Clinical Journal of Gastroenterology最新文献

Neurofibromatosis type 1 (NF1) is associated with an increased risk of gastrointestinal stromal tumors (GISTs). Vasculopathy, such as aneurysms, is a recognized complication of NF1; however, it is little known among gastroenterologists. We report a 65-year-old woman with NF1 who developed hemorrhagic shock due to rupture of an intercostal artery aneurysm during sunitinib therapy. The patient underwent distal gastrectomy for duodenal GIST at age 49, but later developed hepatic and peritoneal recurrence. Because of failure of imatinib therapy and multiple transarterial embolization procedures, sunitinib therapy was initiated, stabilizing the disease for nine months. During treatment, she developed acute back pain with severe hypertension (220/106 mmHg) and was immediately admitted, where intravenous nicardipine was administered. Despite initial stabilization, the patient abruptly went into shock the following day. Dynamic computed tomography (CT) revealed a massive right hemothorax, posterior mediastinal hematoma, and contrast extravasation from a right intercostal artery aneurysm. Emergency angiography confirmed rupture of the ninth intercostal artery aneurysm measuring 18 mm. Embolization with microcoils achieved complete hemostasis. The patient recovered without sequelae and was discharged on day 34. Through a literature review, we identified 16 cases of intercostal artery aneurysm rupture in NF1. Of these, 11 were treated with embolization, although two experienced rebleeding. Sunitinib inhibits vascular endothelial growth factor signaling, which can induce hypertension and destabilize vascular integrity. Sunitinib therapy in NF1 patients requires strict blood pressure control. Additionally, pre-treatment vascular screening with CT angiography may be warranted.

Recurrence of intraductal papillary mucinous neoplasms (IPMNs) in the remnant pancreas after surgery is a significant clinical challenge. A 68-year-old woman was incidentally found to have a 50-mm mixed-type intraductal papillary mucinous carcinoma (IPMC) in the pancreatic head during a health check. She underwent subtotal stomach-preserving pancreaticoduodenectomy with pancreatogastrostomy. Pathology revealed intestinal-type IPMC (pStage IA) with venous invasion and negative margins. Adjuvant oral S-1 chemotherapy was administered for six months. Eighteen months later, she developed rapid increases in HbA1c and tumor markers. Computed tomography showed 8-mm main pancreatic duct dilation in the remnant pancreas. Upper gastrointestinal endoscopy revealed a papillary tumor extending into the gastric mucosa, and biopsy confirmed adenocarcinoma. She was diagnosed with recurrent IPMC in the remnant pancreas and underwent total pancreatectomy with splenectomy. Intraoperative gastric endoscopy determined the extent of gastric wall resection. Postoperative chylous leakage occurred but resolved, and she was discharged on day 26. Pathology revealed non-invasive gastric-type IPMC, distinct from the intestinal-type lesion in the initial surgery. This case represents a rare metachronous recurrence of gastric-type IPMC following resection of intestinal-type IPMC, emphasizing the need for vigilant long-term surveillance and awareness of potential histologic subtype changes.

Plexiform fibromyxoma (PF) is a rare gastric submucosal tumor that presents diagnostic challenges due to its nonspecific clinical and imaging findings. Preoperative diagnosis is difficult, and optimal management remains unclear. We report the successful diagnosis and resection of PF using laparoscopic endoscopic cooperative surgery (LECS), a minimally invasive approach that preserves gastric function. An 81-year-old woman with a known gastric submucosal tumor was referred after endoscopy showed tumor enlargement and central depression. Endoscopic ultrasound revealed a hypoechoic mass originating from the second layer of the gastric wall. Bite-on-bite biopsies of the delle identified spindle cells embedded in a mucinous stroma. Immunohistochemistry showed positivity for alpha-smooth muscle actin and negativity for c-KIT, CD34, S100, and desmin, confirming PF. Given the increase in size, central depression, and associated anemia, surgical resection was indicated. The patient underwent LECS, which allowed for complete resection with minimal margins and preservation of gastric anatomy. The procedure was uneventful, and recovery was smooth. Histopathological findings confirmed the diagnosis of PF. This is the first reported case of PF resected by planned LECS following preoperative diagnosis by bite-on-bite biopsy. LECS offers a safe and effective option for managing PF and other submucosal tumors in anatomically sensitive locations.

A 59-year-old man underwent computed tomography (CT) for evaluation of abdominal pain, which revealed a pancreatic tumor. The patient was subsequently referred to our hospital for further evaluation. Contrast-enhanced CT revealed a 22-mm lesion in the pancreatic tail with a contrast effect extending from the arterial phase. In addition, a 20-mm lesion with slightly less contrast enhancement was observed on the head side of the lesion. Endoscopic ultrasound-guided fine-needle tissue acquisition was performed on the lesion in the pancreatic tail, leading to the diagnosis of pancreatic acinar cell carcinoma, and distal pancreatectomy was performed. Preoperative imaging suggested the presence of two separate lesions; however, the resected specimen showed that the lesions had formed a single mass owing to their extension into the main pancreatic duct. The poorly enhanced region on the head side was primarily composed of edema and inflammatory changes with few tumor cells, whereas the enhanced region on the tail side consisted mostly of tumor cells. This difference in composition was thought to account for the variation in contrast enhancement. Awareness of imaging differences combined with histopathological correlations may aid in biopsy site selection and provide value in routine clinical practice.

Phlegmonous gastritis is a rare, potentially life-threatening bacterial infection affecting the gastric mucosa and submucosa. Clinical presentation is often non-specific, typically including symptoms such as fever, epigastric vomiting, or hematemesis. Due to its rarity and vague clinical features, diagnosis is frequently delayed, increasing the risk of severe complications such as septic shock and death. We describe the case of a 74-year-old previously healthy female who presented with vomiting, hematemesis, and epigastric pain. Endoscopy revealed diffuse erythema and purulent exudate throughout the stomach, raising suspicion for Phlegmonous gastritis. Although blood and gastric juice cultures were negative, gastric tissue culture identified Cronobacter species. Cronobacter species is an opportunistic pathogen primarily associated with severe infections in neonates, though it has also been reported in immunocompromised adults. The patient responded well to targeted antibiotic therapy and recovered without requiring surgery. This case highlights the diagnostic and therapeutic utility of gastric tissue culture in Phlegmonous gastritis, especially when conventional cultures fail to identify a pathogen. It also underscores the role of Cronobacter species as a rare but relevant pathogen in adult gastrointestinal infections such as Phlegmonous gastritis.

Gallbladder amyloidosis is an exceptionally rare condition that may clinically mimic extrahepatic cholestasis. An 81-year-old man presented with persistent cholestatic jaundice and recurrent right upper quadrant pain. Laboratory evaluation showed conjugated hyperbilirubinemia and elevated cholestatic liver enzymes without signs of inflammation. Imaging studies, including ultrasound, MRCP, and CT, demonstrated gallstones and gallbladder wall thickening but no bile duct dilation or obstruction. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound were unremarkable. Because of ongoing pain and cholestasis, laparoscopic cholecystectomy was performed. Histopathological examination revealed chronic fibrosing cholecystitis with marked submucosal and vascular deposition of amorphous eosinophilic material, showing apple-green birefringence under polarized light after Congo red staining, consistent with amyloid. Immunohistochemistry favored transthyretin (ATTR) amyloid deposition. Postoperatively, the patient recovered uneventfully with resolution of pruritus and normalization of bilirubin. Extensive systemic evaluation excluded generalized amyloidosis, indicating a localized form of gallbladder amyloidosis. This case underscores the importance of considering infiltrative diseases such as amyloidosis in the differential diagnosis of unexplained cholestatic jaundice, particularly when imaging fails to show mechanical obstruction. Recognition of this entity may prevent unnecessary invasive interventions and emphasizes the diagnostic value of histopathological confirmation following cholecystectomy.

We present a case of cytomegalovirus (CMV)-associated rectal ulceration that developed as a secondary complication of immune checkpoint inhibitor-induced colitis. A 74-year-old man who received pembrolizumab for metastatic squamous cell carcinoma of the lung developed severe immune-related colitis and required high-dose corticosteroid therapy. Although his condition initially improved, his symptoms recurred approximately four weeks after the initiation of steroid tapering. Colonoscopy revealed a circumferential, band-shaped ulcer located just above the dentate line, and a histopathological examination demonstrated nuclear inclusion bodies suggestive of CMV infection. The diagnosis was confirmed by immunohistochemical staining. Treatment with valganciclovir resulted in complete clinical and virological remission within four weeks. This case highlights the need for monitoring opportunistic infections during immunosuppressive therapy for immune-related adverse events (irAEs). It also underscores the importance of early recognition and prompt intervention for clinical deterioration.

Background: Large cell neuroendocrine carcinoma (LCNEC) arising from the extrahepatic bile duct is a highly uncommon and aggressive malignancy. Due to the absence of an established standard and a lack of detailed reports on effective chemotherapy for recurrent biliary LCNEC, we present a case that demonstrated a temporary response to cisplatin and etoposide.

Case presentation: A Japanese man in his 70 s presented with jaundice and weight loss. Imaging studies identified a tumor in the distal bile duct, and a biopsy of the bile duct confirmed a neuroendocrine tumor. The patient underwent a radical pancreaticoduodenectomy with regional lymph node dissection. Histopathology confirmed LCNEC with a Ki-67 index of 60% and metastases in the regional lymph nodes. Despite the initial surgical treatment, multiple liver metastases appeared 3 months after surgery. Systemic chemotherapy using cisplatin and etoposide was initiated and produced a short-term partial response. However, after six cycles, the disease progressed, and subsequent therapy with amrubicin was ineffective. The patient passed away 14 months after the operation.

Conclusions: This case underscores the aggressive behavior of biliary LCNEC and the limited success of current treatments. To our knowledge, this is the first reported case of biliary LCNEC with recurrent liver metastases demonstrating a radiologically confirmed partial response to cisplatin and etoposide therapy. A review of previously published cases revealed comparable outcomes, underscoring the need for further investigation to develop effective therapeutic strategies for LCNEC of the extrahepatic bile duct.

Extraintestinal manifestations occur in up to 50% of patients with inflammatory bowel disease and can affect various organs, including the joints, skin, and eyes. Scleritis, one such extraintestinal manifestation, is associated with severe eye pain, vision impairment, and potential permanent vision loss. However, it is rare, and detailed descriptions of its characteristics and management are limited. We report the case of a 55-year-old man initially diagnosed with proctitis ulcerative colitis who simultaneously developed scleritis as the disease progressed to left-sided colitis. Treatment with 5-aminosalicylic acid, systemic steroids, and thiopurines failed to prevent a gradual decline in vision. Neither tacrolimus nor infliximab induced remission of ulcerative colitis or scleritis. Ultimately, total proctocolectomy was performed to treat refractory colitis. After surgery, the scleritis resolved immediately. Remission has persisted for over 10 years without recurrence, despite discontinuation of all medications (including ophthalmic solutions). This is the first documented case of severe, intractable scleritis resolving after proctocolectomy, suggesting an intestinal-ocular interaction in the pathogenesis of ulcerative colitis.