Large cell neuroendocrine carcinoma of the ampulla of Vater with glandular differentiation
- 1Department of Surgery, Mackay Memorial Hospital, Taipei 10449, Taiwan
- 2Department of Pathology, Mackay Memorial Hospital
- 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.
