TY - JOUR
T1 - A long-term recurrence-free survival of a patient with the mixed adeno-neuroendocrine bile duct carcinoma
T2 - A case report and review of the literature
AU - Izumo, Wataru
AU - Higuchi, Ryota
AU - Yazawa, Takehisa
AU - Uemura, Shuichiro
AU - Matsunaga, Yutaro
AU - Shiihara, Masahiro
AU - Furukawa, Toru
AU - Yamamoto, Masakazu
N1 - Publisher Copyright:
© 2017 The Author(s)
PY - 2017
Y1 - 2017
N2 - Introduction Neuroendocrine tumors arising primarily in the bile duct are rare. And among these tumors, mixed adeno-neuroendocrine carcinoma (MANEC) is quite uncommon. We report a patient with MANEC who achieved long-term recurrence-free survival. And our case report includes analysis previous case reports. Presentation of case A 66-year-old man underwent investigation for persistent anorexia and fatigue. Laboratory tests showed that the values of hepatobiliary enzymes were increased. On CT, a 10 mm × 8 mm hypervascular tumor was observed in the distal bile duct and the proximal bile duct was markedly dilated. Endoscopic retrograde cholangiography (ERC) also showed a stenosis with a long diameter of 10 mm. Examination of a biopsy specimen obtained from the narrow site of the bile duct at the time of ERC revealed tubular adenocarcinoma. Therefore, pylorus-preserving pancreaticoduodenectomy was performed under a preoperative diagnosis of distal bile duct carcinoma. Postoperative pathologic examination revealed alveolar structures and a mixture of moderately differentiated adenocarcinoma with synaptophysin-positive and chromogranin-A-positive neuroendocrine carcinoma. Therefore, the final diagnosis was MANEC, pT3, pN1, M0, pStage II B (TNM classification of the UICC). Curative resection was achieved and there has been no recurrence after 30 months. Discussion In the previous reports, only five patients (14.7%) survived for 24 months or longer. Median survival was longer (14 months) in the curative resection group and shorter (6 months) in the non-curative resection group. Conclusion Curative resection is essential to achieve long-term survival in patients with bile duct MANEC, even if these patients have lymph node metastasis.
AB - Introduction Neuroendocrine tumors arising primarily in the bile duct are rare. And among these tumors, mixed adeno-neuroendocrine carcinoma (MANEC) is quite uncommon. We report a patient with MANEC who achieved long-term recurrence-free survival. And our case report includes analysis previous case reports. Presentation of case A 66-year-old man underwent investigation for persistent anorexia and fatigue. Laboratory tests showed that the values of hepatobiliary enzymes were increased. On CT, a 10 mm × 8 mm hypervascular tumor was observed in the distal bile duct and the proximal bile duct was markedly dilated. Endoscopic retrograde cholangiography (ERC) also showed a stenosis with a long diameter of 10 mm. Examination of a biopsy specimen obtained from the narrow site of the bile duct at the time of ERC revealed tubular adenocarcinoma. Therefore, pylorus-preserving pancreaticoduodenectomy was performed under a preoperative diagnosis of distal bile duct carcinoma. Postoperative pathologic examination revealed alveolar structures and a mixture of moderately differentiated adenocarcinoma with synaptophysin-positive and chromogranin-A-positive neuroendocrine carcinoma. Therefore, the final diagnosis was MANEC, pT3, pN1, M0, pStage II B (TNM classification of the UICC). Curative resection was achieved and there has been no recurrence after 30 months. Discussion In the previous reports, only five patients (14.7%) survived for 24 months or longer. Median survival was longer (14 months) in the curative resection group and shorter (6 months) in the non-curative resection group. Conclusion Curative resection is essential to achieve long-term survival in patients with bile duct MANEC, even if these patients have lymph node metastasis.
KW - Bile duct
KW - Case report
KW - Long term survival
KW - MANEC
KW - Mixed adeno-neuroendocrine carcinoma
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U2 - 10.1016/j.ijscr.2017.07.052
DO - 10.1016/j.ijscr.2017.07.052
M3 - Article
AN - SCOPUS:85032286932
VL - 39
SP - 43
EP - 50
JO - International Journal of Surgery Case Reports
JF - International Journal of Surgery Case Reports
SN - 2210-2612
ER -