{"title":"A case of hepatic epithelioid hemangioendothelioma with features resembling those of acute-onset autoimmune hepatitis that was undiagnosed before liver transplantation.","authors":"Shingo Usui, Po-Sung Chu, Mikine Hirano, Yasushi Hasegawa, Akihisa Ueno, Rui Nomura, Hideaki Obara, Yuko Kitagawa, Takanori Kanai, Nobuhiro Nakamoto","doi":"10.1007/s12328-025-02117-y","DOIUrl":"https://doi.org/10.1007/s12328-025-02117-y","url":null,"abstract":"<p><p>Hepatic epithelioid hemangioendothelioma (HEHE), which is extremely rare, is considered to have a malignant grade between that of hepatic hemangioma and that of hepatic hemangiosarcoma; however, some cases progress so quickly that they present with portal hypertension. We report the case of a woman with findings similar to those of acute-onset autoimmune hepatitis (AIH) that was not diagnosed before liver transplantation. The patient presented with jaundice and ascites. A hematological examination revealed negative tumor markers, high IgG levels, and negative hepatitis virus markers. Computed tomography findings of the liver showed map-like signs characteristic of acute-onset AIH. Despite some response, immunosuppressive drugs such as prednisolone, cyclosporine, and mycophenolate mofetil did not improve liver failure, and she underwent liver transplantation after 200 days of treatment. The explanted liver exhibited white areas that extended in a map-like manner and were occupied by fibrous stroma. Tumors with WWTR1-CAMTA1 gene fusion were recognized and diagnosed as HEHE. Although a histological examination is essential, a percutaneous liver biopsy could not be performed preoperatively because of the presence of ascites. Furthermore, the rarity of the disease, similarity of imaging findings with non-neoplastic patterns, and serological findings made it difficult to differentiate this case from acute-onset autoimmune hepatitis.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143718255","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}
Kengo Okabe, Naoto Iwai, Tatsuya Hirota, Ken Inoue, Osamu Dohi, Kanji Yamaguchi, Michihisa Moriguchi, Tomohisa Takagi, Hideyuki Konishi, Yoshito Itoh
{"title":"Stent placement for main portal vein occlusion to eradicate ectopic varices after pancreatoduodenectomy.","authors":"Kengo Okabe, Naoto Iwai, Tatsuya Hirota, Ken Inoue, Osamu Dohi, Kanji Yamaguchi, Michihisa Moriguchi, Tomohisa Takagi, Hideyuki Konishi, Yoshito Itoh","doi":"10.1007/s12328-025-02118-x","DOIUrl":"https://doi.org/10.1007/s12328-025-02118-x","url":null,"abstract":"<p><p>Ectopic varices bleeding around the choledochojejunostomy site, potentially caused by portal vein occlusion, is a rare complication following pancreatoduodenectomy. In this report, we present a case of ectopic varices around the choledochojejunostomy site after pancreatoduodenectomy. A 55-year-old man who underwent pancreatoduodenectomy with portal vein reconstruction for pancreatic ductal adenocarcinoma suffered from hematochezia. Contrast-enhanced computed tomography revealed total occlusion of the main portal vein and ectopic varices around the choledochojejunostomy anastomosis site. An attempt at endoscopic hemostasis using clipping was unsuccessful. Consequently, a main portal vein stent was inserted to eliminate the varices. We successfully restored blood flow in the portal vein and eradicated the varices. Additionally, endoscopic changes were directly visualized using double-balloon endoscopy. Therefore, portal vein stent placement may be an effective treatment option for ectopic variceal bleeding around the choledochojejunostomy site following pancreatoduodenectomy.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143699854","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}
Li-Sa Chang, Tsutomu Nishida, Dai Nakamatsu, Kengo Matsumoto, Masashi Yamamoto, Shiro Adachi, Koji Fukui
{"title":"Therapeutic success of factor XIII substitution for IgA vasculitis with gastrointestinal manifestation.","authors":"Li-Sa Chang, Tsutomu Nishida, Dai Nakamatsu, Kengo Matsumoto, Masashi Yamamoto, Shiro Adachi, Koji Fukui","doi":"10.1007/s12328-025-02112-3","DOIUrl":"https://doi.org/10.1007/s12328-025-02112-3","url":null,"abstract":"<p><p>A 52-year-old man presented with severe abdominal pain, elevated C-reactive protein (CRP) levels, and characteristic skin findings, leading to a diagnosis of immunoglobulin A (IgA) vasculitis with gastrointestinal (GI) involvement. Initial evaluation, including contrast-enhanced computed tomography (CT) and esophagogastroduodenoscopy (EGD), revealed marked inflammation of the duodenum and a punched-out ulcer, both of which showed partial improvement with conservative treatment. However, the patient developed worsening abdominal pain, arthralgia, and purpura, accompanied by reduced plasma factor XIII activity (47%). Factor XIII substitution therapy was initiated as a monotherapy, resulting in immediate symptom relief and significant endoscopic improvement of the duodenal ulcer. This case highlights the potential of factor XIII monotherapy as an effective treatment option for adult IgA vasculitis with severe GI manifestations.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673237","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":"Response to the letter to the editor on \"reconsidering invasive diagnostics in asymptomatic atypical autoimmune pancreatitis: a case-inspired discussion''.","authors":"Kazuya Koizumi, Karen Kimura","doi":"10.1007/s12328-025-02116-z","DOIUrl":"https://doi.org/10.1007/s12328-025-02116-z","url":null,"abstract":"","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669123","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":"Metastatic synovial sarcoma masquerading as primary neuroendocrine tumor of pancreas: a diagnostic conundrum.","authors":"Sherrin Jacob, Balamurugan Thirunavukkarasu, Rajni Yadav, Anany Gupta, Samagra Agarwal, Shamim A Shamim, Sameer Rastogi, Adarsh Barwad, Deepali Jain","doi":"10.1007/s12328-025-02110-5","DOIUrl":"https://doi.org/10.1007/s12328-025-02110-5","url":null,"abstract":"<p><p>Metastatic sarcomas to the pancreas are extremely rare, with poor survival rates. Therefore, rapid diagnosis and differentiation from primary malignant tumors of pancreas thereby guiding the treatment is indispensable. Endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) is the current diagnostic modality of choice for pancreatic tumor sampling with promising results. Definitive pathologic diagnosis requires adequate tissue for performing ancillary studies. We present a rare case of metastatic synovial sarcoma in the pancreas in an elderly male to endorse the utility of EUS-FNB with imprint cytology as a rapid and effective diagnostic tool.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143656272","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":"Effectiveness of pure laparoscopic right hepatectomy with a combined anterior and cranio-ventral approach for a giant hemangioma in a young woman.","authors":"Hiroaki Sugita, Shinichi Nakanuma, Tomokazu Tokoro, Ryohei Takei, Mitsuyoshi Okazaki, Kaichiro Kato, Satoshi Takada, Isamu Makino, Shintaro Yagi","doi":"10.1007/s12328-025-02111-4","DOIUrl":"https://doi.org/10.1007/s12328-025-02111-4","url":null,"abstract":"<p><p>Pure laparoscopic right hepatectomy is a challenging procedure especially for large tumors because of difficulties in mobilizing the liver and the risk of bleeding. An anterior approach has been proposed to overcome these problems. In this case report, we described a young unmarried woman with a giant hemangioma who successfully underwent pure laparoscopic right hepatectomy using the anterior approach. After transection of the anterior and posterior Glissonean pedicles, the liver parenchyma was divided using the cranio-ventral approach. The middle hepatic vein was exposed from the root side to periphery without split injury, and branches were safely divided. Because the tumor completely compressed the right hepatic vein (RHV), the inferior RHV (IRHV) was well-developed instead. After transecting both RHV and IRHV, the right lobe was mobilized and removed through a Pfannenstiel incision in the lower abdomen. The surgery was completed without tumor injury, and the blood loss was low. The postoperative course was uneventful and the patient was very satisfied with the wound appearance. This case suggests that the combination of the anterior and cranio-ventral approach may reduce bleeding from the tumor and bleeding due to injury of the hepatic vein in the case of giant tumors, contributing to safe hepatectomy.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143662896","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":"Pancreatic cancer secondary to biliary tract cancer associated with pancreaticobiliary maljunction: intraductal dissemination through the common channel.","authors":"Keishi Sugimachi, Takahiro Tomino, Tomonari Shimagaki, Emi Onishi, Yutaka Koga, Kenichi Taguchi, Masayuki Furukawa, Terumasa Hisano, Rie Sugimoto, Masaru Morita","doi":"10.1007/s12328-025-02108-z","DOIUrl":"https://doi.org/10.1007/s12328-025-02108-z","url":null,"abstract":"<p><p>Patients with pancreaticobiliary maljunction (PBM) have a high risk of biliary tract cancer (BTC). The risk of pancreatic cancer is also reported to be higher in patients with PBM compared to the general population; the underlying cause remains unclear. We report a 73-year-old man with widespread pancreatic cancer involving the entire pancreas. The patient previously underwent curative resection for gallbladder cancer and hilar cholangiocarcinoma concomitant with PBM. A total pancreatectomy was performed for the new pancreatic lesion. Histopathological examination revealed a papillary tumor predominantly composed of intraepithelial lesions that extended throughout the pancreatic duct, with skip lesions and irregular invasive foci at multiple sites within the duct. The morphological features and mucin profile were similar to those of the previous biliary lesions. Genetic analysis of the current lesion showed wild-type KRAS, GNAS, and PIK3CA genes consistent with the previous lesions, indicating that the pancreatic and biliary lesions were molecularly identical clones. Based on the clinicopathological findings and molecular analysis, we concluded that the BTC had spread intraluminally to the pancreatic duct through the common channel of the PBM, resulting in intraductal dissemination. Patients with PBM should be followed for pancreatic lesions, because of the risk of intraductal dissemination of BTC.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143572094","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}
Katsuharu Tono, Tsutomu Nishida, Kengo Matsumoto, Masafumi Yamashita, Aya Sugimoto, Dai Nakamatsu, Masashi Yamamoto, Hiromi Tamura, Junzo Shimizu, Koji Fukui
{"title":"A case of pancreatic arteriovenous malformation diagnosed after the onset of abdominal symptoms.","authors":"Katsuharu Tono, Tsutomu Nishida, Kengo Matsumoto, Masafumi Yamashita, Aya Sugimoto, Dai Nakamatsu, Masashi Yamamoto, Hiromi Tamura, Junzo Shimizu, Koji Fukui","doi":"10.1007/s12328-025-02104-3","DOIUrl":"https://doi.org/10.1007/s12328-025-02104-3","url":null,"abstract":"<p><p>Pancreatic arteriovenous malformation (P-AVM) is an extremely rare vascular anomaly characterized by abnormal connections between arteries and veins bypassing the capillary network. Less than 200 cases have been reported worldwide, and standardized treatment guidelines have not yet been established. A 72-year-old man presented with abdominal distension, diarrhea, and appetite loss. Contrast-enhanced abdominal computed tomography revealed a 5 cm mass in the pancreatic tail with multiple feeding and draining vessels, a portosystemic shunt, and moderate ascites, suggesting a P-AVM. Endoscopic ultrasonography revealed a hypoechoic area in the pancreatic tail measuring 50 mm in diameter. Esophagogastroduodenoscopy revealed F2-type esophageal varices. Based on imaging findings and clinical history, the patient was diagnosed with P-AVM, and Osler-Weber-Rendu disease was excluded. Initial treatment with transarterial embolization was attempted, but proved insufficient due to high blood flow and multiple feeders. Surgical resection via distal pancreatectomy was subsequently performed to alleviate portal hypertension and resolve abdominal symptoms, although significant intraoperative bleeding occurred. This case highlights the diagnostic and therapeutic challenges of P-AVMs, particularly in high-flow lesions with complex vascular anatomy. Surgical resection remains the definitive treatment for symptomatic P-AVM and effectively resolves the associated complications. The insights gained from this case may contribute to the clinical management of this rare condition.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143540247","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}
Yoshiko Nakano, Gen Nishikawa, Kanako Degawa, Koki Moriyoshi, Katsutoshi Kuriyama, Yasuhiro Watanabe, Shin'ichi Miyamoto
{"title":"A case of multiple advanced colon cancers with spontaneous regression of only one lesion after biopsy: a case report and literature review.","authors":"Yoshiko Nakano, Gen Nishikawa, Kanako Degawa, Koki Moriyoshi, Katsutoshi Kuriyama, Yasuhiro Watanabe, Shin'ichi Miyamoto","doi":"10.1007/s12328-025-02106-1","DOIUrl":"https://doi.org/10.1007/s12328-025-02106-1","url":null,"abstract":"<p><p>A 90-year-old man underwent a colonoscopy due to abdominal distension, revealing half-circumferential Type 2 advanced cancers in the ascending and transverse colon. No distant metastasis was detected, and 3 months later, laparoscopic extended right hemicolectomy was performed for both lesions. Pathological examination revealed ulcers and mucus retention in the ascending colon lesion without tumor components. A small number of signet ring cell-like tumor cells were found in the regional lymph node of the ascending colon, while well-to-moderately differentiated tubular adenocarcinoma was observed on the serosal surface in the transverse colon. Tumor regression was observed in the ascending colon cancer and lymph-node metastasis. Mismatch repair (MMR) protein immunostaining was conducted on biopsy tissues from both lesions. The ascending colon lesion showed weak positivity for MLH1, positivity for MSH2 and MSH6, and negativity for PMS2, indicating MMR deficiency, whereas the transverse colon lesion showed positivity for all of them, indicating MMR-proficient tumor. This is the first case report of multiple advanced colon cancers, where only one lesion exhibited spontaneous regression after biopsy, suggesting a potential link between MMR deficiency and the spontaneous regression of colorectal cancer.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143540243","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}