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J Clin Pathol 2004;57:1098-1100 doi:10.1136/jcp.2004.019414
  • Case report

Large cell neuroendocrine carcinoma of the ampulla of Vater with glandular differentiation

  1. S-P Cheng1,
  2. T-L Yang1,
  3. K-M Chang2,
  4. C-L Liu1
  1. 1Department of Surgery, Mackay Memorial Hospital, Taipei 10449, Taiwan
  2. 2Department of Pathology, Mackay Memorial Hospital
  1. Correspondence to:
 Dr T-L Yang
 Department of Surgery, Mackay Memorial Hospital, 92, Sec 2, Chung-Shan North Road, Taipei 10449, Taiwan; yangtlyam.com
  • Revised 8 May 2004

Abstract

Large cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare. A 55 year old woman presented with an ampullary tumour causing pancreaticobiliary obstruction and a pancreaticoduodenectomy was performed. Microscopically, the tumour was diagnosed as a CD117 positive large cell neuroendocrine carcinoma with glandular differentiation. Four months later the patient developed a general recurrence. The metastatic tumours showed CD117 negativity and pure neuroendocrine features. The patient died of disease six months after diagnosis. It is postulated that the two components originated from a common multipotential stem cell. The clinical behaviour of ampullary large cell neuroendocrine carcinomas appears to be highly aggressive, with early metastases and a fatal outcome.

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