Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report.

Abstract

Mixed adenoneuroendocrine carcinomas (MANECs) are rare biphasic tumour types, which are morphologically recognisable as both gland-forming and neuroendocrine neoplasms. Within the gastrointestinal tract, MANECs occur predominantly in the stomach or colorectum. The present study described a case of a MANEC originating from the ampullary region. The patient presented with widespread metastatic disease. Biopsy samples obtained from the ampullary primary tumour disclosed a complex lesion with adenocarcinoma and neuroendocrine small cell carcinoma components, positive for the intestinal transcription factor caudal type homeobox-2 and for neuroendocrine markers, including chromogranin A, synaptophysin, cluster of differentiation 56/neural cell adhesion molecule. By contrast, biopsy samples obtained from metastatic tissue revealed pure neuroendocrine carcinoma. As exemplified by this true mixed tumour, tumour heterogeneity evolves as the major challenge in oncology today, with potentially severe implications for the choice of chemotherapy. The assessment of metastatic sites may render valuable diagnostic information that is crucial for clinical decision-making and patient management.