A mixed adenoneuroendocrine carcinoma of the pancreas: a case report

Abstract

A tumor consisting of an adenocarcinoma component and a neuroendocrine carcinoma component, with each component accounting for at least 30% of the tumor, is defined as a mixed adenoneuroendocrine carcinoma (MANEC). We report a case of MANEC of the pancreas. A 63-year-old man presented with hyperglycemia and was referred to our hospital for further examination. Abdominal contrast-enhanced computed tomography (CT) revealed a mass of 2 cm in size in the pancreas head with portal vein narrowing. Fluorin-18 fluorodeoxyglucose-positron emission tomography (FDG-PET) CT revealed increased accumulation in the mass of the pancreas head. Endoscopic retrograde cholangiopancreatography (ERCP) showed severe narrowing of the main pancreatic duct. Cytological analysis by endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) suggested a neuroendocrine tumor. Under the diagnosis of neuroendocrine tumor, pancreaticoduodenectomy with portal vein resection and regional lymph node dissection was performed with curative intent. Histological examination revealed that the tumor consisted of two cell populations. One was well- to moderately differentiated tubular adenocarcinoma. This cell component accounted for 45% of the whole tumor. The second component was non-adenocarcinoma cells arranged in a nest, and the cells had round nuclei, abundant cytoplasm, and coarse chromatin. The Ki67 labeling index was 40%. Immunohistochemically, the adenocarcinoma cells were positive for CEA but negative for chromogranin A (CgA) and synaptophysin (Syn), while the non-adenocarcinoma cells were positive for the expression of CgA and Syn but negative for CEA. Based on the findings, a diagnosis of MANEC of the pancreas was made. Postoperatively, lymph node metastasis and peritoneal dissemination developed rapidly and he died the 6 months after the operation. Due to the small number of reported cases of MANEC of the pancreas, its clinical behavior remains unclear and a standardized management protocol has not been established. Further investigation of more cases of this rare entity is necessary.

Fig. 1

Abdominal contrast-enhanced computed tomography (CT) showed diffuse enlargement of the pancreas with increased CT level in the peri-pancreatic fatty tissue and revealed a mass of 2 cm in size in the pancreas head (c–d, arrow). The mass was poorly enhanced in the arterial phase and was gradually enhanced in the venous phase. The portal vein showed narrowing (b–f, arrowhead)

Fig. 2

PET-CT revealed increased accumulation in the mass of the pancreas head (maximum standardized uptake value (SUV max): 5.6 (initial) and 7.8 (delayed) (a). Endoscopic retrograde cholangiopancreatography (ERCP) showed severe narrowing and obstruction of the main pancreatic duct by the tumor (arrow) (b). Endoscopic ultrasonography (EUS) showed the tumor as a low echoic mass (c). Cytological analysis by means of EUS-guided fine-needle aspiration (FNA) suggested a neuroendocrine tumor (G2) (d)

Fig. 3

Macroscopically, a 2-cm-sized mass in the pancreas head was presented as the resected specimen. Histological examination revealed that the tumor consisted of two cell populations. The white-shaded area indicates a component of well- to moderately differentiated tubular adenocarcinoma cells and the gray-shaded area indicates a component of non-adenocarcinoma cells (a). The adenocarcinoma cells were arranged in an irregular pattern (b). Non-adenocarcinoma cells are arranged in a nest, and the cells had round nuclei, abundant cytoplasm, and coarse chromatin (c). An intermixed central zone exists between the two cell components (d) (asterisk part in Fig. 3a)

Fig. 4

Immunohistochemically, the non-adenocarcinoma cell components were positive for the expression of CgA and Syn (a, b), but negative for CEA (c). The Ki67 labeling index was 40% (d)