Clinical Journal of Gastroenterology最新文献

Acinar cell carcinoma of the pancreas is a rare malignancy characterized by lipase secretion and a high propensity for distant metastases, most commonly to the liver. Although primary hepatic acinar cell carcinoma has been reported, its existence remains controversial due to limited pathological confirmation. We report the case of a 66-year-old male who presented with sudden right upper quadrant pain and elevated serum lipase. Imaging revealed multiple hepatic tumors, with no evidence of a primary tumor in the pancreas. Histopathological examination of a liver biopsy confirmed acinar cell carcinoma. On this basis, the patient was clinically diagnosed with primary hepatic acinar cell carcinoma in the absence of radiological evidence of pancreatic disease. However, autopsy revealed a 5-mm acinar cell carcinoma in the pancreatic head. Additionally, an incidental intraductal papillary mucinous carcinoma was identified in the pancreatic tail. This case demonstrates that even minute pancreatic acinar cell carcinomas can result in extensive hepatic metastases and may be misdiagnosed as primary hepatic acinar cell carcinoma. Furthermore, the coexistence of acinar cell carcinoma with intraductal papillary mucinous carcinoma represents a novel finding. These observations underscore the importance of autopsy and comprehensive pathological evaluation in establishing accurate diagnoses of rare pancreatic neoplasms.

Standard treatment for metastatic biliary tract cancer is systemic chemotherapy, not local therapy, such as surgery or radiation therapy. This is because long-term survival is not expected with local therapy. We present the case of a 49-year-old man who underwent extended right hepatectomy with extrahepatic bile duct resection for hilar cholangiocarcinoma. Postoperative adjuvant chemotherapy with oral S-1 for six months was completed. At 20 months post-operation, para-aortic lymphadenopathy was detected, and endoscopic ultrasound-guided biopsy confirmed lymph node recurrence. Gemcitabine and cisplatin were administered for six months, resulting in partial tumor regression. As no other lesions were identified, radiotherapy with a total dose of 55 Gy in 22 fractions was performed. At 37 months following the completion of radiotherapy, the patient remained disease-free based on imaging. This case suggests that the addition of local radiotherapy to systemic chemotherapy may be beneficial for patients with oligometastatic recurrence of hilar cholangiocarcinoma.

Cavernous transformation of the portal vein is a rare condition that is often misdiagnosed or diagnosed late due to its insidious onset and nonspecific clinical presentation. It is most often associated with prothrombotic conditions, liver disease, malignancy, or local inflammation. We report a rare case involving a 65‑year‑old woman with HIV infection (CD4 count 96 cells/µL) who developed portal vein cavernous transformation leading to obstructive biliopathy. Cholangioscopy excluded intrinsic mucosal pathology or neoplasm, and additional testing ruled out other hypercoagulable disorders. Following placement of a biliary stent, her cholestatic symptoms resolved and her alkaline phosphatase and bilirubin levels declined. This case highlights HIV as an unusual precipitant of portal vein cavernous transformation and discusses imaging findings, underlying etiologies, and other management options.

A 43-year-old man with a family history of gastric cancer had undergone endoscopic submucosal dissection (ESD) for a signet-ring cell carcinoma (SRCC) lesion. Five years later, a recurrent SRCC lesion was treated with a second ESD. Two months later, multiple recurrent SRCC lesions were again detected. Immunohistochemical staining revealed reduced E-cadherin expression. Genetic testing for CDH1 identified an R732Q missense mutation. The patient subsequently underwent total gastrectomy, which identified 22 intramucosal SRCC lesions. Notably, the most distal carcinoma was located in the duodenal bulb and appeared to arise from mucosa containing gastric fundic glands, gastric foveolar epithelium, CD10-positive cells, MUC2-positive cells, and Brunner's glands, suggesting origin from ectopic gastric mucosa in the duodenum. To our knowledge, this is the first reported case of SRCC arising from ectopic gastric mucosa in patients with hereditary diffuse gastric cancer. Screening for ectopic gastric mucosa may be warranted in patients with CDH1 mutations.

Eosinophilic cholangitis is a rare condition characterized by the infiltration of the bile duct wall by inflammatory cells, predominantly eosinophils, leading to bile duct wall thickening, narrowing, and dilatation. Differentiation between primary sclerosing cholangitis and cholangiocarcinoma remains challenging owing to overlapping clinical and radiological features, and no standardized diagnostic criteria currently exist. We report a case of eosinophilic cholangitis presenting as a localized biliary stricture in a 65 year-old man. The patient underwent computed tomography for the evaluation of worsening diabetes, which incidentally revealed a stricture at the hepatic hilum. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) demonstrated a hilar biliary stricture with left intrahepatic bile duct dilatation. Bile cytology was interpreted as atypical, brush cytology was negative for malignancy, and bile duct biopsy showed no evidence of malignancy. Transoral cholangioscopy also revealed no malignant features; however, follow-up ERCP showed progression of the right-sided biliary stricture. As hilar cholangiocarcinoma could not be ruled out, the patient opted for surgical intervention. A left hepatectomy with caudate lobectomy was performed. Histopathological examination revealed no malignancy but showed submucosal fibrosis and inflammatory cell infiltration. Notably, marked eosinophilic infiltration was observed at the site of the right biliary stricture. The final diagnosis of the patient was eosinophilic cholangitis.

Background: Penile metastasis represents one of the rarest metastatic sites despite the organ's rich vascular network. Most reported cases originate from malignancies of the genitourinary tract, whereas metastasis from pancreatic ductal adenocarcinoma (PDAC) is exceedingly uncommon. Primary tumors responsible for penile metastases are typically located in the prostate or rectum and are frequently associated with disseminated disease and poor prognosis.

Case presentation: An 86-year-old man presenting with abdominal pain underwent distal pancreatectomy with regional lymphadenectomy for PDAC. Three months after surgery, computed tomography revealed multiple liver metastases, para-aortic lymph node involvement, and peritoneal dissemination. Subsequently, the patient developed penile pain and persistent erection. Physical examination revealed multiple whitish indurated nodules on the glans penis. Ultrasonography demonstrated a hypoechoic mass, and a penile biopsy confirmed metastatic adenocarcinoma consistent with the primary pancreatic lesion based on morphological features and KRT7/20 immunoprofiles. The patient's condition progressively deteriorated, and he died five months after surgery.

Conclusion: Penile metastasis from PDAC is an extremely rare event and portends a dismal prognosis. Including our patient, only six cases have been documented to date. Awareness of this manifestation is important, as penile symptoms such as pain or persistent erection in patients with PDAC may indicate advanced systemic dissemination.

Background: Primary splenic angiosarcoma is a rare aggressive vascular neoplasm. Although hepatic metastases are common, diffuse intrasinusoidal hepatic involvement is extremely rare and poses a significant diagnostic challenge.

Case report: A 60-year-old woman presented with bilateral lower extremity edema, hypoalbuminemia, and thrombocytopenia. Contrast-enhanced computed tomography revealed marked splenomegaly, but no distinct hepatic lesions. Gadoxetic acid-enhanced magnetic resonance imaging revealed reduced hepatic uptake without focal abnormalities. ¹⁸F-fluorodeoxyglucose positron emission tomography-computed tomography revealed diffuse radiotracer uptake in both the liver and spleen, raising the suspicion of an infiltrative malignant process, such as lymphoma. Liver biopsy revealed sinusoidal infiltration by atypical cells positive for cluster of differentiation 31 and ETS-related gene, but lacked architectural features diagnostic of angiosarcoma. Due to diagnostic uncertainty and concerns regarding splenic rupture, splenectomy was performed. Histopathological examination confirmed a primary splenic angiosarcoma with diffuse intrasinusoidal hepatic metastasis.

Conclusion: Primary splenic angiosarcoma rarely presents with non-nodular intrasinusoidal hepatic metastasis, mimicking infiltrative liver diseases. Liver biopsy may fail to yield a definitive diagnosis. Therefore, a splenectomy may be required to establish a definitive diagnosis and guiding appropriate management when liver biopsy is inconclusive. Recognizing this uncommon metastatic pattern is crucial to avoid misdiagnosis and ensure timely treatment.

The patient, a man in his 40 s, underwent total colectomy 14 years after the onset of refractory ulcerative colitis. One year postoperatively, he developed recurrent pouchitis. At admission of the patient to the hospital with anorexia and vomiting, acute kidney injury was noted. After admission, the patient was diagnosed with pouchitis and secondary to ileal pouch dysfunction. After administration of ciprofloxacin (800 mg/day), the patient's symptoms improved temporarily, but the disease relapsed shortly thereafter. Although he resumed oral intake 9 days after the relapse, vomiting occurred the following day. Abdominal computed tomography revealed fluid retention within the gastrointestinal tract. As a result of prolonged fluid retention in the gastrointestinal tract and impaired absorption because of intestinal edema, the patient remained in hypovolemic shock. Cardiopulmonary arrest occurred the day after the relapse. Systemic status and response to therapy must be evaluated carefully in severe cases of pouchitis with unstable general condition. Such evaluation can indicate appropriate timing of surgical intervention, including stoma reconstruction, instead of persistence with conservative treatments using modalities such as antimicrobial agents. Early recognition of risks of severe disease and prompt intervention are crucially important for improving clinical outcomes.

Although immune checkpoint inhibitors have demonstrated efficacy in advanced gastric cancer, reports of long-term complete remission in early-stage disease are lacking. We report a case of a 75-year-old man who presented with concurrent malignant melanoma and early gastric cancer (cT1bN0M0). Following surgical excision of melanoma with positive sentinel lymph node biopsy (T3bN1M0 Stage III), we prioritized treatment of advanced melanoma over early gastric cancer and initiated nivolumab adjuvant therapy every 2 weeks. After six cycles, treatment was discontinued due to drug-induced pneumonitis with elevated KL-6 levels. Repeat endoscopy performed 2 months after initial examination revealed partial tumor regression, with complete disappearance of the gastric lesion confirmed 4 months later. Histopathological examination showed no malignant findings. The patient achieved complete response of gastric cancer with only six cycles of nivolumab, despite negative PD-L1 expression (combined positive score 0%). During 6 years of follow-up, endoscopic and CT examinations consistently showed no gastric cancer recurrence, maintaining complete response. This case demonstrates sustained long-term efficacy of nivolumab against early gastric cancer, achieving 6-year disease-free survival with minimal treatment exposure. These findings suggest that immune checkpoint inhibitors may represent potential treatment options for patients with early gastric cancer contraindicated for surgery, even in PD-L1-negative cases.