Concurrent ductal adenocarcinoma, pseudocyst, and neuroendocrine tumor of the pancreas: A case report

Introduction: Pancreatic pseudocyst is one of the most common cystic lesions. It always occurs following pancreatitis and is rarely found in combination with pancreatic adenocarcinoma. The coexistence of exocrine and neuroendocrine tumors of the pancreas is also infrequent. We herein report a case of simultaneous occurrence of a pancreatic ductal adenocarcinoma (PDAC), pseudocyst, and neuroendocrine tumor (NET), showing a "side-by-side pattern."

Patient concern: A 74-year-old man was hospitalized for epigastric pain and poor appetite. He had no history of pancreatitis, alcohol consumption, or smoking.

Diagnosis and intervention: Abdominal enhanced computed tomography and magnetic resonance imaging revealed a 15 × 8 cm cystic lesion with poor enhancement located in the tail of the pancreas. The distal aspect of the main pancreatic duct was dilated. The pancreatic parenchyma adjacent to the cystic lesion showed slightly heterogeneous enhancement on computed tomography and magnetic resonance imaging. Laboratory examination showed an elevated carbohydrate antigen 19-9 serum level. The patient was preoperatively diagnosed with intraductal papillary mucinous neoplasm and subsequently underwent laparotomy. During the operation, a hard white tumor measuring about 4 × 3 cm was palpated adjacent to the cystic lesion on the duodenal side, and a 0.6-cm nodule was simultaneously found in the pancreatic tail. Therefore, total pancreatectomy and splenectomy were performed. Histopathological examination showed that the tumor was PDAC with an adjacent pseudocyst, and the small nodule was suggestive of a NET.

Outcomes: The patient survived without recurrence or metastasis in the follow-up visit 10 months after the operation and adjuvant chemotherapy.

Conclusions: The concomitant occurrence of a PDAC, pseudocyst, and NET has not been previously reported. We suggest that if a pancreatic cyst is found, the coincidental occurrence of a malignant tumor should be considered, especially if the carbohydrate antigen 19-9 level is increased. Additionally, dilation of the pancreatic duct may be a diagnostic clue. Furthermore, the simultaneous occurrence of pancreatic endocrine and exocrine tumors is very uncommon. Preoperative diagnosis becomes difficult because of the lack of specific symptoms and radiological features.