先天性胆道拡張症に合併したmixed adenoneuroendocrine carcinoma(MANEC)の1例  [in Japanese] A case of mixed adenoneuroendocrine carcinoma (MANEC) with congenital biliary dilatation  [in Japanese]

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Abstract

<p>症例は42歳男性.先天性胆道拡張症 戸谷Ia型に対して嚢腫切除+肝外胆管切除(分流手術)施行され,経過観察されていた.術後2年半の精査で膵内遺残胆管に隆起性病変を認め,乳頭浸潤型の膵内遺残胆管癌と術前診断し,亜胃温存膵頭十二指腸切除を施行した(SSPPD).切除病理標本は,異型腺管を伴う腺癌成分30%と胆管内腔に隆起するNeuroendocrine carcinoma成分70%の比較的明瞭な境界をもつ像を呈していた.Stage IB(UICC:pT2N0M0 fStage IB)の混合型腺神経内分泌癌(mixed adenoneuroendocrine carcinoma:MANEC)と最終診断した.補助療法は施行せず,厳重経過観察中である.先天性胆道拡張症の手術時に拡張胆管を残存させない術式が肝要である.本症例は,先天性胆道拡張症に合併した稀なMANECの1例であり,文献的考察を加え報告する.</p>

<p>This case is of a 42-years-old man with a history of surgery for congenital biliary dilatation. Two years after surgery, a 25-mm mass was indicated at the intrapancreatic bile duct or residual cyst on computed tomography (CT) and magnetic resonance imaging (MRI). After confirming a diagnosis of residual bile duct or cyst carcinoma, we performed surgical resection. Pathological examination indicated the presence of neuroendocrine carcinoma (NEC) and tubular adenocarcinoma components in the mass, each occupying 70% and 30% of the mass, respectively. Based on these findings, the final diagnosis was MANEC at the residual cyst. The patient remains alive more than 5 months after diagnosis, without adjuvant therapy. According to previous reports, MANEC of the bile duct is a disease with poor prognosis. Moreover, carcinoma of the residual bile duct/cyst also exhibits poor prognosis. Therefore, surgical treatment for congenital biliary tract dilatation should not leave a cyst. This is the rare case report of MANEC with congenital biliary dilatation.</p>

Journal

  • Tando

    Tando 32(2), 295-306, 2018

    Japan Biliary Association

Codes

  • NII Article ID (NAID)
    130007396494
  • Text Lang
    JPN
  • ISSN
    0914-0077
  • Data Source
    J-STAGE 
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