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


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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    ABSTRACT: Tumors of Vater's ampulla are generally uncommon. In this location intestinal type adenomas are frequently found, followed by noninvasive papillary neoplasms of the pancreaticobiliary type and neuroendocrine tumors (carcinoids). Carcinomas of Vater's ampulla represent about 0.5% of all gastrointestinal malignancies. Intestinal type adenocarcinoma is the most common malignant epithelial tumor followed by the pancreaticobiliary type adenocarcinoma. Highly malignant neuroendocrine carcinomas of Vater's ampulla are very uncommon. Carcinomas of the ampullary region can be sporadic or a component of several disease syndromes. Designation of large carcinomas as tumors with an ampullary or extra-ampullary origin can be difficult but is of relevance for a TNM conform classification. Helpful in the decision are the relationship between the tumor centre and Vater's ampulla, the existence of premalignant lesions in the ampullary epithelium as well as histology and immunostaining of the tumor.
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    ABSTRACT: Large cell neuroendocrine carcinomas of the ampulla of Vater are rare and confer a very poor prognosis despite aggressive therapy. There are few case reports of large cell neuroendocrine carcinomas of the ampulla of Vater in the literature and to date no studies have been done to establish optimal management. We describe a pooled case series from published reports of neuroendocrine carcinomas of the ampulla of Vater including a case which presented to our institution. A narrative review was undertaken including all published English case reports of large cell neuroendocrine carcinomas of the ampulla of Vater. Our primary outcome was to determine the overall survival. Twenty cases of large cell neuroendocrine carcinomas of the ampulla of Vater were identified. Seventy-six percent of patients were reported to have died of disease with a mean survival of 11.8 months. Twenty percent of the tumours were associated with an adenoma. The approximate median survivals were 15 months for those with an associated adenoma and 11 months without. This pooled analysis demonstrates both the rarity and poor prognosis of large cell neuroendocrine carcinomas of the ampulla of Vater. Although surgical resection is the mainstay of treatment, we review common adjuvant chemotherapy regimes. Prognosis may be improved when these tumours are associated with adenomas, however, further studies are needed.
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