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Cowden syndrome (CS) is an autosomal dominant genetic cancer predisposition disease caused by mutation of the germline phosphatase and tensin homolog (PTEN) gene. A 60s-year-old man who has been diagnosed as CS at our hospital 10 years ago due to his medical history of Lhermitte-Duclos disease (LDD), thyroid medullary carcinoma and gastrointestinal polyposis. He was regularly followed up by the endoscopic examination of gastrointestinal polyps at our hospital. A recent routine examination revealed a surface irregular and hemorrhagic gastric polyp at the lower body of stomach. As a biopsy report showed a high possibility of carcinoma, an endoscopic mucosal resection (EMR) was performed. Histopathological examination of the EMR specimen revealed well-differentiated tubular adenocarcinoma developed in a hamartoma polyp. Immunohistochemical staining using anti-PTEN antibody showed that PTEN expression was completely deficient in the nuclei of carcinoma cells as well as majority of hamartoma cells, whereas a part of hamartoma cells showed PTEN nuclear expression. Furthermore, we applied the specimen to gene panel analysis and confirmed pGly127Arg (c379G > A) mutation present in PTEN gene. Helicobacter pylori (H. pylori) infection in the biopsied gastric specimen and immunohistochemically high expression of p16INK4a and p53 in cancer cells were detected. In addition to PTEN mutation, these changes may contribute to the gastric carcinogenesis of this patient. Although only few cases of gastric carcinoma in CS patients were reported, our case highlights the importance of regular and careful endoscopic observation of hamartoma polyps to detect early-gastric carcinoma in those with CS.

Functional lumen imaging probe (FLIP) Panometry classifies esophageal motility using esophagogastric junction (EGJ) opening and esophageal contractile patterns. A previous study evaluated FLIP Panometry interpretation among esophageal specialists (which demonstrated an average accuracy of 78% for FLIP motility diagnoses), but the interpretation performance of nonexperts remains unknown. This study aimed to assess the accuracy and interrater agreement of novice raters using FLIP Panometry v1.0 and the novel v2.0 (Dallas Consensus) motility classification schemes. Eight non-gastroenterology trainees without previous FLIP or esophageal motility experience interpreted 40 FLIP studies. Brief training included a video tutorial and review of 10 practice studies with an esophageal specialist. Accuracy was assessed using percent agreement with the reference standard assigned by two experienced raters. Interrater agreement was assessed using intraclass correlation coefficient (ICC) and Fleiss' kappa. Accuracy of motility diagnosis was mean (95% CI) 0.73 (0.68-0.78) for v2.0 and 0.78 (0.73-0.82) for v1.0. Accuracies ≥ 78% were achieved by four raters for v2.0 and five for v1.0, while other raters' accuracies ranged from 55% to 75%. Accuracy for EGJ opening classification was 0.80 (0.75-0.84) and for contractile response pattern was 0.78 (0.74-0.83) for v2.0 and 0.78 (0.74-0.83) for v1.0. Moderate-to-good interrater agreement was observed (ICC 0.80-0.83; kappa 0.55-0.61). Following brief training, some novice raters achieved accuracy akin to esophageal motility specialists when interpreting FLIP Panometry motility studies. While variation in accuracy highlights the need for improved and adequate training methods for esophageal motility interpretation, accurate FLIP Panometry interpretation was achievable among nonspecialists, supporting feasibility for broad use. Gastroenterologists use a tool called FLIP Panometry to diagnose swallowing disorders by measuring how well the esophagus opens and contracts. Until now, only motility specialists had been tested on how accurately they could interpret FLIP results. This study tested eight trainees with no prior experience reading FLIP (nor esophageal manometry). After brief training, which included a video tutorial and review of 10 practice cases, the trainees interpreted 40 real studies. The results were promising. Some trainees performed as accurately as specialists (around 78% accuracy). Raters also showed reasonable agreement with each other. These results suggest that with even brief training, nonspecialists can learn to read FLIP Panometry studies reliably. They also suggest that FLIP could realistically be used widely and beyond just expert centers, though ongoing efforts to optimize training tools are still needed.

Efferent loop stenosis (ELS) after pancreaticoduodenectomy (PD) with gastrojejunostomy reconstruction is an uncommon cause of delayed gastric emptying. Reoperation in the early postoperative period carries substantial risk. Endoscopic balloon dilation and stenting are often used for benign postoperative strictures, but when fixed angulation rather than a short concentric narrowing is the main problem, these approaches may be unsafe or ineffective. A 51-year-old woman with a more than 2-year history of intermittent epigastric fullness and pain underwent laparoscopic PD with Child reconstruction for an approximately 2 cm pancreatic head mass. Imaging suggested a pancreatic neuroendocrine tumor and histopathology confirmed a grade 2 pancreatic neuroendocrine tumor (Ki-67 about 5%) with negative margins. The early postoperative course was initially stable. On postoperative day 7, she developed upper abdominal distension and high-output bilious nasogastric drainage (800-1,100 mL/day), and oral intake could not be advanced. Conservative treatment with nasogastric decompression, bowel rest, intravenous fluids, proton-pump inhibitor therapy, and prokinetic agents was continued for five days but did not improve the symptoms. A barium meal examination showed delayed gastric emptying with impaired flow into the efferent limb. Upper endoscopy demonstrated viscous bile pooling in the stomach and a sharply angulated efferent loop at the gastrojejunostomy, without a discrete anastomotic ring stricture. These findings were consistent with ELS caused mainly by loop angulation. Because stable coaxial positioning of a balloon catheter across the kinked segment was considered difficult and potentially unsafe, we chose a combined strategy. Endoscopic submucosal dissection (ESD) was used to partially remove a saddle-shaped mucosal ridge at the efferent-loop entrance. Clips were applied for hemostasis and to provide traction, straightening the efferent loop and improving alignment with the gastric lumen. A 20 mm × 80 mm fully covered self-expanding metal stent (FCSEMS) was then placed to maintain patency. After the procedure, the patient's abdominal distension improved, nasogastric drainage decreased, and the tube was removed on day 3. Oral intake was gradually resumed. At 1 month, a contrast study and repeat endoscopy showed good passage through a straightened efferent limb, and the FCSEMS was removed without complications. At 3 months, she reported good appetite, a weight gain of about 5 kg, and endoscopy showed a widely patent efferent loop without recurrent stenosis. ESD-assisted mucosal release with clip-based traction and temporary FCSEMS placement corrected angulation-type ELS early after PD in this patient. This endoscopic approach, which focuses on modifying the loop geometry rather than simple radial dilation, may be considered as an alternative to surgical revision in carefully selected cases where standard balloon dilation is not feasible.

Gastric cardia cancer is a biologically heterogeneous malignancy arising at the gastroesophageal junction that is, the upper opening of the stomach rather than the heart. It develops along an inflammation-metaplasia-dysplasia-carcinoma sequence and remains clinically challenging because early lesions are often occult and advanced disease is shaped by a treatment-resistant tumor microenvironment. A clinically useful review therefore needs to distinguish gastric cardia cancer from non-cardia gastric cancer and from esophageal adenocarcinoma, while also clarifying where current evidence is direct and where it is only extrapolated. Banxia Xiexin Tang (BXT) is a seven-botanical formula containing Pinellia ternata (Thunb.) Makino. [Araceae] (Pinelliae Rhizoma), Coptis chinensis Franch. [Ranunculaceae] (Coptidis Rhizoma), Scutellaria baicalensis Georgi [Lamiaceae] (Scutellariae Radix), Zingiber officinale Roscoe [Zingiberaceae] (Zingiberis Rhizoma Recens), Panax ginseng C.A.Mey. [Araliaceae] (Ginseng Radix et Rhizoma), Glycyrrhiza uralensis Fisch. ex DC. [Fabaceae] (Glycyrrhizae Radix et Rhizoma), and Ziziphus jujuba Mill. [Rhamnaceae] (Jujubae Fructus). Available preclinical and supportive-care studies suggest that Banxia Xiexin Tang or selected metabolites within the formula may modulate inflammatory signaling, oxidative stress, epithelial barrier injury, and treatment-related gastrointestinal toxicity. However, the current evidence base is uneven: much of it comes from gastritis, reflux injury, or non-cardia gastric disease models rather than directly from human gastric cardia cancer. This review critically synthesizes the available literature on integrative management of gastric cardia cancer and related precancerous lesions, with emphasis on what the current data do support, what they do not support, and what research gaps remain. The revision adds a transparent search strategy, clearer terminology, a more cautious interpretation of pharmacological claims, and an explicit discussion of limitations. Because the included studies were heterogeneous in design, population, intervention, and outcome reporting, the evidence is synthesized narratively rather than through quantitative meta-analysis.

The American Gastroenterological Association (AGA) Clinical Care Pathway provides a tiered, noninvasive algorithm for fibrosis risk stratification in populations at high risk for metabolic dysfunction-associated steatotic liver disease (MASLD). We assessed the 2-year longitudinal performance of the AGA pathway using magnetic resonance elastography (MRE) as the reference. This prospective cohort study enrolled adults aged 50-79 years with type 2 diabetes mellitus from ambulatory care clinics between the dates of February 2016 and February 2025. Participants underwent a standardized clinical research visit with Fibrosis-4 index (FIB-4), vibration-controlled transient elastography (VCTE), and MRE at baseline and at a 2-year interval at the UCSD MASLD Research Center. Of 626 participants with a baseline assessment, 209 had longitudinal follow-up assessment and were included in the study. The prevalence of MASLD was 69.9%, and 19.0% had significant fibrosis at baseline (MRE ≥3.30 kPa). Applying the AGA pathway of FIB-4 and VCTE, the false negative rate (low risk by pathway with MRE ≥3.30 kPa) at baseline was 7% with 17.6% of participants qualifying for specialty referral. At 2-year follow-up, the false negative rate decreased to 3% and an additional 7%, respectively qualified for specialty referral. Applying a FIB-4 cut point of 1.0 decreased the false negative rate to 0%; however, the number of patients requiring VCTE increased by 54% over 2-years. Longitudinal reassessment of patients initially classified as low risk by the AGA Clinical Care Pathway substantially reduced misclassification of significant fibrosis, while maintaining a low rate of specialty referral. These findings support serial re-evaluation as a key component of noninvasive fibrosis risk stratification in at-risk populations.

Rett syndrome (RTT) is a rare, X-linked neurodevelopmental disorder that primarily affects females. Trofinetide (TROF) was approved for RTT in individuals aged ≥2 years. Although the DAFFODIL trial examined TROF efficacy in children aged 2-4 years, real-world evidence in this age group remains limited. A retrospective cohort study was conducted using linked medical and pharmacy claims from 01/01/2021-09/30/2024 (study period). Individuals aged 2-4 years with RTT who initiated TROF during 04/01/2023-03/31/2024 (identification period) were included (index date=first TROF RX). Outcomes included baseline characteristics, prescriber specialty, TROF persistence (≤90-day allowable gap), dosing patterns based on shipped/dispensed RXs (BID, mg; % target daily dose [%TDD]), time on treatment, and restarts among non-persistent individuals. Variables were summarized descriptively. Kaplan-Meier analyses assessed the time to treatment non-persistence. Among 159 individuals, 65.4% were persistent and 34.6% were non-persistent; 14.5% of non-persistent individuals restarted TROF. Mean±SD age was 3.2±0.8 vs 3.3±0.7 years in persistent vs non-persistent groups. Females comprised 95.2% vs 87.3%, respectively. Child neurology was the most common prescriber specialty (54.8% vs 63.6%). Non-persistent individuals had higher rates of dysphagia (34.5% vs 16.3%), gastrostomy (20.0% vs 3.8%), and breathing irregularities (16.4% vs 1.9%). Mean BID dose and %TDD were similar between groups across shipments. Median (IQR) time on treatment was 13.3 (6.1-17.2) months in the persistent group vs 4.4 (0.8-12.6) months in the non-persistent group. Kaplan-Meier analysis showed >87.5% remained on TROF beyond 3 months and >65.0% remained on TROF through end of available follow-up. In routine US practice, approximately two-thirds of children aged 2-4 years initiating TROF remained persistent during available follow-up, with sustained use beyond 1 year and a subset (14.5% of non-persistent children) restarting after discontinuation. Non-persistent children had significantly higher baseline rates of dysphagia, gastrostomy, and breathing irregularities, suggesting that early disease-related multisystem complications may be associated with reduced treatment continuity. These findings complement DAFFODIL and provide early real-world evidence on TROF persistence, restarts, and dosing patterns in this young RTT population. Why was the study done? Rett syndrome is a rare genetic disorder that impacts brain development, primarily in young girls. In March 2023, the drug trofinetide became the first approved treatment for this condition in the United States. While clinical trials showed the drug is safe and effective for young children aged 2 to 4, doctors and families need real-world evidence to see how well children stick with the treatment in everyday life outside of strict trial settings. What did the researchers do and find? We analyzed health insurance and pharmacy data for 159 children aged 2 to 4 with Rett syndrome who started trofinetide between April 2023 and March 2024. We looked at how long they stayed on the medication, their daily dosages, and whether those who stopped eventually restarted. Our study revealed that: Two-thirds of the children (65.4%) successfully stayed on their trofinetide treatment continuously for over a year.Children who stopped taking the medication usually did so around the 4-month mark. However, about 14.5% of those children eventually restarted the drug after a break.Children who stopped the medication had higher rates of other health issues before starting, such as stomach problems and seizures, compared to those who stayed on it. What do these results mean? Real-world data shows most young children with Rett syndrome stay on trofinetide long-term. Since those with more pre-existing health issues often stop treatment, doctors can use this information to closely support high-risk patients.

Krukenberg tumors are rare metastatic ovarian neoplasms, most commonly originating from gastrointestinal adenocarcinomas, and are histologically characterized by mucin-producing signet-ring cells. Their clinical and radiological presentation is often nonspecific, which may delay diagnosis and complicate differentiation from primary ovarian malignancies. Imaging plays a crucial role in the initial assessment, with ultrasonography typically revealing bilateral or unilateral adnexal masses that are heterogeneous, predominantly solid, and associated with irregular margins and internal necrotic areas. Transvaginal ultrasound further refines lesion characterization by demonstrating a disorganized architecture with mixed solid and cystic components, highly suggestive of malignancy. Definitive diagnosis relies on histopathological examination supported by immunohistochemical profiling to identify the primary origin. Early recognition is essential given the poor prognosis and the need for multidisciplinary management. In this context, we report a case of a metastatic ovarian tumor consistent with a Krukenberg-type lesion.

The CheckMate 649 trial showed the superiority of first-line nivolumab plus chemotherapy (Nivo-CT) over chemotherapy alone for advanced gastric or gastroesophageal junction (GEJ) cancer with a programmed cell death-ligand 1 (PD-L1) combined positive score (CPS)&#x2009;&#x2265;&#x2009;5. However, few studies have investigated the correlation between PD-L1 CPS and the efficacy of first-line Nivo-CT in the real-world setting. This multicenter retrospective study included patients with unresectable advanced or recurrent gastric or GEJ cancer who were started on first-line Nivo-CT between December 2021 and December 2023 at any of four institutions in Japan. PD-L1 CPS was classified into three groups (<&#x2009;1, 1 to <&#x2009;5, and &#x2265;&#x2009;5) before the start of Nivo-CT. A total of 234 patients were included (median age 70&#x2009;years; 64.5% male; 89.7% with gastric primary). PD-L1 CPS was <&#x2009;1 in 35 patients (15.0%), 1 to <&#x2009;5 in 76 (32.5%), and &#x2265;&#x2009;5 in 123 (52.5%). Median follow-up was 14.0&#x2009;months (range 2.3-30.3). Median overall survival was 18.7 (95% confidence interval [CI] 17.4-not reached), 23.4 (95% CI 17.7-not reached), and 18.3&#x2009;months (95% CI 13.3-22.5), with objective response rates of 30.8%, 66.7%, and 61.3%. Multivariate analysis did not identify PD-L1 CPS as a prognostic factor, whereas an Eastern Cooperative Oncology Group performance status &#x2265;&#x2009;1, a GEJ primary, and two or more metastatic sites were associated with poor prognosis. The PD-L1 CPS had no significant effect on prognosis in patients with unresectable advanced or recurrent gastric or GEJ cancer treated with first-line Nivo-CT.

Campylobacter jejuni is a common cause of gastro-enteritis, whereas bacteraemia is an infrequent and under-recognized manifestation. Initial standard culture conditions failed to yield growth, indicating possible diagnostic delays. Identification was achieved by incubating the culture plates in microaerophilic conditions and rapid identification by MALDI-TOF MS (Matrix-Assisted Laser Desorption/Ionization Time-of-Flight Mass Spectrometry).

CDH1 pathogenic variant carriers are at high lifetime risk of hereditary diffuse gastric cancer (HDGC). Endoscopic surveillance is recommended for individuals who delay risk-reducing total gastrectomy, although detection of signet ring cell carcinoma (SRCC) remains challenging. No Australian cohort has reported real-world endoscopic performance with surgical correlation in this population. We performed a 15-year retrospective cohort study of adult CDH1 carriers undergoing esophagogastroduodenoscopy (EGD) at a tertiary familial cancer centre (2008-2023). Patients presenting with symptomatic invasive gastric cancer at initial endoscopy were excluded. Most EGDs were conducted as preoperative assessments prior to risk-reducing total gastrectomy, with a minority undertaken as longitudinal surveillance. Endoscopic findings, biopsy performance, surgical pathology, and postoperative outcomes were analysed. Eighty-two CDH1 carriers from 31 families underwent 136 EGDs (median 1 per patient). SRCC was detected endoscopically in 38 patients (46.3%), predominantly via random biopsies. Overall sensitivity for SRCC detection was 67.9%, with random biopsies outperforming targeted sampling. Most lesions were identified at the initial EGD. Sixty-nine patients (84.1%) proceeded to total gastrectomy; SRCC was confirmed in 56 (81.2%), with variable tumour burden (1-73 foci). Invasive disease beyond pT1a was uncommon (2.9%). Postoperative complications occurred in 43.5%, most frequently anastomotic strictures requiring dilatation; there were no procedure-related deaths. Although occult SRCC was common, invasive malignancy beyond pT1a was infrequent, suggesting that many intramucosal lesions may have a more indolent course than previously assumed. Detection of SRCC at endoscopy remains challenging; however, these findings support a more individualized, risk-adapted approach to CDH1 management. Longer-term prospective data are required to better define SRCC progression risk and guide decisions regarding surveillance and risk-reducing surgery.

Primary gastrointestinal (GI) melanoma is an exceptionally rare and controversial diagnostic entity. Because the mucosa of the gallbladder, stomach, small bowel, and large bowel is not considered a common site of melanocytic proliferation, most GI melanomas are regarded as metastatic lesions originating from a primary cutaneous melanoma. Whether some of these tumors may truly arise primarily in GI sites remains a matter of debate. The diagnosis is further complicated by the possibility of occult, clinically subtle, or amelanotic cutaneous melanomas that may remain unrecognized until advanced metastatic disease becomes symptomatic. In addition, spontaneous regression of the primary cutaneous lesion may lead to complete or partial disappearance of the original tumor, creating further diagnostic uncertainty. We performed a literature review focused on GI and gallbladder melanoma, with emphasis on the distinction between primary and metastatic lesions and the role of spontaneous regression. In addition, we analyzed all cases of GI and gallbladder melanoma diagnosed in our department during the last five years. Three cases were identified: one gallbladder melanoma and two small bowel melanomas. These cases illustrated distinct clinical scenarios, including GI bleeding associated with gallbladder involvement, amelanotic ileal melanoma, and intussusception caused by metastatic melanoma from a known cutaneous primary lesion. Our findings support the need for cautious interpretation when diagnosing primary GI melanoma. Metastatic disease from occult, regressed, or previously undiagnosed cutaneous melanoma should always be carefully excluded through multidisciplinary clinicopathological evaluation.

Post-transplant immunosuppression increases malignancy risk. Gastric cancer is one of the most common post-transplant malignancies among high-risk groups including Asian patients. Helicobacter pylori is a known risk factor for gastric cancer; however, pre-transplant screening is not standard. This retrospective study assesses the frequency with which patients with end-stage liver disease, prior to liver transplant, are screened for H. pylori using endoscopic or non-endoscopic methods. We conducted a retrospective chart review of 127 patients who underwent upper endoscopy prior to liver transplant. Data were collected for age, ethnicity, indications for endoscopy, endoscopic findings, endoscopic treatment, gastric biopsy results, and antibiotic/proton pump inhibitor use. Non-endoscopic H. pylori testing within 5 years before transplant was recorded. Among 127 patients, 15 were of Asian and 9 were of Indigenous heritage. The most common indication for endoscopy was variceal surveillance (83 patients), then gastrointestinal bleeding (33 patients). Thirty-nine patients had biopsies taken for H. pylori, with four testing positive. Twenty patients had non-endoscopic testing, with three testing positive. Seventy-six patients (60%) underwent no form of testing. In Asian patients, 8 of 15 (53%) had no form of testing. Indigenous and Asian patients demonstrated high positivity rates, with 50% and 14% testing positive, respectively. H. pylori testing was performed in under 40% patients awaiting liver transplant. When tested, positivity was 10% by biopsies and 15% by other methods. Given high prevalence of H. pylori positivity, routine screening via endoscopic or non-endoscopic methods should be employed to mitigate post-transplant gastric malignancy risk. Liver transplant offers a life-saving treatment for many patients with advanced liver disease. However, patients who have received a liver transplant require long-term immunosuppressive medications. Such medications come with side effects that may increase a patient's susceptibility to infections and various cancers. Stomach cancer is one of the more common cancers after transplant, with higher incidences observed in certain ethnic groups, including Asian populations. Helicobacter pylori (H. pylori) is a well-established risk factor for stomach cancer. Currently, routine screening for this bacterium is not standard practice before liver transplant. Our study looked at patients who underwent liver transplantation over a 2-year period. We examined the frequency of testing, methods of testing, and infection rates of H. pylori in liver transplant recipients to better understand current screening practice. We identified that H. pylori infection rate was higher in Asian and Indigenous patients; however, screening remained underutilized despite these patients&#x2019; increased risks. Our findings highlight a potential gap in pre-transplant care and suggest that hospitals should consider including routine H. pylori screening as part of standard pre-transplant workup. Detection and early treatment of this infection may decrease the risk of stomach cancers and improve the long-term outcomes of people receiving liver transplants.

The Department of Gastroenterological Surgery at Kumamoto University has maintained a commitment to integrating cutting-edge clinical practice with fundamental research, particularly concerning malignant diseases of the digestive tract. This comprehensive review aimed to synthesize and consolidate the key clinical and translational research achievements published by our department, primarily focusing on gastric cancer (GC) and the pathophysiological mechanisms that drive its progression. We systematically reviewed the most impactful English-language original research papers from our institution, since 2005. The identified studies span critical areas including molecular carcinogenesis, tumor microenvironment (TME) components, and mechanisms of systemic metastasis and drug resistance, and so on. Our accumulated research has yielded significant insights into the molecular basis of GC. A core area of contribution includes the genetic and epigenetic changes during carcinogenesis of gastric cancer or Gastric Adenocarcinoma and Proximal Polyposis of the Stomach (GAPPS), the tumor microenvironment (TME) especially focusing on Cancer-Associated Fibroblasts (CAFs), a factor promoting peritoneal dissemination of ECM-related genes and gut microbiota. Furthermore, our translational work has explored mechanisms of resistance to chemotherapeutic agents. The collective research from our department represents a crucial contribution to the global understanding of gastric cancer pathogenesis, progression, and clinical management. These findings underscore our commitment to translating basic scientific discoveries into actionable strategies that advance individualized therapy, ultimately improving the long-term prognosis for patients with digestive malignancies.

Acute gastric distension (AGD) is a rare but life-threatening acute abdominal condition. Despite the rich blood supply to the stomach, extreme distension can lead to ischemic necrosis, perforation, and gas accumulation in the portal vein, with a mortality rate as high as 80-100%. We report the case of a 15-year-old male patient who presented with severe abdominal pain, bloating, and vomiting following a binge-eating episode. Physical examination revealed signs of diffuse peritonitis and shock (heart rate 154 beats per minute). Laboratory results showed severe metabolic acidosis (pH 7.265) and hyperlactatemia (11.3&#x202f;mmol/L). An abdominal CT scan confirmed massive gastric distension and gas accumulation in the portal vein, biliary tract, and esophageal wall. The patient underwent emergency laparotomy within 2&#x202f;hours of admission. Intraoperatively, the stomach was found to be distended into the pelvic cavity, with two areas of hemorrhagic necrosis: one on the lesser curvature (4&#x202f;cm&#x202f;&#xd7;&#x202f;5&#x202f;cm) and another on the posterior wall of the greater curvature (3&#x202f;cm&#x202f;&#xd7;&#x202f;4&#x202f;cm). Gastric decompression, debridement of the necrotic areas, and creation of a gastrostomy were performed. After 5&#x202f;days of intensive care unit (ICU) monitoring (for treatment of septic shock), the patient recovered well and was discharged on the 14th postoperative day; follow-up revealed no recurrence. This case demonstrates that even healthy individuals without eating disorders may develop AGD due to acute binge eating. Portal air is a critical warning sign of mucosal necrosis. Early diagnosis via CT and prompt surgical decompression are key to saving the lives of patients with ischemia and shock.

Pancreatic cancer (PC) is an aggressive malignancy with limited therapeutic options and generally poor responsiveness to immune checkpoint inhibitors (ICIs). Microsatellite instability-high (MSI-High) and tumor mutation burden-high (TMB-High) phenotypes are rare, each occurring in approximately 1% of PC cases, but may predict improved response to ICIs. We report a case of recurrent PC harboring both MSI-High and markedly elevated TMB, in which pembrolizumab achieved an exceptional and durable response. A 72-year-old man presented with appetite loss, and contrast-enhanced CT revealed a large pancreatic tail tumor invading the stomach, colon, spleen, and left kidney. Fine-needle biopsy confirmed adenocarcinoma. Despite initial modified FOLFIRINOX therapy showing progressive disease, subsequent gemcitabine plus nab-paclitaxel achieved partial response (PR). However, gastrointestinal bleeding due to gastric invasion necessitated surgery. The patient underwent extensive resection, including distal pancreatectomy with splenectomy, total gastrectomy, partial colectomy, nephrectomy, adrenalectomy, and portal vein resection and reconstruction using the left renal vein. Pathology demonstrated well-differentiated ductal adenocarcinoma with multiorgan invasion and portal vein tumor thrombus (ypT3N1M0, Stage IIB, Evans Grade IIb). Although adjuvant chemotherapy was considered, it could not be initiated because of persistent postoperative diarrhea and delayed recovery of the patient's general condition. Four months after surgery, recurrence was detected in the para-aortic lymph nodes, with suspected peritoneal dissemination causing right hydronephrosis, accompanied by elevated carbohydrate antigen 19-9 (CA19-9). Comprehensive genomic profiling using the surgically resected tumor specimen revealed MSI-High status and extremely high TMB (75 mutations/Mb), and genomic alterations in MSH2, MSH3, KRAS, TP53, ARID1A, ATR, and SMAD2. Pembrolizumab was initiated, leading to rapid normalization of CA19-9 and continuous tumor shrinkage. The best overall response was classified as PR, and this PR has been maintained. At 26 months after surgery, the patient remains on pembrolizumab with sustained clinical benefit and no significant immune-related adverse events. This case highlights the potential for exceptional and durable response to pembrolizumab in PC characterized by concurrent MSI-High and markedly elevated TMB. Given the rarity of these biomarkers and the typically poor prognosis of PCs, early genomic profiling may facilitate personalized treatment strategies and improve outcomes in selected patients.