Erdheim-Chester Disease with Pancreatic Enlargement Observed Using Contrast-enhanced Endoscopic Ultrasonography.

We herein report an unusual case of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, and emphasize its unique presentation and diagnostic challenges. Our patient exhibited uncommon symptoms and significant organ involvement, particularly pancreatic enlargement that is not typically associated with ECD. Contrast-enhanced harmonic endoscopic ultrasonography (CEH-EUS) and EUS-fine needle aspiration (EUS-FNA) play crucial roles in the comprehensive assessment of the disease, demonstrating their superiority in identifying and characterizing elusive ECD lesions. This is the first report to document pancreatic lesions in patients with ECD evaluated using CEH-EUS. EUS-FNA is valuable for diagnosing rare diseases, including ECD, with diffuse pancreatic enlargement.

Transarterial embolization of radicular arteriovenous fistula at the craniocervical junction.

Craniocervical junction arteriovenous fistula (CCJ AVF) is a rare vascular disorder. Direct surgery for CCJ AVF is generally reported to have better outcome compared to endovascular treatment. However, no certain consensus has been obtained so far. We report a case of radicular CCJ AVF treated by transarterial embolization that resulted in a good outcome. A 69-year-old man presented with subarachnoid hemorrhage primarily in the posterior cranial fossa. Based on digital subtraction angiography showed radicular CCJ AVF with varix. Transarterial embolization was performed with n-butyl-2-cyanoacrylate on day 17 after onset and successfully cured. The neurovascular anatomy of CCJ AVF is complicated, but endovascular treatment may be a treatment option with detailed understanding of angioarchitecture and selective endovascular procedure.

[Pre-surgical diagnosis of neuroendocrine carcinoma of the extrahepatic bile duct: a case study].

A 70-year-old woman presented to our hospital with jaundice. Abdominal ultrasonography showed biliary duct dilatation. Blood tests revealed elevated total bilirubin and hepatobiliary enzyme levels. A contrast-enhanced computed tomography of the abdomen showed bile duct thickening with wall enhancement. Transpapillary bile duct biopsy showed an invasive carcinoma proliferating in a follicular pattern. Pathology revealed positive synaptophysin and chromogranin A and a Ki67 index >40%, consistent with a diagnosis of neuroendocrine carcinoma (NEC). After confirming the absence of distant metastases, a subtotal stomach-preserving pancreaticoduodenectomy was performed. The result of the postoperative pathology was the same as the preoperative biopsy. According to previous reports, 7 out of 28 cases with NEC/mixed adenoneuroendocrine carcinoma could be diagnosed as NEC before surgery. However, biliary cytology and bile duct scraping cytology were used in many cases;only 11 cases underwent bile duct biopsy. For the latter, 5 out of 11 cases could be diagnosed preoperatively. NEC of the extrahepatic duct often exhibits a submucosal tumor-like morphology, which may result in a false negative result with biliary cytology or bile duct scraping cytology. In our case, the transpapillary bile duct biopsy sample was sufficient to diagnose NEC. This method could be an attractive option for the diagnosis of these tumors.

Surgical management of brain metastasis as a part of systematic metastases from adenoid cystic carcinoma of the external auditory canal: illustrative case.

BACKGROUND: Adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) is a rare tumor that accounts for approximately 5% of all EAC tumors. ACC is generally known as a slow-growing tumor, but patients often experience recurrence or distant metastasis in the long clinical course. While the major pattern of recurrence is pulmonary metastasis, brain metastasis of ACC of the EAC is rare. OBSERVATIONS: The authors describe the case of a 72-year-old male who was diagnosed with ACC of the EAC. Approximately 7 years later, brain magnetic resonance imaging revealed an intra-axial homogenously enhancing mass lesion that had no direct connection with the skull base in the left frontal lobe. The patient underwent tumor resection and histopathological examination revealed a mixture of cribriform and tubular patterns. The image and pathological characteristics of the tumor were similar to those of primary ACC or ACC from other sites of origin. LESSONS: While patients with ACC of the EAC often experience recurrence or distant metastasis in the long clinical course, they survive for a relatively long period of time, even though an optimal treatment has not been established. The authors therefore recommend surgical resection for brain metastasis of ACC of the EAC to improve neurological symptoms.

A rare cause of acute kidney injury with chronic myelomonocytic leukemia.

An 80-year-old man with myelodysplastic syndrome developed acute kidney injury (AKI) and peripheral blood monocyte-dominant leukocytosis. Glomerular disease was suspected from urinalysis, which showed proteinuria and microscopic hematuria with red cell casts. Eventually, he died of respiratory failure, after which a postmortem was performed. In the glomeruli, the extracapillary space was filled with numerous mononuclear cells and erythrocytes. At first interpretation, the glomerular findings appeared to represent cellular crescents. However, immunostaining revealed that the extracapillary mononuclear cells were in fact leukemic cells. Furthermore, tubular injury due to marked accumulation of lysozyme was also recognized together with infiltration of leukemic cells in the interstitium. The diagnosis of kidney infiltration by chronic myelomonocytic leukemia (CMML) and lysozyme-induced tubular injury was eventually made. Our case is the first report showing extracapillary infiltration of leukemic cells by immunostaining. In addition, lysozyme-induced tubular injury is a forgotten cause of kidney injury in patients with CMML. This case teaches us the rare and forgotten causes of AKI with CMML.

Treatment Strategies for Basilar Top Syndrome Caused by Acute Vertebral Artery Occlusion.

Objective: We report a patient with basilar artery embolism caused by vertebral artery stenosis who was successfully treated using simultaneous percutaneous transluminal angioplasty (PTA) and mechanical thrombectomy. Case Presentation: A 64-year-old male, who had undergone medical treatment for cerebellum infarction at another hospital, was referred to our hospital due to disturbance of consciousness. Angiography revealed acute occlusion of the first part of the right vertebral artery and an embolism of the top of basilar artery. After performing PTA to create an approach route for the embolism, we collected it using a clot recovering device. The postoperative course was good, and the patient was discharged with mild ataxia and dysarthria. Conclusion: We report the successful treatment of progressive cerebral infarction of the posterior circulation with revascularization 30 hours after symptom onset. Unlike the anterior circulation, the posterior circulation consists of smaller arteries and fewer collateral arteries, making it vulnerable to ischemic attack. Therefore, shortening the time until treatment may improve the outcome.

Evaluation of a new 2-paste glass ionomer cement.

A new 2-paste resin-reinforced glass ionomer cement, Fuji Ortho Band Paste Pak (GC Corporation, Tokyo, Japan), for the placement of orthodontic bands, has been developed for easier handling. The aim of this study was to compare the fluoride release and uptake characteristics of this cement with that of 3 others commonly used to cement orthodontic bands: a conventional resin-reinforced glass ionomer cement, a polyacid-modified composite resin, and a conventional glass ionomer cement. Fluoride release was measured during a 28-day period. After the measurement on day 28, experimental samples were exposed to 1000 ppm sodium fluoride solution for 5 minutes, and fluoride release was then measured for 7 days. Initially, the new 2-paste resin-reinforced glass ionomer cement released the greatest amount of fluoride; the polyacid-modified composite resin released the least initially, and it continued to show the lowest values throughout the study. The fluoride uptake and release values of the new 2-paste resin-reinforced glass ionomer cement were statistically significantly higher than those of the conventional resin-reinforced glass ionomer cement or the conventional glass ionomer cement. The new 2-paste resin-reinforced glass ionomer cement might be a good alternative to conventional products for cementing orthodontic bands.