A 54 year-old man with epigastric and back pain was found to have pancreatic tumors at a community hospital and referred to our center. An abdominal CT scan showed poorly enhanced tumors in the head and body of the pancreas. The main pancreatic duct was dilated at the head-body border and the tail of the pancreas, but the tumors were poorly demarcated. The tumor in the pancreatic body involved the splenic vein. MRCP showed interruption and dilatation of the main pancreatic duct in two areas. On FDG-PET scan, abnormal accumulations were detected in the head and body of the pancreas. EUS revealed a nodule in the main pancreatic duct. The preoperative diagnosis was neuroendocrine tumors or acinar cell carcinoma.

We performed a subtotal pylorus-preserving pancreatoduodenectomy with D2 lymph node dissection and splenic vein resection. Pathological diagnosis was acinar cell carcinoma with tumor extension to the main pancreatic duct. Despite receiving adjuvant chemotherapy with S-1, multiple liver metastases were found six months postoperatively, and the patient died nine months after surgery.

Surgical resection is recommended in patients with acinar cell carcinoma, but standard treatment has not yet been established.

A 66-year-old woman with a 25-year history of multiple endocrine neoplasia type 1 presented with left-sided abdominal pain. She was diagnosed with acute pancreatitis, and imaging studies at a local hospital revealed a 3cm tumor in the tail of the pancreas. Further evaluation at our hospital showed elevated serum glucagon (630pg/ml) and gastrin (280pg/ml) levels. A selective arterial calcium injection test suggested a glucagonoma in the tail of the pancreas and a gastrinoma in the hepato-pancreaticoduodenal region. The body and tail of the pancreas was resected to remove the glucagonoma. Computed tomography, magnetic resonance imaging, and endoscopic ultrasound failed to demonstrate any lesion in the pancreas or duodenum, and the patient did not wish to undergo total pancreatectomy/duodenectomy. During three years of postoperative follow-up, the serum gastrin levels in this patient have fluctuated between 150 and 300pg/ml, but there is no evidence of a recurrent glucagonoma or the suspicion of a gastrinoma.

We report a 67-year-old male who was diagnosed with intraductal papillary-mucinous carcinoma (IPMC) in the pancreatic head extending to the common bile duct and duodenum, with portal vein stenosis and thrombosis at the portal vein bifurcation. The patient underwent subtotal pylorus-preserving pancreaticoduodenectomy without portal vein resection. On postoperative day 1, laboratory tests revealed serum AST of 10,965IU/l, ALT of 3,288IU/l, and total bilirubin of 4.46mg/dl. CT scan showed portal vein stenosis and extensive acute intrahepatic portal vein thrombosis. We then performed laparotomy and radiological intervention using the transileocolic vein approach. Transileocolic vein portography demonstrated severe portal vein stenosis and the absence of intrahepatic portal venous blood flow. The stenotic segment of the portal vein was dilated with a balloon and a self-expanding metallic stent subsequently placed. Immediately afterward, marked improvement of the hepatopetal flow and restoration of intrahepatic portal venous blood flow was observed. Subsequently, the serum levels of AST and ALT decreased, and liver failure gradually resolved. At six days after stent placement, CT scan showed resolution of portal vein stenosis, with only small residual clots in the umbilical portion of the portal vein. The patient was discharged without any symptoms 48 days after stent placement.

The patient is an 86 year old woman who was diagnosed with carcinoma of the pancreatic head. She was followed without aggressive therapy, because of her advanced age. She had obstructive jaundice and a plastic stent (PS) was placed at the time of diagnosis, which was then exchanged to a self-expandable metallic stent (SEMS) two months later for occlusion. Eighteen days after SEMS placement, she presented with dizziness and melena. We performed upper and lower digestive endoscopy, but no obvious cause of bleeding was found. Thirty-seven days after SEMS placement, she presented in shock with recurrent melena. Enhanced computed tomography scan showed active bleeding near the duodenal papilla. A pseudoaneurysm of the posterior superior pancreatic duodenal artery (PSPDA) was found on emergency angiography, with extravasation into the duodenum and treated by transcatheter arterial embolization (TAE) using n-butyl-2-cianoacrylate. There was no recurrent bleeding for six months when she died of her primary disease. We report a rare patient with biliary bleeding from a pseudoaneurysm of the PSPDA after SEMS placement.

Tumors with two malignant components, including both adenocarcinoma and neuroendocrine components, with each accounting for at least 30% of the tumor, are defined as mixed adeno-neuroendocrine carcinomas (MANEC). Pancreatic MANECs are rare. We reviewed four patients with pancreatic MANECs, who underwent surgery at the National Cancer Center Hospital East. One patient had a tumor with neuroendocrine (NET) and adenocarcinoma components. Tumors in three other patients included neuroendocrine carcinoma (NEC) and adenocarcinoma. One of the three patients with NEC died three months after surgery due to multiple liver metastases. Another patient developed liver metastases one month after resection. Biopsy of the liver metastasis showed that the tumor consisted of the NEC component. The patient was treated with chemotherapy for NEC, and died 29 months after surgery. The third patient has survived 12 months after surgery without recurrence. The patient with an NET component survived 78 months after surgery without recurrence. The clinical course of patients with pancreatic MANEC varies depending on the histopathological classification. It is also important to note that we should select the treatment based on the histopathological classification. The histologic diagnosis of recurrent tumors is of critical importance.

A 74-year-old woman underwent a distal pancreatectomy for stage IIA cancer of the pancreatic body (JPS 7th) in February 2013. She received S-1 as adjuvant chemotherapy for 6 months. In February 2015, computed tomography scan showed a left inguinal mass, and FDG-PET imaging showed abnormal uptake in the left inguinal mass. Metastasis of pancreatic cancer to the left inguinal region was suspected and no other metastases were detected. In March 2015, the left inguinal mass was resected. An inguinal hernia was not detected during the operation. Histological examination of the resected mass revealed well-differentiated adenocarcinoma compatible with a metastasis from the pancreatic cancer. The patient survived for 54 months after distal pancreatectomy and 29 months after resection of the metastasis until August 2017, when she died of pneumonia. Metastasis of pancreatic cancer to the inguinal canal in women is very rare, especially in the absence of an inguinal hernia.

A 47-year-old Japanese female underwent mastectomy for right breast cancer 2 years earlier and started tamoxifen 2 months later. Twenty-seven months after starting tamoxifen, she presented with left abdominal pain. She was diagnosed with acute pancreatitis, with pancreas swelling and fat necrosis in the left anterior pararenal space seen on CT scan. Treatment included nothing by mouth, intravenous fluids and nafamostat mesilate infusion, 20mg/day. She recovered and was discharged on the 13th hospital day. Serum triglyceride levels during the hospitalization were remarkably high, as high as 5512mg/dl, and we suspected that hypertriglyceridemia secondary to tamoxifen was the cause of the acute pancreatitis. The tamoxifen was withdrawn immediately, and we followed the patient's serum triglyceride levels after stopping tamoxifen and after discharge. At 4 months post-discharge, her triglyceride level had decreased to normal. Tamoxifen-induced hypertriglyceridemia is rare, but may occasionally cause severe pancreatitis. The serum triglyceride level of patients taking tamoxifen should be closely monitored, and when hypertriglyceridemia is detected, it is necessary to be aware of the risk of pancreatitis.

A 69-year-old man presented with back pain and weight loss. CT scan showed a 7 cm mass in the tail of the pancreas invading the spleen and transverse colon. Based on pathological examination, he was diagnosed with adenosquamous carcinoma of the pancreas and treated with FOLFIRINOX chemotherapy due to liver metastases. During the fourth course of chemotherapy, complications occurred necessitating cessation of chemotherapy including intratumor pneumatosis at the site of colonic invasion, abscess in the spleen and left subphrenic space, hemorrhage into the colonic and gastric invasion. After developing hypercalcemia due to parathyroid hormone-related peptide (PTHrP) production and leukocytosis due to granulocyte-colony stimulating factor (G-CSF) production, he died 4 months after starting chemotherapy. Autopsy showed squamous cell carcinoma of the pancreas with advanced invasion into surrounding organs, multiple liver metastases and peritoneal dissemination.