Extra-pulmonary neuroendocrine carcinomas in the biliary system: Two case reports and a literature review

Masaru Baba, Ken Furuya, Tadashi Koizumi, Kuniaki Sadaoka

Abstract


Primary biliary tract neuroendocrine tumors (NET) are extremely rare and only 237cases have been published to date. Herein, we describe two patients with neuroendocrine carcinoma (NEC) of the biliary system and present a literature review of this rare malignancy. The overall and disease-free survival tended to be shorter among patients with biliary NEC than biliary adenocarcinomas, probably because a definitive preoperative diagnosis and effective treatment options for advanced biliary NEC are unavailable. Therefore, a precise preoperative diagnosis and adjuvant and/or neoadjuvant therapy including chemoradiation leading to radical surgery appeared important to improve the prognosis of patients with biliary NEC.

Keywords


NEC; NET; Neuroendocrine

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DOI: http://dx.doi.org/10.18103/imr.v2i9.198

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