{"title":"A case of BRCA1-mutated giant pancreatic acinar cell carcinoma successfully treated with modified FOLFIRINOX therapy and radical resection.","authors":"Toru Watanabe, Yasuhiro Nagaoka, Nana Kimura, Mina Fukasawa, Yoshihiro Shirai, Katsuhisa Hirano, Kazuto Shibuya, Isaku Yoshioka, Takeru Hamashima, Tsutomu Fujii","doi":"10.1007/s12328-024-01992-1","DOIUrl":"10.1007/s12328-024-01992-1","url":null,"abstract":"<p><p>Pancreatic acinar cell carcinoma (PACC) is a rare type of pancreatic cancer; further, its pathogenesis and treatment strategies remain unclear. We report the case of a 70-year-old man who presented with a chief complaint of abdominal distention. Computed tomography scans revealed a large lobulated mass (tumor diameter: 150 mm) in the pancreatic body tail, which was diagnosed as a PACC through endoscopic ultrasonography fine needle aspiration. The other imaging modalities did not reveal distant metastases, and the tumor was classified as resectable. Neoadjuvant chemotherapy was planned after staging laparoscopy ruled out microscopic distant metastasis. First-line chemotherapy with gemcitabine + nab-paclitaxel failed due to tumor growth and worsening abdominal distention. Evaluation using the BRACAnalysis<sup>®</sup> device indicated that the patient was positive for BRCA1 mutation. Second-line modified FOLFIRINOX (mFFX) resulted in a marked decrease in elastase 1 levels; moreover, a partial antitumor response was observed, which prompted radical resection. After distal pancreatectomy, the patient has survived for 3.5 years without recurrence. BRCA-mutated pancreatic cancer is more likely to respond to mFFX, including platinum, and BRCA mutations have been reported to be highly prevalent in PACC. It is important to evaluate the presence of BRCA mutations in patients with PACC prior to treatment.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141247789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of ulcerative colitis with a variety of autoimmune diseases including ankylosing spondylitis, type 2 autoimmune pancreatitis, and primary sclerosing cholangitis.","authors":"Marie Kurebayashi, Akira Hashimoto, Mizuki Kawachi, Shoma Sawai, Takahiro Ono, Yuichi Tahara, Naoki Kuroda, Naohiko Yoshizawa, Hiroyuki Fuke, Atsuya Shimizu","doi":"10.1007/s12328-024-02001-1","DOIUrl":"10.1007/s12328-024-02001-1","url":null,"abstract":"<p><p>Ankylosing spondylitis (AS), primary sclerosing cholangitis (PSC), and autoimmune pancreatitis (AIP) are known as extraintestinal manifestations (EIMs) of ulcerative colitis (UC). A 74-year-old Japanese man visited our hospital because of white stool. He had been diagnosed with AS when he was 30 years old, and he was HLA-B27-positive. Based on various examination results, it was suspected that AIP had caused bile duct stricture. During the clinical course, he was diagnosed with UC and PSC. Then, AIP was diagnosed because he had localized pancreatic enlargement, irregular stenosis of the main pancreatic duct, PSC, and no tumor cells of pancreas. A patient with all four of these diseases, AS, AIP, PSC, and UC, is very rare. Therefore, we report a quite rare case with three EIMs (AS, PSC, and AIP) of UC.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141300143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A rare case of acute obstructive suppurative pancreatic ductitis (AOSPD) which developed pyogenic spondylitis.","authors":"Ayako Hirata, Tomoaki Matsumori, Muneji Yasuda, Yoshihiro Nishikawa, Masahiro Shiokawa, Norimitsu Uza, Hiroshi Seno","doi":"10.1007/s12328-024-02004-y","DOIUrl":"10.1007/s12328-024-02004-y","url":null,"abstract":"<p><p>Acute obstructive suppurative pancreatic ductitis (AOSPD) is an acute suppuration of the pancreatic duct. Endoscopic retrograde cholangiopancreatography (ERCP) drainage and intravenous antibiotics treatment is the mainstay of therapy. Herein we describe an extremely rare case of AOSPD leading to pyogenic spondylitis. A 61-year-old male with a past medical history of chronic pancreatitis and diabetes mellitus presented to our hospital with abdominal and dorsal pain, fever, and shock status. Laboratory data showed severe inflammation, disseminated intravascular coagulation, and normal pancreatic enzymes. Computed tomography showed dilated main pancreatic duct and surrounding pancreatic abscesses. Spinal abnormalities were not detected at this point. He was initially diagnosed as infected pancreatic pseudocyst, but did not respond well to conservative intravenous antibiotic treatment. ERCP performed one week later revealed purulent pancreatic juice and the diagnosis was changed to AOSPD. Upon ERCP, we experienced technical difficulty in passing obstructing calculi. However, successful pancreatic drainage was achieved using new dilation and penetration devices. The patient responded quickly to drainage, but later developed pyogenic spondylitis. Our case highlights the difficulty of diagnosing AOSPD, the usefulness of new devices in urgent endoscopic drainage, and underscores the possibility of progression of pyogenic spondylitis even after adequate treatment.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141431546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vikram S Gill, Sayi P Boddu, Sami Abujbarah, Kellie L Mathis, Amit Merchea, Justin T Brady
{"title":"Secondary amyloidosis in inflammatory bowel disease patients: findings from three tertiary medical centers.","authors":"Vikram S Gill, Sayi P Boddu, Sami Abujbarah, Kellie L Mathis, Amit Merchea, Justin T Brady","doi":"10.1007/s12328-024-02003-z","DOIUrl":"10.1007/s12328-024-02003-z","url":null,"abstract":"<p><p>Secondary amyloidosis (AA) is a disorder of protein conformation associated with inflammatory disorders. Detailed reports of patients diagnosed with AA and inflammatory bowel disease (IBD) are limited. This study reports the cases of eight patients, across three tertiary medical centers, diagnosed with both IBD and AA between 2000 and 2020. Seven patients had a diagnosis of Crohn disease (CD), while one had ulcerative colitis (UC). All patients were diagnosed with AA after being diagnosed with IBD (median: 15 years later). The small bowel (62.5%) and the colon (62.5%) were the most common IBD locations. 4 patients had undergone TNF-alpha inhibitor therapy and all CD patients required surgical treatment of their IBD. A history of fistula or abscess was identified in 5 patients. The most common initial site of AA was the kidney (75%). All 8 patients presented with some form of renal dysfunction and proteinuria (median: 1500 mg/24 h). Hypoalbuminemia was found in most patients. Six patients developed chronic kidney disease and 4 required dialysis. Anti TNF-alpha antibody therapy led to rapid improvement of renal function in one of four patients who received it. Three patients required a renal transplant. Four patients had died upon the latest follow-up (5-year survival: 75%). The presence of proteinuria, fistula, or abscess should serve as indicators for potentially increased AA risk in CD patients.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141330510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Multiple intraductal papillary neoplasms of bile duct diagnosed based on endoscopic ultrasonography and peroral cholangioscopy findings.","authors":"Masaki Onoe, Nobuhiko Fukuba, Yasuhide Kodama, Akihiko Oka, Kousaku Kawashima, Kotaro Shibagaki, Norihisa Ishimura, Yoshinori Kushiyama, Yasushi Uchida, Toru Furukawa, Shunji Ishihara","doi":"10.1007/s12328-024-02000-2","DOIUrl":"10.1007/s12328-024-02000-2","url":null,"abstract":"<p><p>A man in his 30s was referred to our department for evaluation of bile duct stricture and removal of an intrahepatic bile duct stone. Five years before his presentation, he underwent left hepatectomy for a giant hepatic hemangioma. There were no abnormalities in blood biochemical tests. Magnetic resonance cholangiopancreatography showed one 5 mm oval defect in region B6 and two 8 mm semicircular defects in the hilar bile duct. Endoscopic ultrasound revealed a 3.5 mm hypoechoic focal raised lesion in the hilar bile duct. Oral cholangioscopy revealed his two lesions in the hilar bile duct as white papillary elevations with mucus production. The pathological diagnosis of intraductal papillary neoplasm was determined (low-grade dysplasia, type 1, gastric type). After 1 and a half years, no expansion of the bile duct lesion was observed. Initially, it was thought to be a benign stenosis after liver resection, but based on the results of endoscopic ultrasound, we suspected a tumorous lesion, and we were able to make an accurate diagnosis, including histological type, using transoral cholangioscopy.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11436404/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141544575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yu Miyakawa, Sozaburo Ihara, Saaya Ishii, Yang Rui, Shoh Yajima, Yoku Hayakawa, Yosuke Tsuji, Mutsumi Okazaki, Yasuyuki Seto, Mitsuhiro Fujishiro
{"title":"First case report of intestinal lymphangiectasia with refractory bleeding from the duodenum, successfully treated by intra-abdominal lymphaticovenous anastomosis with venous ligation.","authors":"Yu Miyakawa, Sozaburo Ihara, Saaya Ishii, Yang Rui, Shoh Yajima, Yoku Hayakawa, Yosuke Tsuji, Mutsumi Okazaki, Yasuyuki Seto, Mitsuhiro Fujishiro","doi":"10.1007/s12328-024-02021-x","DOIUrl":"10.1007/s12328-024-02021-x","url":null,"abstract":"<p><p>Intestinal lymphangiectasia (IL) is a protein-losing enteropathy (PLE) that occasionally leads to gastrointestinal bleeding (GIB). We encountered a 41-year-old female with a 9-year history of duodenal IL with PLE and GIB that progressively worsened. Despite a diet, supplemented with medium-chain triglycerides, antiplasmin therapy, oral corticosteroids, octreotides, sirolimus, and repeated endoscopic hemostasis, her symptoms remained uncontrolled, leading to blood transfusion dependence. Lymphangiography revealed significant leakage from abnormal abdominal lymph vessels into the duodenal lumen. The patient subsequently underwent an abdominal-level lymphaticovenous anastomosis combined with local venous ligation. This approach resulted in a dramatic improvement and sustained resolution of both the PLE and GIB. More than 6 months after surgery, the patient remained free of symptoms and blood transfusion dependence.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11436469/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141626129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pathological complete response with FOLFIRINOX therapy for recurrence of pancreatic acinar cell carcinoma.","authors":"Katsuhito Teramatsu, Nao Fujimori, Masatoshi Murakami, Sho Yasumori, Kazuhide Matsumoto, Kohei Nakata, Masafumi Nakamura, Yutaka Koga, Yoshinao Oda, Yoshihiro Ogawa","doi":"10.1007/s12328-024-01983-2","DOIUrl":"10.1007/s12328-024-01983-2","url":null,"abstract":"<p><p>Pancreatic acinar cell carcinoma (PACC) is a very rare subtype of pancreatic cancer. Due to small number of patients, no standard chemotherapy protocol has been established. We experienced an extremely rare case of PACC with liver metastasis that showed a pathological complete response after modified FOLFIRINOX (mFFX) therapy. A 42-year-old man who underwent distal pancreatectomy for an 80 mm tumor at the pancreatic tail 3 years ago was referred to our hospital in September 2017 for the treatment of a recurrent liver tumor. Percutaneous biopsy revealed an acinar-neuroendocrine carcinoma, similar to the surgical specimen. He received eight cycles of irinotecan plus cisplatin chemotherapy. However, the tumor increased in size, and treatment was switched to mFFX therapy. The tumor in the liver shrank remarkably after nine cycles of mFFX therapy. Conversion surgery was selected, and the patient underwent hepatic left and caudate lobectomy 8 months after administration of mFFX. The resected specimen showed no viable tumor cells, indicating a pathological complete response. The histological diagnosis was reconsidered, and PACC was finally diagnosed via an additional immunohistological review. The patient has remained well with no recurrence for 6 years after surgery. This study is the first to report a case of pathological complete response with mFFX therapy for the recurrence of PACC.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140956392","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Minimal endoscopic sphincterotomy followed by papillary balloon dilation to relieve choledocholithiasis in a 6-year-old girl with hereditary spherocytosis.","authors":"Kiyoaki Yabe, Wataru Yamagata, Masamichi Satou, Itsuhiro Oka, Hideyuki Horike, Shin Namiki, Kenji Hosoi","doi":"10.1007/s12328-024-01960-9","DOIUrl":"10.1007/s12328-024-01960-9","url":null,"abstract":"<p><p>A 6-year-old girl previously diagnosed with hereditary spherocytosis was admitted to our hospital with gallstones and cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) was performed, and fluoroscopy revealed a dilated common bile duct (CBD) without evident stones, possibly due to spontaneous excretion through the papilla of Vater. A 7-French plastic stent was inserted into the CBD. After the procedure, a marked increase in pancreatic enzyme levels was observed, and she was diagnosed with post-ERCP pancreatitis (PEP). Stent placement could have been a cause of pancreatitis; therefore, we removed the stent. Subsequently, recovery from pancreatitis was confirmed, although she suddenly complained of abdominal pain and was diagnosed with choledocholithiasis recurrence. ERCP was repeated, and fluoroscopy revealed a dilated CBD with a stone. A minimal endoscopic sphincterotomy (EST) was performed to reduce the risk of PEP, and a biliary dilation balloon placed across the papilla was gradually inflated until the waist of the balloon disappeared. Stones were extracted using a retrieval balloon catheter. The abdominal pain resolved immediately, and the patient recovered without developing PEP. To our knowledge, this is the first case report of a pediatric patient treated with minimal EST followed by papillary balloon dilation for choledocholithiasis.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140189528","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Investigating morphological changes in a simple mucinous cyst during the follow-up period: a case report.","authors":"Tatsunori Satoh, Shinya Kawaguchi, Chinatsu Tsuchikabe, Hideyuki Kanemoto","doi":"10.1007/s12328-024-01955-6","DOIUrl":"10.1007/s12328-024-01955-6","url":null,"abstract":"<p><p>A 64-year-old man was referred to our department after a small pancreatic cystic lesion was discovered on computed tomography performed to assess choledocholithiasis. Multiple standard imaging modalities, including endoscopic ultrasound (EUS), failed to reveal pancreatic masses, wall thickening, or mural nodules. Consequently, a benign pancreatic cystic lesion was suspected, and the patient underwent biannual imaging studies including rotating magnetic resonance imaging and EUS. Six years after the initial detection of the pancreatic cyst, wall thickening was observed, leading to a shortened observation period of once every 3 months. After 6.5 years, hypoechoic area surrounding the cyst, which could be interpreted as thickening of the cyst wall was observed, prompting distal pancreatectomy due to the suspicion of malignant disease. The histopathological examination revealed a unilocular mucinous cyst with a single layer of cuboidal cells and low-grade dysplasia. A fibrous proliferation of the polycystic stroma and no ovarian-type stroma was observed. Malignant cells were absent from the cystic epithelium and stroma. The final histopathological diagnosis was a simple mucinous cyst of the pancreatic tail.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140193516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}