[in Japanese]

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Autoimmune pancreatitis (AIP) is worldwide accepted as distinct pancreatitis with steroid response. The Japan Pancreas Society (JPS) first proposed the diagnostic criteria of AIP in 2002 (Suizo 2002; 17: 587) and the revised one in 2006 (J Gastroenterology 2006; 40: 626-31). The most important issue in diagnosing AIP is how to distinguish it from pancreatic or biliary cancer. The Japanese clinical criteria have been proposed for the practical use and the minimum consensus features of AIP in order to avoid the misdiagnosis of malignancy as far as possible. Internationally, two subtypes of AIP have been proposed in the International Consensus of Diagnostic Criteria (ICDC) for AIP in 2011 (Pancreas 2011; 40: 352-358): type 1 related with IgG4 (lymphoplasmacytic sclerosing pancreatitis: LPSP), and type 2 with granulocytic epithelial lesions (idiopathic duct-centric pancreatitis: IDCP). As the ICDC are still complicated for the practical use, The Research Committee of Intractable Diseases of the Pancreas supported by the Japanese Ministry of Health, Labor and Welfare and the JPS have proposed the revised diagnostic criteria in 2011. Since type 2 is extremely rare in Japan, the diagnostic criteria described here are intended to cover type 1, commonly seen in Japan, with type 2 noted only as reference.

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Chemotherapy is the standard therapy for pancreatic adenocarcinoma with distant metastases. We sometimes experience localized recurrence in the liver after curative resection of pancreatic carcinoma; therefore, there is hesitation regarding surgical resection. We analyzed 7 patients who underwent hepatic resection for liver metastases of pancreatic carcinoma at the National Cancer Center Hospital East. Of these, 3 patients underwent synchronous hepatic resection, while the others underwent metachronous hepatic resection. Liver metastases were solitary in 6 patients and showed 2 metastatic lesions in 1 patient. Median recurrence-free survival after synchronous and metachronous hepatic resection was 1.1 and 9.4 months, respectively. Overall survival after synchronous and metachronous hepatic resection was 7.5 and 20.7 months, respectively. Synchronous hepatic resection should not be performed in pancreatic cancer patients with liver metastases. However, whether metachronous hepatic resection should be performed in such patients is unclear. Currently, chemotherapeutic treatment for patients with liver metastases has become difficult to continue because side effects. Moreover, hesitation regarding surgical resection is high. It is therefore important to collect more data on such cases in order to better address these issues.

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A 56-year-old woman was admitted with a sudden onset of left abdominal pain. Laboratory data showed inflammatory reactions and a marked increase in serum concentrations of CEA (240ng/ml) and CA19-9 (200,867U/ml), which showed enormous variation thereafter. A computed tomography scan revealed a cystic lesion, 13cm in diameter, in the pancreatic tail, a 7cm-sized cystic lesion in the retroperitoneal space, and pleural effusion on the left side. Although the retroperitoneal cystic lesion thought to be a pseudocyst gradually shrank, the cyst in the pancreatic tail showed no change in size. We performed distal pancreatectomy under the preoperative diagnosis of mucinous cystic neoplasm. The resected specimen showed a 15cm-sized multilocular cyst with a smooth inner surface and a fibrous capsule. The cyst contains brownish-red mucin and haemorrhagic-necrotic material. Histological findings revealed the large cystic lesion was lined by columnar epithelium showing severe dysplasia with multifocal stromal invasion, and ovarian-type stroma positive for estrogen and progesterone receptors was evident. Thus, the final diagnosis was mucinous cystadenocarcinoma. Mucinous cystadenocarcinoma may be presented with acute pancreatitis and marked variation of serum tumor markers.

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A 76-year-old woman underwent spinal surgery, for a compression fracture of thoracic vertebra #12, in the prone position. The surgery was prolonged for 6 hours 20 minutes with massive bleeding. After the spinal surgery, she developed abdominal pain due to acute pancreatitis. The next day, enhanced CT showed grade 2 pancreatitis. She was diagnosed as a severe acute pancreatitis and treatment for pancreatitis was started. However, on the 16^th day post-surgery, enhanced CT revealed infected pancreatic necrosis with duodenal necrosis and perforation. A pancreaticoduodenectomy (PD) was performed and reconstruction was carried out according to Child's method. The pancreas head and the duodenum were widely necrosed and the duodenum was perforated with a retroperitoneal abscess formation. After PD, she recovered without major complications.

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An 80-year-old woman with advanced pancreatic cancer underwent chemotherapy and metallic stent placement 304 days after her first visit. The patient admitted 441 days after first visit and an endoscopy and duodenography revealed duodenal stenosis due to infiltration. Surgical procedures were rejected, therefore an endoscopic duodenal stent was placed on the 457th day after her first visit. Because of early stent obstruction, a second stent was inserted. The patient was able to take soft solids soon; however, cholangitis developed and a PTBD had to be placed on day 485 and was discharged. The patient was readmitted on day 519 and third stent was placed. There were no complications and liquids were ingested until her death, 553 days after the first visit. Placement of multiple duodenal self-expandable metallic stents was a useful technique, and contributed the patients' quality of life.

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A 74-year-old man underwent follow-up for early colon cancer, and a pancreatic tumor was detected during this examination. Abdominal enhanced computed tomography (CT) showed a poorly enhanced tumor (diameter, 5cm) in the pancreatic tail and multiple liver tumors. The tumor in the pancreatic tail was diagnosed as pancreatic cancer by endoscopic ultrasound-guided biopsy. A subcutaneous mass was also simultaneously detected in the left axilla, where the patient experienced pain. The mass was excised and the results of excisional biopsy led to a diagnosis of skin metastasis from pancreatic cancer. The patient underwent combination chemotherapy with gemcitabine and S-1. One month after the first cycle of chemotherapy, he experienced pain in the left lateral femoral region; magnetic resonance imaging (MRI) and CT findings were highly suggestive of muscle metastasis. The patient died from progression of pancreatic cancer 200 days after the first visit. Cases of pancreatic cancer with skin or muscle metastasis are rare. These metastases sometimes aid in detecting advanced pancreatic cancer, and therefore, careful attention should be paid to symptoms such as subcutaneous nodules and continuous muscle pain in cases where the reason for these symptoms is unclear.

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We report a case of long-term survivor of stage IVb pancreatic cancer with peritoneal dissemination. A 70-year-old man with pancreatic cancer (TS2, PV (+), T4N0M0, stage IVa) underwent pancreaticoduodenectomy after neoadjuvant chemoradiotherapy (40Gy radiation+5-FU, MMC, and CDDP). Pathological examination of the resected specimen revealed a T4N0M1 (PER; omentum), Stage IVb tumor. In addition, neoadjuvant therapy led to further dissemination of the omental nodule and primary tumor. The patient underwent postsurgical chemotherapy with gemcitabine because of an increase in serum CEA levels. Follow-up CT performed 62 months after primary resection detected a mass in his right lung, which was treated by right lower lobectomy. Immunohistochemical study of the lung mass revealed it to be a metastatic lesion of the primary pancreatic tumor. Seventeen months after lung surgery, the patient died because of pleural dissemination of the pancreatic cancer.

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The patient was a 23-year-old woman. Imaging revealed a 40-mm tumor in the pancreatic body and multiple diffused liver tumors. She was given a diagnosis of non-functioning pancreatic neuroendocrine carcinoma based on the liver tumor biopsy. Systemic chemotherapy was ineffective. After obtaining written informed consent, transplantation surgeons performed distal pancreatectomy with splenectomy and simultaneous living-donor liver transplantation. Approximately 6 months after transplantation, abdominal lymph node re-operation was performed. Because SSTR2 immunostaining of the preparation was positive, octreotide therapy was started. Her symptoms showed amelioration. She continued visiting the hospital after surgery, but metastatic lesions such as abdominal lymph node, bone and lung metastases gradually enlarged. In addition to the octreotide, S-1 therapy was started. She died 4 years and 4 months after the initial therapy. Liver transplantation could be a therapeutic option for a young patient suffering from pancreatic endocrine carcinoma with unresectable multiple liver metastases.

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A 76 year old man who was referred to our hospital presented with jaundice and a pancreatic head tumor. Enhanced CT showed a solid mass 26mm in diameter at the pancreatic head and also detected a hepatic tumor 8mm in diameter at segment 8. In addition, MRI showed a cystic tumor adjacent to the pancreatic head tumor. FDG-PET showed strong FDG uptake in areas corresponding to the pancreatic head and hepatic tumors in which SUVmax values were 15.6 and 7.56, respectively. Clinical diagnosis was pancreatic adenocarcinoma and hepatic metastasis. A pylorus-preserving pancreaticoduodenectomy and partial hepatectomy were performed according to the patient's and family's wishes, and due to the unusual isolated hepatic metastasis. Histopathologic evaluation showed that a component of a squamous cell carcinoma intermingled with that of a ductal adenocarcinoma in the pancreatic and hepatic tumors. In addition, proliferation of papillary columnar epithelium was detected at the branch of the pancreatic duct corresponding to an intraductal papillary mucinous neoplasm (IPMN), The IPMN did not have continuity to the ductal adenocarcinoma or squamous cell carcinoma. Therefore, the final histopathologic diagnosis was a coexistence of pancreatic adenosquamous carcinoma and IPMN.

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