Clinical Journal of Gastroenterology

, Volume 11, Issue 3, pp 240–244 | Cite as

A case of small intestinal neuroendocrine carcinoma diagnosed using double-balloon endoscopy with long-term survival

  • Seiji Kawano
  • Yuichi Miyashima
  • Yoshio Miyabe
  • Yoshinari Kawai
  • Toshihiro Murata
  • Masashi Uda
  • Toshihiro Inokuchi
  • Hiroyuki Okada
Case Report


Neuroendocrine neoplasms, including neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs), are rare epithelial tumors with a predominant neuroendocrine differentiation. Compared with NETs, NECs have been reported to be rarer and have a poorer prognosis. We present a rare case of small bowel NEC diagnosed using double-balloon endoscopy (DBE) and the long-term survival accomplished via intensive therapy. DBE revealed an ulcerative tumor in the deep jejunum, and biopsy specimens showed large and highly dysplastic tumor cells; immuno-histological synaptophysin and chromogranin A tests were positive, and the Ki-67 index was more than 90%. Partial intestinal resection without complete lymph node dissection was performed and, postoperatively, chemotherapy was administered. The patient was observed for 3 years after chemotherapy, and complete remission was maintained.


Neuroendocrine carcinoma Small intestine Double balloon endosopy 


Compliance with ethical standards

Conflict of interest

Seiji Kawano, Yuichi Miyashima, Yoshio Miyabe, Toshihiro Inokuchi and Hiroyuki Okada declare that they have no conflict of interest.

Human rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments..

Informed consent

Informed consent was obtained from all patients for being included in the study.


  1. 1.
    Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumors. Lancet Oncol. 2008;9(1):61–72.CrossRefPubMedGoogle Scholar
  2. 2.
    Bosman FT, Carneiro F, Hruban RH, et al. WHO classification of tumours of the digestive system. 4th edition. Lyon: International Agency for Research on Cancer; 2010.Google Scholar
  3. 3.
    Bosman FT, Carneiro F, Hruban RH, et al. WHO classification of tumours of the digestive system. Geneva, Switzerland: World Health Organization; 2010.Google Scholar
  4. 4.
    Current trends of the incidence and pathological diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in Korea 2000–2009: multicenter study. Cancer Res Treat. 2012;44(3):157–165.Google Scholar
  5. 5.
    Zhang X, Ma L, Bao H, et al. Clinical, pathological and prognostic characteristics of gastroenteropancreatic neuroendocrine neoplasms in China: a retrospective study. BMC Endocr Disord. 2014;8(14):54. Scholar
  6. 6.
    Brierley JD, Gospodarowicz MK, Wittekind C, et al. UICC TNM classification of malignant tumours. Eight Edition. Wiley Blackwell. 2016; 201:99–101.Google Scholar
  7. 7.
    Mitsui K, Tanaka S, Yamamoto H, et al. Role of double-balloon endoscopy in the diagnosis of small-bowel tumors: the first Japanese multicenter study. Gastrointest Endosc. 2009;70(3):498–504.CrossRefPubMedGoogle Scholar
  8. 8.
    Ito T, Igarashi H, Nakamura K, et al. Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis. J Gastroenterol. 2015;50:58–64.CrossRefPubMedGoogle Scholar
  9. 9.
    Yamaguchi T, Machida N, Morizane C, et al. Multicenter retrospective analysis of systemic chemotherapy for advanced neuroendocrine carcinoma of the digestive system. Cancer Sci. 2014;105:1176–81.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Chen J, Zhang L, Zhang W, et al. A case of neuroendocrine malignant tumor with capsule retention diagnosed by double-balloon enteroscopy. Case Rep Gastroenterol. 2010;4(1):52–6.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Martini C, Sturniolo GC, De Carlo E, et al. Neuroendocrine tumor of small bowel. Gastrointest Endosc. 2004;60(3):431.CrossRefPubMedGoogle Scholar
  12. 12.
    Sorbye H, Welin S, Langer SW, et al. Predictive and prognostic factors for treatment and survival in 305 patients with advanced gastrointestinal neuroendocrine carcinoma (WHO G3): the NORDIC NEC study. Ann Oncol. 2013;24(1):152–60.CrossRefPubMedGoogle Scholar
  13. 13.
    National Comprehensive Cancer Network National. Comprehensive Cancer Network (NCCN) guidelines. Available from URL: (Ver. 1 2013).
  14. 14.
    Pavel M, Baudin E, Couvelard A, et al. ENETS Consensus Guidelines for the management of patients with liver and other distant metastases from neuroendocrine neoplasms of foregut, midgut, hindgut, and unknown primary. Neuroendocrinology. 2012;95:157–76.CrossRefPubMedGoogle Scholar
  15. 15.
    Strosberg JR, Coppola D, Klimstra DS, et al. The NANETS consensus guidelines for the diagnosis and management of poorly differentiated (high-grade) extrapulmonary neuroendocrine carcinomas. Pancreas. 2010;39:799–800.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  • Seiji Kawano
    • 1
    • 4
  • Yuichi Miyashima
    • 2
  • Yoshio Miyabe
    • 2
  • Yoshinari Kawai
    • 2
  • Toshihiro Murata
    • 3
  • Masashi Uda
    • 3
  • Toshihiro Inokuchi
    • 1
  • Hiroyuki Okada
    • 1
  1. 1.Department of Gastroenterology and HepatologyOkayama University Graduate School of Medicine, Dentistry, and Pharmaceutical SciencesOkayamaJapan
  2. 2.Department of GastroenterologyOnomichi Municipal HospitalOnomichiJapan
  3. 3.Department of SurgeryOnomichi Municipal HospitalOnomichiJapan
  4. 4.Department of EndoscopyOkayama University HospitalOkayamaJapan

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