Yuta Takahashi

Early use of allogeneic hematopoietic stem cell transplantation for infants with MLL gene-rearrangement-positive acute lymphoblastic leukemia

Sixty-two infants with MLL gene-rearrangement-positive acute lymphoblastic leukemia (MLL-r ALL) were treated with the MLL03 protocol of the Japanese Pediatric Leukemia/Lymphoma Study Group: short-course intensive chemotherapy followed by early allogeneic hematopoietic stem cell transplantation (HSCT) within 4 months of the initial induction. The 4-year event-free survival and overall survival rates were 43.2% (95% confidence interval (CI)=30.7–55.1%) and 67.2% (53.8–77.4%), respectively. A univariate analysis showed younger age (<90 days at diagnosis), central nervous system disease and poor response to initial prednisolone therapy significantly associated with poor prognosis (P<0.05). In a multivariate analysis, younger age at diagnosis tended to be associated with poor outcome (hazard ratio=1.969; 95% CI=0.903–4.291; P=0.088). Although the strategy of early use of HSCT effectively prevented early relapse and was feasible for infants with MLL-r ALL, the fact that substantial number of patients still relapsed even though transplanted in their first remission indicates the limited efficacy of allogeneic HSCT for infants with MLL-r ALL. Considering the risk of severe late effects, indications for HSCT should be restricted to specific subgroups with poor risk factors. An alternative approach incorporating molecular-targeted drugs should be established.

Pancreatobiliary drainage using the EUS-FNA technique: EUS-BD and EUS-PD

The recent progression of endoscopic ultrasonography (EUS) enables EUS-guided transmural drainage based on the EUS-guided fine-needle aspiration biopsy technique. Prior to the development of EUS-guided drainage procedures, the options for treating obstruction of the pancreatobiliary system included surgical drainage, percutaneous drainage using ultrasound and radiological guidance, and endoscopic (non EUS-guidance) transmural drainage. Today, using EUS guidance and dedicated accessories, it is possible to create bilio- or pancreato-digestive anastomosis, EUS-guided biliary drainage (EUS-BD), and EUS-guided pancreatic drainage (EUS-PD). The recent literature describes that EUS-BD and EUS-PD have acceptable success and complication rates. These procedures are anticipated for use as alternatives to surgery or percutaneous drainage when endoscopic transpapillary procedures fail.

Two Cases of Retroperitoneal Liposarcoma Diagnosed Using Endoscopic Ultrasound-Guided Fine-Needle Aspiration (EUS-FNA)

This report describes our experience with two cases that were ultimately diagnosed as retroperitoneal liposarcoma using endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). Case 1 is that of a 54-year-old woman with chief complaints of nausea and abdominal distention. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large (15 cm diameter) tumor, which was significantly compressing the stomach and apparently occupied the entire left abdominal cavity. Although advanced primary gastrointestinal stromal tumor (GIST) or retroperitoneal tumor was inferred as the differential diagnosis, a definitive diagnosis was difficult using imaging alone. After EUS-FNA was done, the tumor was diagnosed histopathologically as high-grade liposarcoma. Case 2 is that of a 73-year-old man. Abdominal ultrasonography and CT showed a 6 cm diameter tumor within the pelvic cavity. The tumor had high MRI signal-intensity on both T1 and T2 images. Endorectal EUS showed a hyperechoic mass. The images suggested lipoma or liposarcoma containing lipoma-like components. Myxoid liposarcoma was revealed by subsequent EUS-FNA. Performing EUS-FNA was clinically useful for determining the subsequent therapeutic strategy in these cases where a tumor of unknown origin existed in the retroperitoneum.

Cyst-like extension of hepatic subcapsular bleeding caused by ruptured hepatocellular carcinoma into the bursa omentalis.

An 81‐year‐old man, who experienced upper abdominal pain after shoveling snow, was admitted to a local hospital where a computed tomography (CT) showed a cystic lesion adjoining the pancreas. He was transferred to our department for detailed investigations and treatment. On ultrasonography, a tumor of the caudate lobe of the liver, with which the cystic lesion was continuous, was seen. The tumor of the caudate lobe of the liver was enhanced in the early phase of the CT but was washed out in the delayed phase. Subsequently, T1‐weighted and T2‐weighted magnetic resonance imaging (MRI) images showed a low intensity and a high intensity, respectively. Because the cystic lesion was continuous with the tumor of the caudate lobe of the liver, its CT value was higher than that of water, and both the T1‐weighted and T2‐weighted MRI images showed a high intensity, which was attributed to a hematoma. Examination of the image suggested that rupture of a hepatocellular carcinoma (HCC) might have caused intracavitary hemorrhage. After the HCC was treated by transcatheter arterial embolization therapy, the patient was discharged. Subsequently, tumor enlargement was confirmed, and surgical removal of the tumor was conducted at the hospital where the patient had originally presented. On histology, moderately differentiated HCC was diagnosed, but the cyst‐like lesion was confirmed to be a hepatic subcapsular hematoma extending into the bursa omentalis. Although ruptured HCC often causes intraperitoneal bleeding, this rare case showed a cyst‐like imaging finding in the form of a subcapsular hematoma within the bursa omentalis.

Analysis of prognostic factors in patients with gastric varices after endoscopic treatment.

Background:  The prognostic factors, including gastric variceal bleeding itself, in patients with gastric varices (GV) after endoscopic treatment remain unclear. The aim of this study was to analyze prognostic factors in patients with GV after endoscopic treatment as well as to evaluate safety and efficacy of our endoscopic treatment.

Chronic intestinal pseudoobstruction ileus in a patient with rheumatoid arthritis: outcome of a stoma construction and its histopathological features

We report the first case of a chronic intestinal pseudoobstruction (CIPO) in a patient with rheumatoid arthritis (RA) in whom a stoma was constructed at the sigmoid colon to decompress intracolonic pressure. The treatment improved quality of life in this patient, suggesting that surgical resection of the colon to decompress intracolonic pressure would be one of the options for the treatment of intractable CIPO. The resected specimen obtained from the sigmoid colon was found to be palisaded, with nuclei crowded in some areas of the inner circular muscle layer and thickened, with zonal or focal myxoid regions with interstitial fibrosis, composed of collagenous and elastic fibers, in the outer longitudinal muscle layer. Some of the smooth muscle cells in the outer longitudinal muscle layer showed mild degeneration with cytoplasmic vacuolar changes, but no inflammatory changes. However, further studies are required to determine whether these histopathological changes are unique to RA.

Endosonographic features of solitary gastric amyloidosis

Although gastrointestinal involvement in cases of systemic amyloidosis is very common, primary and solitary amyloid tumors in the gastrointestinal tract, especially in the stomach, are rare. 1-5 Various changes in endoscopic findings due to the deposition of amyloid proteins in the gastrointestinal tract have been evaluated in comparison with histopathologic findings of amyloid deposition. 6-8 Endoscopic ultrasonographic (EUS) images of amyloidosis have also been investigated, but EUS features of amyloidosis have been poorly characterized in a few published reports. 2,4,5,9 Here we report a case of solitary amyloidosis of the stomach that was observed on EUS images, and we review the literature with regard to EUS features of amyloidosis in the gastrointestinal tract.

Hemorrhagic duodenal varices treated successfully with endoscopic injection sclerotherapy using cyanoacrylate and ethanolamine-oleate: a case report.

We report a case of a 50-year-old man with a medical history of alcoholic cirrhosis, in addition to esophagogastric and duodenal varices (DV), who was transferred to our institution because of hemorrhagic DV. Emergent esophagogastroduodenoscopy showed hemorrhagic varices in the horizontal portion of the duodenum. Abdominal contrast-enhanced CT showed hemodynamics of DV derived from anastomosis between the superior mesenteric vein and right renal vein. Cyanoacrylate was injected into the DV. Subsequently, 5% ethanolamine-oleate was injected endoscopically as a sclerosant into the DV feeding vein. Radiographic fluoroscopic findings revealed that the injected cyanoacrylate and sclerosant remained, respectively, in the varices and its feeder. Five days later, CT showed that the injected cyanoacrylate occupied the DV, and thrombus formation of the afferent vein led to bifurcation of superior mesenteric vein. This case showed the usefulness of endoscopic injection sclerotherapy using cyanoacrylate and sclerosant for the management of DV.

Peptic Ulcers in Fukushima Prefecture Related to the Great East Japan Earthquake, Tsunami and Nuclear Accident

Objective Due to the Great East Japan Earthquake, which occurred in March 2011, many residents of Fukushima Prefecture were affected by a radiation accident in addition to suffering loss or damage from the earthquake and the subsequent tsunami. The aim of this study was to evaluate the actual condition of patients with peptic ulcers related to the disaster. Methods Patients with peptic ulcers at six hospitals in three different regions of Fukushima Prefecture during the two months following the disaster and the corresponding period of the year before and the year after the disaster were enrolled in this study. Changes by period and region in the number of esophagogastroduodenoscopy (EGD) examinations and the number of peptic ulcer patients were evaluated as the primary endpoints. Changes in the frequencies of hemorrhagic ulcers were evaluated by period and by region as secondary endpoints. Results The numbers of EGDs and peptic ulcer cases compared to the previous year decreased in 2011 and then increased in 2012. However, the ratio of hemorrhagic ulcers to peptic ulcers was higher in 2011 (51.9%) than in 2010 (38.1%) and 2012 (31.1%), and the 2011 hemorrhagic ulcer ratio was the highest at 63.6% in the coastal area. Regarding bleeding cases during 2011, the rate at 1 month after the disaster (64.1%) was higher than the rate at 2 months after the disaster (40.5%) (p=0.033). Conclusion The number of patients with peptic ulcers did not increase immediately following the disaster in Fukushima Prefecture. However, the rate of bleeding patients increased soon after the disaster, especially in the coastal area.

An autopsy of intravascular large B-cell lymphoma with hemophagocytic syndrome

Intravascular large B-cell lymphoma presents with highly variable symptoms caused by the occlusion of small vessels by neoplastic cells in a variety of organs.