duct. Histopathology. 1990;16:398–400.CrossRefPubMed
9.
go back to reference Nishihara K, Tsuneyoshi M, Niiyama H, Ichimiya H. Composite glandular-endocrine cell carcinoma of the extrahepatic bile duct: immunohistochemical study. Pathology. 1993;25:90–4.CrossRefPubMed Nishihara K, Tsuneyoshi M, Niiyama H, Ichimiya H. Composite glandular-endocrine cell carcinoma of the extrahepatic bile duct: immunohistochemical study. Pathology. 1993;25:90–4.CrossRefPubMed
10.
go back to reference Yamamoto J, Abe Y, Nishihara K, Katsumoto F, Takeda S, Abe R, et al. Composite glandular-neuroendocrine carcinoma of the hilar bile duct: report of a case. Surg Today. 1998;28:758–62.CrossRefPubMed Yamamoto J, Abe Y, Nishihara K, Katsumoto F, Takeda S, Abe R, et al. Composite glandular-neuroendocrine carcinoma of the hilar bile duct: report of a case. Surg Today. 1998;28:758–62.CrossRefPubMed
11.
go back to reference Kim SH, Park YN, Yoon DS, Lee SJ, Yu JS, Noh TW. Composite neuroendocrine and adenocarcinoma of the common bile duct associated with Clonorchis sinensis: a case report. Hepatogastroenterology. 2000;47:942–4.PubMed Kim SH, Park YN, Yoon DS, Lee SJ, Yu JS, Noh TW. Composite neuroendocrine and adenocarcinoma of the common bile duct associated with Clonorchis sinensis: a case report. Hepatogastroenterology. 2000;47:942–4.PubMed
12.
go back to reference Miyashita T, Konishi K, Noto M, Taniguchi K, Kaji M, Kimura H, et al. A case of small cell carcinoma of the common bile duct. Nihon Shokakibyo Gakkai Zasshi. 2001;98:1195–8.PubMed Miyashita T, Konishi K, Noto M, Taniguchi K, Kaji M, Kimura H, et al. A case of small cell carcinoma of the common bile duct. Nihon Shokakibyo Gakkai Zasshi. 2001;98:1195–8.PubMed
13.
go back to reference Edakuni G, Sasatomi E, Satoh T, Tokunaga O, Miyazaki K. Composite glandular-endocrine cell carcinoma of the common bile duct. Pathol Int. 2001;51:487–90.CrossRefPubMed Edakuni G, Sasatomi E, Satoh T, Tokunaga O, Miyazaki K. Composite glandular-endocrine cell carcinoma of the common bile duct. Pathol Int. 2001;51:487–90.CrossRefPubMed
14.
go back to reference Kuraoka K, Taniyama K, Fujitaka T, Nakatsuka H, Nakayama H, Yasui W. Small cell carcinoma of the extrahepatic bile duct: case report and immunohistochemical analysis. Pathol Int. 2003;53:887–91.CrossRefPubMed Kuraoka K, Taniyama K, Fujitaka T, Nakatsuka H, Nakayama H, Yasui W. Small cell carcinoma of the extrahepatic bile duct: case report and immunohistochemical analysis. Pathol Int. 2003;53:887–91.CrossRefPubMed
15.
go back to reference Hazama K, Suzuki Y, Takahashi M, Takahashi Y, Yoshioka T, Takano S, et al. Primary small cell carcinoma of the common bile duct, in which surgical treatment was performed after neoadjuvant chemotherapy: report of a case. Surg Today. 2003;33:870–2.CrossRefPubMed Hazama K, Suzuki Y, Takahashi M, Takahashi Y, Yoshioka T, Takano S, et al. Primary small cell carcinoma of the common bile duct, in which surgical treatment was performed after neoadjuvant chemotherapy: report of a case. Surg Today. 2003;33:870–2.CrossRefPubMed
16.
go back to reference Arakura N, Hasebe O, Yokosawa S, Imai Y, Furuta S, Hosaka N. A case of small cell carcinoma of the extrahepatic bile duct which could be diagnosed before operation. Nihon Shokakibyo Gakkai Zasshi. 2003;100:190–4.PubMed Arakura N, Hasebe O, Yokosawa S, Imai Y, Furuta S, Hosaka N. A case of small cell carcinoma of the extrahepatic bile duct which could be diagnosed before operation. Nihon Shokakibyo Gakkai Zasshi. 2003;100:190–4.PubMed
17.
go back to reference Park HW, Seo SH, Jang BK, Hwang JY, Park KS, Cho KB, et al. A case of primary small cell carcinoma in the common bile duct. Korean J Gastroenterol. 2004;43:260–3.PubMed Park HW, Seo SH, Jang BK, Hwang JY, Park KS, Cho KB, et al. A case of primary small cell carcinoma in the common bile duct. Korean J Gastroenterol. 2004;43:260–3.PubMed
18.
go back to reference Thomas NE, Burroughs FH, Ali SZ. Small-cell carcinoma of the extrahepatic bile duct and concurrent clonorchiasis. Diagn Cytopathol. 2005;32:92–3.CrossRefPubMed Thomas NE, Burroughs FH, Ali SZ. Small-cell carcinoma of the extrahepatic bile duct and concurrent clonorchiasis. Diagn Cytopathol. 2005;32:92–3.CrossRefPubMed
19.
go back to reference Kaiho T, Tanaka T, Tsuchiya S, Yanagisawa S, Takeuchi O, Miura M, et al. A case of small cell carcinoma of the common bile duct. Hepatogastroenterology. 2005;52:363–7.PubMed Kaiho T, Tanaka T, Tsuchiya S, Yanagisawa S, Takeuchi O, Miura M, et al. A case of small cell carcinoma of the common bile duct. Hepatogastroenterology. 2005;52:363–7.PubMed
20.
go back to reference Sato K, Waseda R, Tatsuzawa Y, Fujinaga H, Wakabayashi T, Ueda Y, et al. Composite large cell neuroendocrine carcinoma and adenocarcinoma of the common bile duct. J Clin Pathol. 2006;59:105–7.PubMedCentralCrossRefPubMed Sato K, Waseda R, Tatsuzawa Y, Fujinaga H, Wakabayashi T, Ueda Y, et al. Composite large cell neuroendocrine carcinoma and adenocarcinoma of the common bile duct. J Clin Pathol. 2006;59:105–7.PubMedCentralCrossRefPubMed
21.
go back to reference Viana Miguel MM, García-Plata Polo E, Vidal Doce O, Aldea Martínez J, de la Plaza GM, Santamaría García JL. Oat cell carcinoma of the common bile duct. Cir Esp. 2006;80:43–5.CrossRefPubMed Viana Miguel MM, García-Plata Polo E, Vidal Doce O, Aldea Martínez J, de la Plaza GM, Santamaría García JL. Oat cell carcinoma of the common bile duct. Cir Esp. 2006;80:43–5.CrossRefPubMed
22.
go back to reference Jeon WJ, Chae HB, Park SM, Youn SJ, Choi JW, Kim SH. A case of primary small cell carcinoma arising from the common bile duct. Korean J Gastroenterol. 2006;48:438–42.PubMed Jeon WJ, Chae HB, Park SM, Youn SJ, Choi JW, Kim SH. A case of primary small cell carcinoma arising from the common bile duct. Korean J Gastroenterol. 2006;48:438–42.PubMed
23.
go back to reference Nakai N, Takenaka H, Hamada S, Kishimoto S. Identical p53 gene mutation in malignant proliferating trichilemmal tumour of the scalp and small cell carcinoma of the common bile duct: the necessity for therapeutic caution? Br J Dermatol. 2008;159:482–5.CrossRefPubMed Nakai N, Takenaka H, Hamada S, Kishimoto S. Identical p53 gene mutation in malignant proliferating trichilemmal tumour of the scalp and small cell carcinoma of the common bile duct: the necessity for therapeutic caution? Br J Dermatol. 2008;159:482–5.CrossRefPubMed
24.
go back to reference Arakura N, Muraki T, Komatsu K, Ozaki Y, Hamano H, Tanaka E, et al. Small cell carcinoma of the extrahepatic bile duct diagnosed with EUS-FNA and effectively treated with chemoradiation. Intern Med. 2008;47:621–5.CrossRefPubMed Arakura N, Muraki T, Komatsu K, Ozaki Y, Hamano H, Tanaka E, et al. Small cell carcinoma of the extrahepatic bile duct diagnosed with EUS-FNA and effectively treated with chemoradiation. Intern Med. 2008;47:621–5.CrossRefPubMed
25.
go back to reference Hosonuma K, Sato K, Honma M, Kashiwabara K, Takahashi H, Takagi H, et al. Small-cell carcinoma of the extrahepatic bile duct: a case report and review of the literature. Hepatol Int. 2008;2:129–32.PubMedCentralCrossRefPubMed Hosonuma K, Sato K, Honma M, Kashiwabara K, Takahashi H, Takagi H, et al. Small-cell carcinoma of the extrahepatic bile duct: a case report and review of the literature. Hepatol Int. 2008;2:129–32.PubMedCentralCrossRefPubMed
26.
go back to reference Okamura Y, Maeda A, Matsunaga K, Kanemoto H, Boku N, Furukawa H, et al. Small-cell carcinoma in the common bile duct treated with multidisciplinary management. J Hepatobiliary Pancreat Surg. 2009;16:575–8.CrossRefPubMed Okamura Y, Maeda A, Matsunaga K, Kanemoto H, Boku N, Furukawa H, et al. Small-cell carcinoma in the common bile duct treated with multidisciplinary management. J Hepatobiliary Pancreat Surg. 2009;16:575–8.CrossRefPubMed
27.
go back to reference Yamaguchi Y, Shimizu H, Yoneda E. A case of recurrent endocrine cell carcinoma of the common bile duct successfully treated by hepatic artery infusion with CPT-11 and CDDP. Gan To Kagaku Ryoho. 2009;36:823–5.PubMed Yamaguchi Y, Shimizu H, Yoneda E. A case of recurrent endocrine cell carcinoma of the common bile duct successfully treated by hepatic artery infusion with CPT-11 and CDDP. Gan To Kagaku Ryoho. 2009;36:823–5.PubMed
28.
go back to reference Demoreuil C, Thirot-Bidault A, Dagher C, Bou-Farah R, Benbrahem C, Lazure T, et al. Poorly differentiated large cell endocrine carcinoma of the extrahepatic bile ducts. Gastroenterol Clin Biol. 2009;33:194–8.CrossRefPubMed Demoreuil C, Thirot-Bidault A, Dagher C, Bou-Farah R, Benbrahem C, Lazure T, et al. Poorly differentiated large cell endocrine carcinoma of the extrahepatic bile ducts. Gastroenterol Clin Biol. 2009;33:194–8.CrossRefPubMed
29.
go back to reference Sasatomi E, Nalesnik MA, Marsh JW. Neuroendocrine carcinoma of the extrahepatic bile duct: Case report and literature review. World J Gastroenterol. 2013;19(28):4616–23.PubMedCentralCrossRefPubMed Sasatomi E, Nalesnik MA, Marsh JW. Neuroendocrine carcinoma of the extrahepatic bile duct: Case report and literature review. World J Gastroenterol. 2013;19(28):4616–23.PubMedCentralCrossRefPubMed
30.
go back to reference Levenson RM, Ihde DC, Matthews MJ, Cohen MH, Gazdar AF, Bunn PA, et al. Small cell carcinoma presenting as an extrapulmonary neoplasm: sites of origin and response to chemotherapy. J Natl Cancer Inst. 1981;67:607–12.PubMed Levenson RM, Ihde DC, Matthews MJ, Cohen MH, Gazdar AF, Bunn PA, et al. Small cell carcinoma presenting as an extrapulmonary neoplasm: sites of origin and response to chemotherapy. J Natl Cancer Inst. 1981;67:607–12.PubMed
31.
go back to reference Dumitrascu T, Dima S, Herlea V, Tomulescu V, Ionescu M, Popescu I. Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis. Langenbecks Arch Surg. 2012;397(6):933–43.PubMedCentralCrossRefPubMed Dumitrascu T, Dima S, Herlea V, Tomulescu V, Ionescu M, Popescu I. Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis. Langenbecks Arch Surg. 2012;397(6):933–43.PubMedCentralCrossRefPubMed
32.
go back to reference Kim JH, Lee SH, Park J, Kim HY, Lee SI, Nam EM, et al. Extrapulmonary small-cell carcinoma: a single-institution experience. Jpn J Clin Oncol. 2004;34:250–4.CrossRefPubMed Kim JH, Lee SH, Park J, Kim HY, Lee SI, Nam EM, et al. Extrapulmonary small-cell carcinoma: a single-institution experience. Jpn J Clin Oncol. 2004;34:250–4.CrossRefPubMed
33.
go back to reference Hogan BA, Thornton FJ, Brannigan M, Browne TJ, Pender S, O’Kelly P, et al. Hepatic metastases from an unknown primary neoplasm (UPN): survival, prognostic indicators and value of extensive investigations. Clin Radiol. 2002;57:1073–7.CrossRefPubMed Hogan BA, Thornton FJ, Brannigan M, Browne TJ, Pender S, O’Kelly P, et al. Hepatic metastases from an unknown primary neoplasm (UPN): survival, prognostic indicators and value of extensive investigations. Clin Radiol. 2002;57:1073–7.CrossRefPubMed
34.
go back to reference Quoix E, Breton JL, Daniel C, Jacoulet P, Debieuvre D, Paillot N, et al. Etoposide phosphate with carboplatin in the treatment of elderly patients with small-cell lung cancer: a phase II study. Ann Oncol. 2001;12:957–62.CrossRefPubMed Quoix E, Breton JL, Daniel C, Jacoulet P, Debieuvre D, Paillot N, et al. Etoposide phosphate with carboplatin in the treatment of elderly patients with small-cell lung cancer: a phase II study. Ann Oncol. 2001;12:957–62.CrossRefPubMed
Metadata
Title
A case report of primary neuroendocrine carcinoma of the perihilar bile duct
Authors
Yasuhiro Kihara
Hiroshi Yokomizo
Takahiro Urata
Michiko Nagamine
Toshihiko Hirata
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Surgery / Issue 1/2015
Electronic ISSN: 1471-2482
DOI
https://doi.org/10.1186/s12893-015-0116-z

Other articles of this Issue 1/2015

BMC Surgery 1/2015 Go to the issue