The authors declare that they have no competing interests.
YK performed the surgery, wrote the case report, made literature review and drafted the manuscript. HY is the senior surgeon who performed all operations assisted by the authors, and revised the manuscript. TU is the gastroenterologist and performed preoperative examination. MN is the pathologist, performed conventional and immunohistochemical staining of NEC of extrahepatic biliary tract, and revised the manuscript. TH is the chief of our division of general surgery, and revised the manuscript. All authors read and approved the final manuscript.
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.
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.
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.
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.
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.
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
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
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
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
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
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
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
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
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
- A case report of primary neuroendocrine carcinoma of the perihilar bile duct
- BioMed Central
Neu im Fachgebiet Chirurgie
Mail Icon II