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父方の被膜の小細胞神経内分泌癌の1例
A case of small cell neuroendocrine carcinoma of the ampulla of Vater.
PMID: 32592083 PMCID: PMC7320127. DOI: 10.1186/s40792-020-00915-9.
抄録
背景:
消化器神経内分泌腫瘍（NET）の6.2％が消化器神経内分泌腫瘍（NEC）であり、消化器NETの1％以下にしか発生しない。AoVのNECの臨床的特徴は不明瞭なままである。
BACKGROUND: Gastroenteric neuroendocrine carcinomas (NECs) account for 6.2% of gastroenteric neuroendocrine tumors (NETs), and only 1% or less of gastroenteric NETs occur in the ampulla of Vater (AoV). Clinical features of NEC of the AoV remain obscure.
ケースプレゼンテーション:
65歳男性が黄疸のため開業医を受診し，腹部造影CT検査で直径11mmの腫瘍を認め，十二指腸乳頭で動脈相が亢進し，上流の胆管が拡張していることを確認した．消化管スコープではAoVの未露出腫瘍を認めた。生検の結果，中等度に分化した管状腺癌を疑い，膵十二指腸切除術を施行した．病理組織学的検査では、異形成と高度に増殖した小腫瘍細胞を認め、AoV部には固形・結節形成が認められた。組織学的解析では高い有糸分裂数を示し，免疫組織化学染色ではKi-67指数が40～50％，シナプトフィシン，クロモグラニンA，p53に陽性の細胞が認められた。最終的に小細胞型NECと診断された。膵頭十二指腸切除術後4ヶ月で多発性肝転移を発症し、全身化学療法を施行した。膵十二指腸切除術から14ヵ月後に肝転移に対するサルベージ肝切除術を行った。残念ながら肝切除後2ヵ月後に多発性肝転移が発生し、膵頭十二指腸切除術後18ヵ月後に死亡した。
CASE PRESENTATION: A 65-year-old man visited a general practitioner because of jaundice, and an abdominal contrast-enhanced computed tomography scan revealed a tumor of 11 mm in diameter, which was enhanced in the arterial phase at the duodenal papilla, with dilation of the upstream bile duct. Gastrointestinal scope revealed an unexposed tumor of the AoV. Based on a biopsy of the site, a moderately differentiated tubular adenocarcinoma was suspected, and pancreatoduodenectomy was performed. Histopathological examination revealed dysplasia and highly proliferative small tumor cells, with solid and nodular formation at the AoV. Histological analysis showed a high mitotic count, and immunohistochemical staining revealed a Ki-67 index of 40-50% and cells positive for synaptophysin, chromogranin A, and p53. Small cell-type NEC was finally diagnosed. Four months post pancreatoduodenectomy, multiple liver metastases developed, and systemic chemotherapy was administered. Salvage liver resection for liver metastases was performed 14 months after the pancreatoduodenectomy. Unfortunately, multiple liver metastases developed 2 months after liver resection, and the patient died 18 months after the pancreatoduodenectomy.
結論:
胆管由来の神経内分泌がんは非常にまれであるため、本論文では文献のレビューと症例報告を行う。
CONCLUSIONS: Neuroendocrine carcinoma originating from the bile duct is very rare; therefore, in this article, we provide a review of the literature and a case report.