Secondary Logo

Journal Logo


Proceedings of the Japan Pancreas Society, 2019, Suizo, the Journal of the Japan Pancreas Society, Volume 34, 2019, Naohiro Sata, MD, PhD, Editor-in-Chief

Author Information
doi: 10.1097/MPA.0000000000001581
  • Free


Clinical Features of Healthy Subjects Undergoing Evaluation to Diagnose Early Pancreatic Cancer

Masayuki Furukawa, Rie Sugimoto, Terumasa Hisano, Risa Hashimoto, Sho Yasumori, Tatsuya Noguchi. Department of Hepato-biliary-pancreatology, National Kyushu Cancer Center, Fukuoka, Japan. Suizo. Vol 34, No 5, pp 206-213.

A medical evaluation including ultrasonography, contrast CT scan, MRCP, and blood tests was conducted on healthy people without health insurance since August 2016 to make the early diagnosis of pancreatic cancer. By December 2018, 92 people had been examined. Abnormalities in test results led to follow-up examinations such as EUS, medical treatment, and observation paid by national health insurance. It was striking that no less than 37% of people evaluated indicated a family history of pancreatic cancer as their motive. In comparison, the number of consultations tended to be lower for those with a history of smoking, diabetes, and pancreatitis, which are considered risk factors for developing pancreatic cancer.1 The results reflect the lack of public awareness of the relationship of smoking and diabetes with pancreatic cancer.2 Through these examinations, we found that 66% of healthy individuals had at least one biliary or pancreatic abnormality (Table 1). Three patients with malignant tumors (pancreatic neuroendocrine tumor, gallbladder cancer, and recurrent renal cell carcinoma) were identified with this medical evaluation but we found no patients with pancreatic cancer. There were two patients from two families in which at least one family member had been diagnosed with pancreatic cancer, diagnosed with chronic pancreatitis and pancreatic divisum. MRCP was generally superior for the detection of pancreatic lesions, but the detection rate of other modalities was higher depending on the disease. Despite the small number of cases, the absence of any patients with pancreatic cancer might indicate that a high-risk group could not be identified. We hope that this study will be useful for developing a surveillance plan people at high risk of developing pancreatic cancer.

Biliary-pancreatic Diseases and Abnormalities Found at Medical Evaluation (n = 92)


1. Anderson K, Potter JD, Mack TM. Pancreatic cancer. In: Schottenfield D, Fraumeni J Jr, eds. Cancer epidemiology and prevention. New York, NY: Oxford University Press; 2006:721-762.

2. Matsubayashi H, Maeda A, Kanemoto H, et al. Risk factors of familial pancreatic cancer in Japan: current smoking and recent onset of diabetes. Pancreas. 2011;40:974-978.


Short- and Long-Term Outcomes After Pancreatectomy for Pancreatic Ductal Adenocarcinoma in Octogenarians

Kensuke Nitta,* Yusuke Watanabe,* Sho Okuda,* Sho Endo,* Shingo Kozono,* Keijiro Ueda,† Yusuke Mizuuchi,* Nobuhiro Suehara,* Yuji Abe,* Kazuyoshi Nishihar,* Toru Nakano.* Departments of *Surgery and †Gastroenterology, Kitakyushu Municipal Medical Center, Fukuoka, Japan. Suizo. Vol 34, No 5, pp 195-205.

The purpose of this study was to investigate the short- and long-term outcomes after pancreatectomy for pancreatic ductal adenocarcinoma (PDAC) in octogenarians. The medical records of 141 patients who underwent pancreatectomy for PDAC were retrospectively reviewed. The patients were divided into two groups, non-octogenarian (n = 124) and octogenarian patients (n = 17). The prevalence of comorbidities was significantly higher in octogenarian than non-octogenarian patients, and the performance status was significantly worse in octogenarian than non-octogenarian patients. However, 82% of octogenarian patients had a performance status of ≤1. There was no difference in the American Society of Anesthesiologists physical status (ASA-PS) between the 2 groups. The postoperative clinical courses were not different between the two groups. There were no significant differences in recurrence-free survival (RFS) or overall survival (OS) between the 2 groups in univariate analysis (Fig. 1). Age as an independent factor was not significantly associated with RFS or OS in multivariate analysis. Pancreatectomy for PDAC can be performed safely even in octogenarian patients based on the assessment of general condition using the ASA-PS or performance status. The benefit of pancreatectomy for PDAC was the same for octogenarian and non-octogenarian patients.

Survival curves of octogenarian patients (n = 17) and non-octogenarian patients (n = 124). A, Recurrence-free survival (P = 0.53). B, Overall survival (P = 0.91).


Long-Term Survival Following Multidisciplinary Therapy for Liver Metastases in a Patient With Pancreatic Cancer

Tadatoshi Kakimoto,* Osamu Miura,* Koji Matsuoka,* Sachiko Takeo,* Amane Kamouchi,* Yukinori Okazaki,† Junko Fujiwara.† Departments of *Digestive Surgery and †Gastroenterology, Hofu Institute of Gastroenterology, Yamaguchi, Japan. Suizo. Vol 34, No 4, pp 172-180.

A 64-year-old man noted jaundice, and was found to have pancreatic cancer, treated with radical resection. However, 4 months after resection, his serum carcinoembryonic antigen level was elevated and a low-density area suggesting a metastasis was detected in the liver (S7). The patient was treated with FOLFIRINOX chemotherapy and GEM+nab-PTX combination chemotherapy. After chemotherapy, the liver (S7) metastasis was smaller, but 14 months after tumor resection, a new recurrence was detected in the liver (S6) (Fig. 1). Treating the new liver metastasis only with chemotherapy was considered difficult, so proton radiation therapy (64 Gy) was given. After radiation therapy, administration of GEM+nab-PTX chemotherapy was continued for 2 years, and a complete response (CR) was achieved. At 62 months after resection, the patient is alive and without new recurrent disease.

The second recurrent lesion in the liver (S6) 14 months after resection seen on a contrast enhanced computed tomography scan (A), contrast enhanced T1WI magnetic resonance imaging study with fat suppression (B), and FDG-positron emission tomography (C). Proton radiation therapy (64 Gy) was given (D).


1. De Jong MC, Farnell MB, Sclabas G, et al. Liver-directed therapy for hepatic metastases in patients undergoing pancreatico-duodenectomy: a dual-center analysis. Ann Surg. 2010;252:142-148.

2. Singh A, Singh T, Chaudhary A. Synchronous resection of solitary liver metastases with pancreaticoduodenectomy. JOP. 2010;11:434-438.

3. Fujisaki S, Takashina M, Tomita R, et al. Long-term survival following hepatectomy, radiation, and chemotherapy for recurrent pancreatic carcinoma: a case report. World J Surg Oncol. 2017;15:157.


A Pancreatic Cyst Associated With IgG4-related Autoimmune Pancreatitis

Makiko Ozawa,* Takayuki Watanabe,* Norihiro Ashihara,* Yasuhiro Kuraishi,* Akira Nakamura,* Keita Kanai,* Yuki Matsumoto,† Mikiko Kobayashi,† Eiji Tanaka.* *Department of Gastroentelorogy, Shinshu University School of Medicine, Nagano; †Department of Diagnostic Pathology, Shinshu University School of Medicine, Nagano, Japan. Suizo. Vol 34, No 5, pp 239-246.

A 68-year-old woman was diagnosed with IgG4-related dacryoadenitis and sialadenitis in 2009. Administration of prednisolone in 2011 improved her symptoms and was later discontinued. Serum IgG4 levels were elevated in July 2017. CT scan revealed a round cyst surrounded by delayed perfusion in the tail of the pancreas (Fig. 1). T1-weighted images on MRI scan showed the inside of the cyst had low intensity and the wall of the cyst had high intensity. On T2-weighted images, however, the inside of the cyst displayed high intensity and the wall of the cyst had low intensity. There were no apparent abnormalities in the main pancreatic duct. EUS disclosed a low echoic layer between the cyst and pancreatic parenchyma. ERP showed no communication between the pancreatic cyst and the main pancreatic duct. Laparoscopic distal pancreatectomy was performed with a preoperative diagnosis of mucinous cystic neoplasm or pancreatic neuroendocrine neoplasm. Histopathological examination revealed that the cyst was surrounded by fibrosis and infiltration of chronic inflammatory cells, most of which were IgG4-positive plasma cells. Based on these findings, a revised diagnosis of focal autoimmune pancreatitis with storiform fibrosis and obliterative phlebitis around the cyst was made. Pancreatic cystic lesions have been reported in patients with autoimmune pancreatitis.1,2 Some cysts with IgG4-related autoimmune pancreatitis are refractory to corticosteroid treatment.1-3 In patients with IgG4-related disease who have atypical cystic pancreatic neoplasms, corticosteroid treatment should be considered because of the possibility of focal autoimmune pancreatitis.

Abdominal contrast enhanced computed tomography (CT) scan. CT scan revealed a round cyst surrounded by delayed perfusion in the tail of the pancreas.


1. Kubota K, Fujita Y, Sato T, et al. Autoimmune pancreatitis associated with pancreatic cyst: how can we manage it? J Hepatobiliary Pancreat Sci. 2014;21:902-910.

2. Matsubayashi H, Iwai T, Matsui T, et al. Pancreatic cystic lesions with atypical steroid response should be carefully managed in cases of autoimmune pancreatitis. J Gastroenterol Hepatol. 2016;31:270-276.

3. Donet JA, Barkin JA, Keihanian T, et al. Pancreatic pseudocysts and parenchymal necrosis in patients with autoimmune pancreatitis: a systematic review. Pancreas. 2018;47:952-957.


Severe Disseminated Intravascular Coagulation and Tumor Lysis Syndrome in a Patient With Pancreatic Cancer Treated With Gemcitabine and Nab-paclitaxel: A Case Report

Daisuke Muroya,* Shinichi Taniwaki,† Satoki Kojima,† Syoichirou Arai,† Yohei Nakama,† Yuhei Kitazato,† Toru Hisaka,† Hiroto Ishikawa,* Masafumi Yasunaga,† Masayuki Okabe.* *Department of Surgery, Munakatasuikoukai General Hospital, Fukuoka; Department of Surgery, Kurume University Hospital, Fukuoka, Japan. Suizo. Vol 34, No 5, pp 232-238.

Tumor lysis syndrome (TLS) and disseminated intravascular coagulation (DIC) are oncologic emergencies resulting from treatment with cytotoxic chemotherapy. We present TLS and DIC occurring in a 74-year-old male with known pancreatic cancer metastatic to the liver (Fig. 1). Shortly after diagnosis, gemcitabine and nab-paclitaxel chemotherapy was initiated. Three days after starting chemotherapy, the patient developed weakness and dyspnea. Laboratory studies revealed LD 1450 U/L, potassium 5.4 mEq/L, uric acid 9.0 mg/dL, fibrinogen degradation products 140 μg/ml, international normalized ratio 1.93, antithrombin-III 32%, and his platelet count had dropped from 233,000/mcL to 45,000/mcL. The diagnosis of TLS and DIC were made based on clinical findings and laboratory data. Despite aggressive treatment with intravenous hydration, electrolyte monitoring, Radburicase and renal replacement therapy, he died 4 days after starting treatment. There are very few reports of TLS in patients with pancreatic cancer. This is the first report in the world of TLS and DIC due to gemcitabine and nab-paclitaxel chemotherapy in a patient with pancreatic cancer.

Abdominal contrast enhanced computed tomography scan. A low density tumor in the pancreatic tail was seen (A). Dilatation of the peripheral main pancreatic duct was seen (B). Multiple metastases are seen in the liver (C).


1. Ling W, Sachdeva P, Wong AS, et al. Unprecedented case of tumor lysis syndrome in a patient with metastatic pancreatic adenocarcinoma. Pancreas. 2012;41:659-661.

2. Kolin M, Ben-Shahar M, Lapin O, et al. Severe tumor lysis syndrome in a patient with pancreatic adenocarcinoma. Pancreas. 2016;45:e12-e13.

3. Umar J, Kalakonda A, Panebianco L, et al. Severe case of tumor lysis syndrome presenting spontaneously in a metastatic pancreatic adenocarcinoma patient. Pancreas. 2017;46:e31-e32.


A Patient With Hemobilia Due to a Pseudoaneurysm of the Pancreatic Arcade After Biliary SEMS Placement

Shuhei Shintani,* Yoshihiro Yokota,* Yuto Sato,* Hiroto Inoue,* Hiroki Tanabe,* Hiromichi Bamba,* Yasunobu Komai,* Koutarou Shimada,† Osamu Inatomi,‡ Akira Andoh.‡ *Department of Gastroenterology, Nagahama Red Cross Hospital, Shiga; Department of Radiology, Nagahama Red Cross Hospital, Shiga; Department of Gastroenterology University of Medical Science, Shiga, Japan. Suizo. Vol 33, No 5, pp 826-833.

We report a patient with hemobilia due to a pseudoaneurysm after placement of a self-expandable metallic stent (SEMS) in the biliary tract. An 86-year-old woman diagnosed with carcinoma of the head of the pancreas was followed without treatment due to her advanced age. Concomitant obstructive jaundice was noted and a plastic stent was inserted. However, 2 months later, it was replaced with a SEMS due to stent occlusion. Eighteen days after SEMS placement, she presented to the emergency unit because of dizziness and melena. Contrast-enhanced computed tomography (CE-CT) scan and upper and lower digestive endoscopy revealed no obvious cause of bleeding. Thirty-seven days after SEMS placement, CE-CT was performed for recurrent melena, and extravasation near the major duodenal papilla was observed. A pseudoaneurysm of the posterior superior pancreatic duodenal artery was identified on emergency angiography, which was associated with extravasation into the duodenum (Fig. 1). It was treated with transcatheter arterial embolization using n-butyl-2-cyanoacrylate. There was no recurrence for 6 months until death from the primary disease. Hemobilia is a relatively rare condition. A SEMS is often inserted for hemostasis due to papillary hemorrhage after procedures, such as endoscopic sphincterotomy. In this case, the pseudoaneurysm could not be identified on imaging studies. Angiography is useful in such a situation, and after performing biliary–pancreatic treatment, it is necessary to perform angiography considering the possible formation of a pseudoaneurysm.

A, B, Abdominal contrast-enhanced computed tomography scan and gastroduodenoscopy at admission. The cause of gastrointestinal bleeding was not determined. C, Extravasation (arrow) from near the major duodenal papilla into the common bile duct was observed. D, A pseudoaneurysm was confirmed on posterior superior pancreatic duodenal artery imaging (arrow) and transcatheter arterial embolization was performed using n-butyl-2-cyanoacrylate.


1. Green MH, Duell RM, Johnson CD, et al. Haemobilia. Br J Surg. 2001;22:1446-1451.

2. Nezu Y, Nakaji S, Fujii H, et al. Pseudoaneurysm caused by a self-expandable metal stent: a report of three cases. Endoscopy. 2014;46:248-251.

3. Monroe PS, Deeter WT, Rizk P. Delayed hemobilia secondary to expandable metal stent. Gastrointest Endosc. 1993;39:190-191.

Long-Term Survival of a Patient With Pancreatic Ductal Adenocarcinoma With Peritoneal Dissemination Treated With S-1 Plus Intravenous and Intraperitoneal Paclitaxel Followed by Conversion Surgery

Masaya Kotsuka, Sohei Satoi, Hiroaki Yanagimoto, Tomohisa Yamamoto, Hisashi Kosaka, Satoshi Hirooka, So Yamaki, Taku Michiura, Kentaro Inoue, Yoichi Matsui. Department of Surgery, Kansai Medical University, Osaka, Japan. Suizo. Vol 34, No 1, pp 1-8.

We report long-term survival of a patient who underwent conversion surgery after a long-term favorable response to S-1 plus intravenous and intraperitoneal paclitaxel therapy. An 81-year-old man was diagnosed with pancreatic ductal adenocarcinoma in the pancreatic tail (34 mm diameter tumor and CA 19-9; 831 U/mL) by endoscopic ultrasound fine needle aspiration cytology. Staging laparoscopy revealed peritoneal dissemination and positive peritoneal washings by cytology. After administration of S-1 combined with intravenous and intraperitoneal paclitaxel therapy, the patient’s CA 19-9 level (32.3 U/ mL) and tumor size (10 mm in a diameter) decreased, and peritoneal washing cytology was negative. Second-look staging laparoscopy revealed resolution of the peritoneal nodules. Distal pancreato-splenectomy was then performed 12 months after initial treatment. The post-operative course was uneventful and the patient discharged on post-operative day 9. Pathological examination of the resected specimen revealed negative margins, pathologic stage T1 (1.0 × 0.5 mm)/N0/M0, stage I. The patient is alive and progression-free 48 months after initial treatment and 36 months after conversion surgery.

Autoimmune Pancreatitis Associated With Pancreatic Necrosis: A Case Report

Ryo Kodama,* Masato Kamakura,* Hisanobu Saegusa,* Hiroyasu Ushimaru,* Mutsuki Makino,† Kenji Kawaguchi.† Departments of *Gastroenterology and †Pathology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan. Suizo. Vol 34, No 1, pp 9-14.

A 60-year-old male was hospitalized with appetite loss, weight loss, and jaundice. Laboratory examinations revealed aggravation of diabetes with elevated IgG and IgG4 levels of 2446 and 232 mg/dl, respectively. Magnetic resonance imaging revealed thickening and stenosis of the common bile duct, enlargement of the pancreatic head, and a capsule-like rim and diffuse narrowing of the main pancreatic duct. Autoimmune pancreatitis was suspected. A cyst-like lesion with a high-intensity area was observed in the pancreatic tail on both the T1- and the T2-weighted images. The high-intensity area was thought to indicate previous bleeding in the pancreas. Endoscopic ultrasound-guided fine needle aspiration biopsy was performed using a 22 G Franseen needle. The patient was diagnosed with autoimmune pancreatitis based on the histology of a biopsy from the pancreatic body. The specimen of the cyst-like lesion in the pancreatic tail revealed pancreatic necrosis. Treatment with steroids normalized the hepatobiliary enzymes, and one month after treatment, stenosis of the biliary duct and enlargement of the pancreas improved. At three months after treatment, the pancreatic necrosis had resolved. This is the first report of autoimmune pancreatitis associated with pancreatic necrosis.

Preoperative Diagnosis of a Pancreatic Metastasis From Rectal Cancer

Tatsuya Hayashi,* Kunio Takuma,* Taku Higashihara,* Dai Inoue,† Yasuhiro Morita.* Departments of *Surgery and †Gastroenterology, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan. Suizo. Vol 34, No 1, pp 15-21.

Metastasis of colorectal cancer to the pancreas often occurs in the terminal stages with widespread systemic metastases, and most are inoperable. Two points in the treatment of a pancreatic metastasis from colorectal cancer need to be considered. The first point is the indications for surgery. The second point is how to differentiate a pancreatic metastasis from a primary pancreatic cancer. A 72-year old woman was scheduled for a hepatectomy for liver metastases from colorectal cancer. Follow-up abdominal CT scan disclosed a tumor in the pancreatic body. Since it was difficult to differentiate a primary pancreatic cancer from a metastasis to the pancreas by CT-imaging alone, we performed liver and pancreas biopsies using endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). Liver and pancreatic metastases from colorectal cancer were diagnosed based on the histopathological examination. Radical excision was considered possible and we performed a liver resection and distal pancreatectomy. In a previous study, there were some patients with long-term survival who had pancreatic metastases from colorectal cancer, and surgery was one of the determinants of the survival. In this patient, because both liver and pancreatic lesions were detected, it was essential to establish the preoperative pathological diagnosis, which was achieved by EUS-FNA.

Difficulty in the Intraoperative Identification of a Small Non-functional Pancreatic Neuroendocrine Tumor Detected by FDG-PET: A Case Report

Yusuke Takemura,* Minoru Kitago,* Osamu Itano,† Masahiro Shinoda,* Hiroshi Yagi,* Yuta Abe,* Go Oshima,* Shutaro Hori,* Yusuke Fujita,* Yuki Masuda,* Naoto Kubota,‡ Hanako Tsujikawa,‡ Miho Kawaida,‡ Yuko Kitagawa.* *Department of Surgery, Keio University School of Medicine, Tokyo; Department of Gastrointestinal Surgery, International University of Health and Welfare, Chiba; Department of Pathology, Keio University School of Medicine, Tokyo, Japan. Suizo. Vol 34, No 1, pp 22-29.

A 74-year-old man was referred for removal of a 10 mm mass in the uncinate process of the pancreas, identified by increased fludeoxyglucose (FDG) uptake (SUVmax 4.32) on a FDG-positron emission tomography-computed tomography (PET/CT) scan, performed during preoperative workup for thyroid carcinoma. A contrast-enhanced CT scan showed a 7 mm early-enhancing mass at the same site. This was diagnosed as a pancreatic neuroendocrine tumor (PNET). A partial pancreatectomy was performed as an excisional biopsy with intraoperative ultrasonography. No tumor was found in the resected specimen, but was seen on CT during follow-up. Four months after the first resection, we performed a repeat partial pancreatectomy. Three resections were performed, but the tumor was not found in the resected specimens by frozen section examination. Therefore, we finally performed a subtotal stomach-preserving pancreaticoduodenectomy. On pathological examination, we finally located a 7 mm tumor in the specimen following detailed analysis. This tumor was immunohistochemically positive for synaptophysin, chromogranin A, and CD56. The Ki-67 index was less than 1.5%. The mass was diagnosed as a PNET (WHO classification Grade 1). We report our experience with difficulty in intraoperative identification of a small PNET.

Duodenal Perforation After the Cutting an ENPD Tube in a Patient With Pancreatic Cancer and Acute Suppurative Pancreatic Ductitis

Akihisa Ohno, Nao Fujimori, Kazuhide Matsumoto, Katsuhito Teramatsu, Yu Takamatsu, Masami Miki, Takehiro Takaoka, Takamasa Oono. Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Suizo. Vol 34, No 1, pp 30-36.

The patient is a 50-year-old male diagnosed with pancreatic cancer with liver metastasis, peritoneal dissemination, and duodenal stenosis. A metallic biliary stent was placed in the common bile duct before initiating chemotherapy. However, one month later, he developed acute pancreatitis, probably because of the metallic stent. The metallic stent was replaced with a plastic stent, along with a pancreatic stent. However, after removal of the pancreatic stent, the fever persisted. He eventually developed acute suppurative pancreatic ductitis and underwent endoscopic nasal pancreatic drainage (ENPD). Due to a stricture in the second portion of the duodenum, the external drainage tube was cut endoscopically and converted to internal drainage. The plastic biliary stent was replaced due to cholangitis, however, the patient developed septic shock. CT scan revealed free air and intraperitoneal abscess, showing the ENPD tube penetrating the duodenum. CT-guided percutaneous drainage was done. The ENPD tube was removed and the duodenal perforation was repaired by clipping. His condition improved.

Pancreatic Schwannoma Diagnosed by EUS-FNAB

Takahiro Sugai,* Makoto Abue,* Wataru Iwai,* Takehumi Miyazaki,* Yuta Wakui,* Hiroki Aizawa,* Tomoyuki Oikawa,* Kiyoshi Uchimi,* Sinichi Suzuki,* Shigemi Ito,† Ikurou Satou.† Departments of *Gastroenterology and Pathology, Miyagi Cancer Center, Miyagi, Japan. Suizo. Vol 34, No 1, pp 37-45.

A 62-year-old man was referred to our hospital for a cystic lesion in the pancreatic head detected by abdominal ultrasonography during a routine medical examination. This pancreatic mass was suspected to be a solid tumor because of gradual enhancement on contrast-enhanced CT and MRI scans, but we could not establish the diagnosis using only imaging findings. Therefore, EUS-FNAB was performed. Histopathological examination revealed a large number of spindle cells arranged in fascicles. Immunohistochemically, the spindle cells were positive for S-100 protein and negative for c-kit, CD34,α-SMA. The mass was diagnosed as a pancreatic schwannoma. The patient is followed without administering treatment, because the Mib-1 index was low (1-2%), and there were no other malignant signs.

Pancreatic Acinar Cell Carcinoma Diagnosed by BCL10 Staining: A Case Report

Kei Yamane, Takayuki Anazawa, Toshihiko Masui, Kazuyuki Nagai, Seiichiro Tada, Kenta Inoguchi, Kenzo Nakano, Yuichiro Uchida, Akitada Yogo, Kyouichi Takaori, Shinji Uemoto. Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan. Suizo. Vol 32, No 1, pp44-54.

Pancreatic acinar cell carcinoma (ACC) is a rare tumor accounting for approximately1% of all primary pancreatic neoplasms. A rare tumor is challenging to diagnose using the small specimen obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Here we report a patient with ACC, diagnosed by preoperative EUS-FNA using a recently established immunostaining procedure. A 35-year-old woman presented with an abdominal mass located in the pancreatic head. CT and MRI scans revealed the absence of central necrosis or a cystic region in the tumor. EUS-FNA revealed oval cells with a high N/C ratio, which were negative for neuroendocrine markers and diffusely positive for BCL10 based on immunostaining. BCL10 is specifically expressed in ACCs. Therefore, the lesion was diagnosed as ACC, and a pylorus-preserving pancreaticoduodenectomy was performed. Histopathological examination revealed that the tumor was ACC. Due to its histologic and radiologic similarities to solid pseudopapillary neoplasm and pancreatic neuroendocrine tumors, ACC is difficult to diagnose preoperatively. BCL10 is specifically and sensitively expressed in ACCs even if the specimen is small, similar to that obtained by EUS-FNA. We believe that BCL10 immunostaining should be performed in patients suspected to have ACC.

Cancer of the Pancreatic Body Associated With a Pancreatic Pleural Effusion: A Case Report

Takashi Miyamoto,* Katsuhisa Ohgi,* Teiichi Sugiura,* Yukiyasu Okamura,* Takaaki Ito,* Yusuke Yamamoto,* Ryo Ashida,* Keiko Sasaki,† Katsuhiko Uesaka.* Divisions of *Hepato-Biliary-Pancreatic Surgery and Diagnostic Pathology, Shizuoka Cancer Center, Shizuoka, Japan. Suizo. Vol 34, No 2, pp 63-70.

A 62-year-old man was referred for further evaluation of a dilated main pancreatic duct with cystic lesions in the pancreatic tail detected on abdominal ultrasonography. He had complained of dyspnea and chest back pain. Chest X-ray showed a massive left pleural effusion. Computed tomography revealed a low-density mass in the pancreatic body with a dilated main pancreatic duct and pseudocysts in the tail of the pancreas. Thoracentesis yielded red-brown pleural fluid with a high amylase level (26,775 U/l). Cytologic examination of the pleural fluid was not consistent with malignancy. Endoscopic retrograde pancreatography (ERP) revealed an occluded main pancreatic duct, and biopsy from the ERP was suspicious for adenocarcinoma. The preoperative diagnosis was pancreatic body cancer with a pancreatic pleural effusion. Thoracic drainage, endoscopic naso-pancreatic drainage, and administration of antibiotics were performed. Twenty days later, pancreaticooduodenectomy was performed with curative intent. The postoperative course was uneventful, and the patient was discharged on postoperative day 18. Although the most common underlying cause of a pancreatic pleural effusion is chronic pancreatitis, this is a rare case of pancreatic cancer presenting as a pancreatic pleural effusion.

Anaplastic Carcinoma With Osteoclast-like Giant Cells: A Case Report

Hiroto Fujisaki,* Norihiro Kishida,* Kaou Matsuda,* Yasuhiro Ito,* Takayuki Takahashi,* Kazuhiko Shimizu,† Junichi Matsui.‡ Departments of *Surgery and Pathology, Japanese Red Cross Ashikaga Hospital, Tochigi; Department of Surgery, Tokyo Dental College Ichikawa General Hospital, Chiba, Japan. Suizo. Vol 34, No 2, pp 114-121.

A 67-year-old man was admitted with abdominal distension and left-sided abdominal pain. Abdominal computed tomography scan revealed a 20 cm tumor of the pancreatic body and tail with both cystic and solid components. The left abdominal cavity was occupied by the tumor, and the tumor was in contact with multiple organs including the stomach, transverse colon, and left kidney. In addition, it was close to the superior mesenteric artery and abdominal aorta. Based on the diagnosis, radical resection of the malignant pancreatic tumor was planned because there were no distant metastases. The tumor appeared mobile and was easily separated from the retroperitoneum and we performed a distal pancreatectomy and left hemicolectomy. Pathological examination revealed proliferation of undifferentiated malignant cells and CD68-positive osteoclast-like multinucleated giant cells. The diagnosis was anaplastic carcinoma with osteoclast-like giant cells. The patient was treated with postoperative adjuvant chemotherapy with gemcitabine and S-1, but multiple lung metastases appeared 6 months after surgery. We changed the chemotherapy regimen to gemcitabine and nab-paclitaxel, but it was not effective and the patient died 13 months after resection.

A Patient With an Asymptomatic Paraganglioma Difficult to Differentiate From a Non-functioning Pancreatic Neuroendocrine Neoplasm

Masanari Sekine, Hirosato Mashima. Department of Gastroenterology, Jichi Medical University Saitama Medical Center, Saitama, Japan. Suizo. Vol 34, No 4, pp 181-187.

A 38 year-old female was evaluated for a pancreatic mass found on abdominal ultrasonographic screening. CT scan revealed an early enhancing tumor in the pancreatic head. MRI revealed that the tumor showed a low signal in the T1 weighted image, a high signal in the T2 weighted image, and a diffusion reduced signal in the diffusion weighted image. EUS showed a 14 mm low echoic tumor with a smooth margin and well-defined border near the portal vein. The tumor was strongly enhancing in the early phase of contrast EUS, and EUS-FNA was performed. Cytologic analysis was consistent with a neuroendocrine tumor and a pancreaticoduodenectomy was performed. Hematoxylin and eosin staining showed a Zellballen pattern and immunohistochemical studies were positive for S100 in cells between the tumor clusters, establishing paraganglioma as the final diagnosis. Peripancreatic paraganglioma is rare and it is very difficult to distinguish a paraganglioma from a pancreatic neuroendocrine neoplasm based on imaging studies alone. In an invasive procedure such as EUS-FNA, the blood pressure might be greatly elevated in a patient with a paraganglioma. When EUS-FNA is performed for a tumor rich in blood flow, the differential diagnosis including paraganglioma is very important and we must be ready for acute elevation of blood pressure.

Melanoma Metastasis to the Pancreas With Interesting MRI Findings: A Case Report

Takeshi Yasuda,* Hiroaki Yasuda,† Hiroki Eguchi,* Yoshiya Takeda,* Kunihiro Fushiki,* Yuriko Onozawa,* Masanobu Katayama,* Motoo Tanaka,* Masamichi Bamba,‡ Tadashi Shigematsu.* *Department of Gastroenterology, Saiseikai Shiga Hospital, Shiga; Department of Gastroenterology & Hepatology, Kyoto Prefectural University of Medicine, Kyoto; Department of Daiagnostic Pathology, Saiseikai Shiga Hospital, Shiga, Japan. Suizo. Vol 34, No 4, pp 188-194.

A woman in her 60s was admitted with jaundice. She had a history of resection of a melanotic melanoma in the right paranasal sinus about six months previously. On admission, abdominal CT scan revealed lesions up to 25 mm in the head and body of the pancreas. Those lesions were hypointense on MRI T1-weighted images. This finding was not typical of melanotic melanoma. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) was performed, and a diagnosis of pancreatic metastasis from melanoma confirmed by immunostaining. In this patient, the primary lesion was melanotic but the biopsy specimen from the metastatic lesion contained few melanin granules, which may explain why the pancreatic lesions had such MRI findings. The specimen was evaluated for genetic mutations using the primary lesion in order to select the best therapeutic agent, because there was insufficient tissue from the pancreatic metastasis. The selected drug had little effect, which suggests that the metastatic lesions had a different genetic character than the primary lesion.

A Patient With a Paraganglioma Mimicking a Lymph Node Metastasis From a Pancreatic Neuroendocrine Tumor

Yuko Kobashi,* Junichi Matsui,† Yutaka Takigawa,† Sakiko Takarabe,‡ Hiroyuki Yakabe,* Aya Sasaki.§ Departments of *Radiology, Surgery, Gastroenterology, and §Pathology, Tokyo Dental College Ichikawa General Hospital, Tokyo, Japan. Suizo. Vol 34, No 5, pp 214-231.

A 74-year-old female presented for evaluation of a mass around the gallbladder. Plain abdominal CT scan revealed a round tumor anterior to the pancreatic head. The tumor showed strong enhancement in the arterial phase and washed-out on the delayed phase on abdominal dynamic CT scan. From the enhanced pattern, we suspected this to be a lymph node metastasis from a pancreaticoduodenal NET. EUS-FNA was performed and the cytological diagnosis was NET. Somatostatin receptor scintigraphy showed increased radioactive tracer suggestive of NET. Serum pancreatic hormone levels such as insulin, gastrin and glucagon were normal. All studies supported the diagnosis of a lymph node metastasis from a pancreaticoduodenal NET although no pancreaticoduodenal NET was detected. The tumor was resected and histological diagnosis showed paraganglioma. Paraganglioma usually arises from the extra-adrenal paraganglionic system. Tumor location and various imaging studies complicated establishing the diagnosis. We report a rare case of paraganglioma anterior to the pancreatic head.

Adenosquamous Carcinoma of the Pancreas With Central Cystic Degeneration Covered With a Thick Pseudo-capsule and Followed by Patient’s Survival for 17 Years After Surgery: A Case Report

Taketoshi Fujimoto,* Yo Kato,† Kunihiko Obara,‡ Masahiro Irimoto.§ *Department of Surgery, Hiratsuka Gastroenterological Hospital, Tokyo (current affiliation: IMS Fujimi General Hospital, Saitama; Department of Pathology, Nikko Medical Center, Dokkyo Medical University, Tochigi; Department of Surgery, Hiratsuka Gastroenterological Hospital, Tokyo (current affiliation: Obara Clinic, Tokyo); §Department of Radiology, Irimoto Medical, Tokyo, Japan. Suizo. Vol 34, No 5, pp 222-231.

Adenosquamous carcinoma (ASC) of the pancreas is believed to be associated with a poor prognosis. We report a patient with ASC who has survived for 17 years after resection without adjuvant chemotherapy. A 60-year-old man presented with left lower abdominal pain. Laboratory data showed an elevation of carbohydrate antigen 19-9 (232 U/ml), and s-pancreas-1 antigen (96 U/ml) without elevation of squamous cell carcinoma-related antigen. Ultrasound revealed a 4×3×3 cm ellipsoid solid tumor with central cystic degeneration in the pancreatic tail. Dynamic CT scan showed a mildly enhanced solid portion of the tumor in the portal and delayed phases. MRI/MRCP showed a 2 cm cystic element with a mural nodule and a dilated distal main pancreatic duct. Distal pancreatectomy (body-tail) with regional lymph node dissection was performed. Pathology showed ASC of the pancreas (T2N0M0, Stage IB). The tumor was covered with a thick pseudo-capsule and composed mostly of squamous cell carcinoma, accompanied by a cavity derived from central necrosis. Inside and adjacent to the tumor was an adenocarcinoma component, mostly in the form of carcinoma-in-situ. He has been well without recurrence for 17 years since surgery.

A Patient With Severe Acute Pancreatitis Associated With Rhabdomyolysis and Review of Cases Reported in Japan

Takehiro Takaoka,*† Tetsuro Akashi,* Yu Takamatsu,*† Masaru Sasaki,* Ritsuya Noda,* Isao Ichino,* Naotaka Sekiguchi,* Toshiaki Ochiai,* Nao Fujimori,† Takamasa Oono,† Yoshihiro Ogawa.† *Department of Internal Medicine, Saiseikai Fukuoka General Hospital, Fukuoka; Department of Medicine and Bioregulatory Science, Graduate School of Medical Science, Kyushu University, Fukuoka, Japan. Suizo. Vol 34, No 5, pp 247-253.

A 42-year-old man was admitted with abdominal pain and leg pain. He had been diagnosed with severe acute pancreatitis. He also developed rhabdomyolysis and acute renal failure because of elevated serum CK and myoglobin. After multidisciplinary treatment including volume repletion and renal replacement therapy, the pancreatitis and rhabdomyolysis improved. Since severe acute pancreatitis associated with rhabdomyolysis is associated with a poor prognosis, we propose urgent and aggressive treatment.

Branch-Duct IPMN With an Atypical Appearance on Imaging

Tomohiro Tanikawa,* Noriyo Urata,* Jun Nakamura,* Takako Sasai,* Miwa Kawanaka,* Munenori Takaoka,† Atsushi Urakami,† Yasumasa Monobe,‡ Hirofumi Kawamoto.* *Department of General Internal Medicine 2, Kawasaki Medical School, Okayama; Department of General Surgery, Kawasaki Medical School, Okayama; Department of Pathology, Kawasaki Medical School, Okayama, Japan. Suizo. Vol 34, No 5, pp 254-261.

A woman in her 70s was admitted with a cystic lesion in the pancreatic body. With no elevation of tumor markers, a CT scan incidentally demonstrated a 10 mm solid lesion in the caudal part of the original cystic lesion, with delayed contrast enhancement. EUS showed a small cystic space around that lesion. Since the lesion had no findings suspicious for malignancy, it was followed for 17 months with imaging studies. During this period, no remarkable changes were seen on imaging. However, to establish the diagnosis, we performed EUS-FNA resulting in a histological diagnosis of branch duct IPMN (BD-IPMN). Due to these abnormal findings, distal pancreatectomy was performed, and the diagnosis of BD-IPMN of gastric type without malignant features was confirmed. The tumor occupied most of a cyst, consistent with the preoperative findings. However, it had a different gross appearance from typical BD-IPMN. We report a BD-IPMN with an atypical shape.

Surgical Resection of Mucinous Cystic Neoplasm Which Showed Increase and Decrease in Size Over 30-Months Follow-up: A Case Report

Makoto Takahashi,*† Takashi Hatori,* Tomohisa Kadomura,* Atsushi Kato,* Yoshifumi Ikeda,* Nobuhiro Nitori,* Ayu Kato,* Junpei Nakadai,* Masaru Miyazaki,*† Masayuki Ohtsuka.† *Digestive Disease Center, International University of Health and Welfare, Mita Hospital, Tokyo; Department of General Surgery, Chiba University, Graduate School of Medicine, Chiba, Japan. Suizo. Vol 34, No 5, pp 262-269.

Although a unilocular cystic tumor 10 mm in diameter was detected in the pancreatic tail of a woman in her 40s during routine evaluation, it was observed because there were no findings consistent with malignancy. At 24 months after identifying the lesion, the tumor increased in size and became multilocular with internal calcifications. Six months later, she was referred for further examination. Dynamic CT scan revealed a decrease in the cystic component and overall tumor size, but a solid component was seen. Based on suspicion of a non-invasive malignant tumor, she underwent laparoscopic distal pancreatectomy. The pathological findings showed low-grade dysplasia of the cystic epithelium and ovarian-type stroma and was positive for progesterone and estrogen receptors. Although the tumor was diagnosed as a mucinous cystic neoplasm, low-grade dysplasia, the absence of a common capsule and the cyst in cyst formation was an interesting feature considering the progression of a mucinous cystic neoplasm. The pathological findings also suggest communication between the tumor and the main pancreatic duct, which might have caused a decrease in the tumor size.

Primary Unresectable Pancreatic Acinar Cell Carcinoma Which Became Resectable After S-1 and Oxaliplatin Treatment

Taro Mashiko, Kohei Tajima, Naoki Yazawa, Yoshihito Masuoka, Toshio Nakagohri. Department of Gastroenterological Surgery, Tokai University School of Medicine, Kanagawa, Japan. Suizo. Vol 34, No 5, pp 270-278.

A 67-year-old male underwent upper gastrointestinal evaluation which revealed abnormalities. Upper gastrointestinal endoscopy identified a large tumor extending from the antrum of the stomach to the descending portion of the duodenum. Biopsy results supported the diagnosis of poorly differentiated adenocarcinoma. Contrast-enhanced computed tomography (CT) scan showed a 68 mm tumor extending from the antrum of the stomach to the duodenum and the head of the pancreas. Resection was not feasible due to a portal vein tumor thrombus. The patient was treated with six cycles of S-1 and oxaliplatin combination chemotherapy. CT scan after chemotherapy showed reduction in size of the primary tumor and the portal vein tumor thrombus. Pancreatoduodenectomy with portal vein reconstruction was then performed. The inside of the tumor was diffusively positive for the immunohistological marker BCL10. Ultimately, the patient was diagnosed with pancreatic acinar cell carcinoma with duodenal invasion. Local recurrence was found four months after resection, but 13 months after starting chemotherapy, the patient is still alive. This suggests, similar to previous reports, that 5-fluorouracil-based chemotherapy is effective for patients with acinar cell carcinoma.

Autoimmune Pancreatitis Accompanied by Pancreatic Fistula Secondary to Pancreatic Cyst Rupture

Shinji Miyajima, Shoutai Takeda, Muneji Yasuda, Tomoko Hoshi, Susumu, Hoshi, Yuichi Tanaka, Haruo Takaya, Kozo Kajimura. Department of Gastroenterology, Kishiwada Municipal Hospital, Osaka, Japan. Suizo. Vol 34, No 6, pp 293-301.

A 70-year-old man presented with epigastric pain and was diagnosed with acute pancreatitis with diffuse pancreatic swelling and pseudocysts on abdominal CT scan and MRI. Despite non-operative management, he developed diarrhea and abdominal discomfort and was confirmed to have massive ascites and thromboses in the aorta and portal vein. A pancreatic fistula was diagnosed on the basis of high a amylase level in the ascitic fluid and ERCP findings of irregular narrowing of the main pancreatic duct with direct leakage of contrast from the pancreatic tail cyst into the abdominal cavity. There was no tumor in the EUS-FNA specimen. He was diagnosed with autoimmune pancreatitis based on the EUS-FNA and ERCP findings and a high level of serum IgG4. After initiating steroid therapy and placement of a pancreatic stent, swelling of the pancreas and ascites were reduced as well as normalization of serum IgG4 levels. The steroid dose was tapered, and four months later, the pancreatic stent was removed with resolution of the pancreatic fistula and confirmation of regression of the pancreatic cyst. This is a rare case of autoimmune pancreatitis with a pancreatic fistula secondary to pancreatic cyst rupture.

A Pancreatic Neuroendocrine Tumor With Autoimmune Pancreatitis and Multifocal Mass Lesions

Naoto Koike,* Tadashi Shiraishi,† Masayoshi Saito,‡ Yuji Ohshima,* Toshiaki Takeuchi,* Seiji Arita,* Daisuke Sasai,§ Makio Kawakami.∥ *Department of Surgery, Seirei Sakura Citizen Hospital, Chiba; Department of Frontier Surgery, Graduate School of Medicine, Chiba University, Chiba; Departments of Gastroenterology and §Pathology, Seirei Sakura Citizen Hospital, Chiba; Department of Pathology, Japanese Red Cross Narita Hospital, Chiba, Japan. Suizo. Vol 34, No 6, pp 302-311.

A 71-year-old female was found to have a 14 mm hypo-echoic tumor in the head of the pancreas head on screening abdominal ultrasonography. Abdominal contrast CT scan and MRI did not show the lesion. However, EUS also revealed two more tumors, 13.4 and 17.4 mm in the tail of the pancreas. Endoscopic ultrasonography-guided fine needle aspiration was performed for the lesions in the pancreatic head and tail with a suspected diagnosis of pancreatic neuroendocrine tumor (pNET). Enucleation of the pancreatic head lesion and distal pancreatectomy with lymph node dissection were performed. Histopathological examination of the surgical specimen showed a pNET in the head of the pancreas, and the pancreatic tail tumors were IgG4 related autoimmune pancreatitis. In addition, two small pNET lesions, found on histopathological examination were found in the pancreatic tail. The postoperative course was unremarkable and the patient is alive without recurrence more than 4 years postoperatively.

Relapsing Pancreatitis With An Excellent Response to Bromhexine Hydrochloride

Toyotaka Kasai,* Koichi Kawabe,* Hiroyuki Eto,* Seiji Muramatsu,* Moriyasu Nakahara,* Yosuke Miyahara,* Taro Ogino,* Shomei Ryozawa.† *Department of Gastroenterology, Fukaya Red Cross Hospital, Saitama; Department of Gastroenterology, Saitama Medical University International Medical Center, Saitama, Japan. Suizo. Vol 34, No 6, pp 312-316.

A 60-year-old male had eight episodes of acute pancreatitis and was hospitalized seven times over 17-months. Although various studies were performed, the cause of acute pancreatitis could not be determined. Treatment with oral bromhexine hydrochloride was started with the expectation that it would prevent relapse of the pancreatitis and he has not had a relapse for 42 months. The administration of bromhexine hydrochloride may be a viable treatment option for patients with relapsing pancreatitis.

Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.