Article

Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater with Adenocarcinoma and Squamous Cell Carcinoma Components

Department of Thoracic and Visceral Organ Surgery, Gunma University Graduate School of Medicine, Maebashi, Gunma 371-8511, Japan.
Japanese Journal of Clinical Oncology (Impact Factor: 2.02). 03/2011; 41(3):434-9. DOI: 10.1093/jjco/hyq186
Source: PubMed

ABSTRACT

A 73-year-old woman visited our hospital complaining of general fatigue and jaundice. Laboratory tests revealed an elevated
total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and γ-glutamyltransferase. Computed
tomograrphy and magnetic resonance imaging demonstrated a mass lesion at the ampulla of Vater with dilatation of the common
bile duct and main pancreatic duct. Percutaneous transhepatic cholangiography revealed dilatation of the bile duct and a negative
filling defect due to the tumor. Pancreatoduodenectomy was performed. The specimen included an ulcerated firm tumor of the
papilla Vater. The surface of the ampulla consisted of well-differentiated papillary adenocarcinoma, whereas the deep layer,
such as submucosal or muscular layer, contained large cell neuroendocrine carcinoma and squamous cell carcinoma. Immunohistochemistry
revealed that the large cell neuroendocrine carcinoma component was positive for chromogranin A, synaptophysin and CD56. The
patient died from multiple liver and bone metastases 13 months after surgery. This is a very rare case of a large cell neuroendocrine
carcinoma accompanied by adenocarcinoma and squamous cell carcinoma components.

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