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  • Title: Large cell neuroendocrine carcinoma of the ampulla of Vater with glandular differentiation.
    Author: Cheng SP, Yang TL, Chang KM, Liu CL.
    Journal: J Clin Pathol; 2004 Oct; 57(10):1098-100. PubMed ID: 15452169.
    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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