A Long-term Recurrence-free Survival of a Patient with the Mixed Adeno-neuroendocrine Bile Duct Carcinoma: A Case Report and Review of the Literature

Overview

Journal Int J Surg Case Rep

Specialty General Surgery

Date 2017 Aug 15

PMID 28806619

Citations 6

Authors

Wataru Izumo

Ryota Higuchi

Takehisa Yazawa

Shuichiro Uemura

Yutaro Matsunaga

Masahiro Shiihara

Toru Furukawa

Masakazu Yamamoto

Affiliations

Soon will be listed here.

Abstract

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 10mm×8mm 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 10mm. 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.

Citing Articles

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Mixed adenoneuroendocrine carcinoma of the hepatic bile duct: a case report and review of the literature.

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