CASE REPORT

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

S-P Cheng¹, T-L Yang¹, K-M Chang² and C-L Liu¹

¹ Department of Surgery, Mackay Memorial Hospital, Taipei 10449, Taiwan
² Department of Pathology, Mackay Memorial Hospital

Correspondence to:
Correspondence to:
Dr T-L Yang
Department of Surgery, Mackay Memorial Hospital, 92, Sec 2, Chung-Shan North Road, Taipei 10449, Taiwan; yangtl{at}yam.com

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.

Abbreviations: LCNEC, large cell neuroendocrine carcinoma

Keywords: ampulla of Vater; CD117; KIT; large cell neuroendocrine carcinoma; mixed tumour

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