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Background: Lipoblastomas are rare benign tumors that arise from embryonic white fat and almost always occur in babies and children. Here, we report a case of a giant popliteal lipoblastoma in a 23-month-old Japanese girl that was successfully treated via complete resection., Case Presentation: Our patient was a 23-month-old Japanese girl. At 6 months of age, she presented at a nearby hospital with a mass on the popliteal side of her lower right leg. She had no symptoms and was diagnosed as having a benign adipose tumor via magnetic resonance imaging. The mass gradually increased in size, and she was referred to our hospital at 1 year and 11 months of age. A physical examination and radiology revealed a localized mass 13 × 10 × 7 cm in size in the aforementioned area that restricted knee movement and caused proximal tibia deformity. Magnetic resonance imaging showed a giant circumscribed subcutaneous mass with multiple partitions that was hyperintense on T1-weighted and T2-weighted images but not fat-saturated on T2-weighted images. Based on these findings, she was diagnosed as having a lipoblastoma. Because the mass surrounded her popliteal artery and vein and part of the popliteal nerve, surgical resection was considered risky, and we opted to simply observe her. However, owing to rapid growth of the mass and the worsening of symptoms, she underwent complete resection at 2 years and 6 months of age. A histological examination confirmed the diagnosis of a lipoblastoma. She was discharged from our hospital 3 days after surgery with no symptoms. She could walk without pain at the 6-month follow-up, and no local recurrence was observed., Conclusions: We successfully treated a giant popliteal lipoblastoma without complications by performing a total resection. Our report provides evidence that lipoblastomas should be considered for surgical resection when they progress or symptoms appear.

We retrospectively investigated the clinical presentation and outcome of patients with spinal intradural extramedullary tumors, which had been surgically treated. A total of 97 consecutive patients(32 males and 65 females;mean age, 48 years;range, 17-79 years)underwent surgery between 2004 and 2014 at Irkutsk State Medical Academy and affiliated hospitals. To determine presentation and outcomes associated with these tumors, we evaluated the waiting period before surgery, neurological symptoms including motor, sensory, urinary, and rectal dysfunction, modified McCormick scale, Macnab's outcome assessment of patient satisfaction, surgical procedure, tumor location, and histological diagnosis. The most frequent preoperative symptom was myelopathy(52.6%)at presentation, followed by radiculopathy(20.6%), and a combination of both(26.8%). Surgical intervention produced highly successful results, in which 77 of 97 cases showed neurological improvement. However, the symptoms were not completely reversed, and there was improvement only the equivalent to one grade in the modified McCormick scale for most patients. Surgical outcomes were not influenced by tumor location, extent of lamina resection, or histological diagnosis, but there was a correlation between greater patient improvement and a shorter waiting period before surgery. As such, we recommend early total resection for symptomatic spinal intradural extramedullary tumors.

Myxofibrosarcoma (MFS) is a mesenchymal malignancy characterized by frequent recurrence even after radical wide resection. To optimize therapy for MFS patients, we aimed to identify candidate tissue biomarkers of MFS invasion potential. Invasion characteristics of MFS were evaluated by magnetic resonance imaging and protein expression profiling of primary tumor tissues performed using two-dimensional difference gel electrophoresis (2D-DIGE). Protein expression profiles were compared between invasive and non-invasive tumors surgically resected from 11 patients. Among the 3453 protein spots observed, 59 demonstrated statistically significant difference in intensity (≥2-fold) between invasive and non-invasive tumors (p<0.01 by Wilkoxon test), and were identified by mass spectrometry as 47 individual proteins. Among them, we further focused on discoidin, CUB and LCCL domain-containing protein 2 (DCBLD2), a receptor tyrosine kinase with aberrant expression in malignant tumors. Immunohistochemistry analysis of 21 additional MFS cases revealed that higher DCBLD2 expression was significantly associated with invasive properties of tumor cells. DCBLD2 sensitivity and specificity, and positive and negative predictive values for MFS invasion were 69.2%, 87.5%, 90%, and 63.6%, respectively. The expression level of DCBLD2 was consistent in different portions of tumor tissues. Thus, DCBLD2 expression can be a useful biomarker to evaluate invasive properties of MFS. Further validation studies based on multi-institutional collaboration and comprehensive analysis of DCBLD2 biological functions in MFS are required to confirm its prognostic utility for clinical application., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: To synthesize complex type N-glycans in silkworms, shRNAs against the fused lobe from Bombyx mori (BmFDL), which codes N-acetylglucosaminidase (GlcNAcase) in the Golgi, was expressed by recombinant B. mori nucleopolyhedrovirus (BmNPV) in silkworm larvae., Results: Expression was under the control of the actin promoter of B. mori or the U6-2 and i.e.-2 promoters from Orgyia pseudotsugata multiple nucleopolyhedrovirus (OpMNPV). The reduction of specific GlcNAcase activity was observed in Bm5 cells and silkworm larvae using the U6-2 promoter. In silkworm larvae, the partial suppression of BmFDL gene expression was observed. When shRNA against BmFDL was expressed under the control of U6-2 promoter, the Man 3 GlcNAc(Fuc)GlcNAc structure appeared in a main N-glycans of recombinant human IgG. These results suggested that the control of BmFDL expression by its shRNA in silkworms caused the modification of its N-glycan synthetic pathway, which may lead to the alteration of N-glycans in the expressed recombinant proteins., Conclusions: Suppression of BmFDL gene expression by shRNA is not sufficient to synthesize complex N-glycans in silkworm larvae but can modify the N-glycan synthetic pathway.

Background: The world's first diagnostic criteria for early CP were proposed in 2009 in Japan. This study aimed to clarify the clinico-epidemiological features of early CP in Japan., Methods: Patients with early CP who were diagnosed according to the diagnostic criteria for early CP and had visited the selected hospitals in 2011 were surveyed. The study consisted of two-stage surveys: the number of patients with early CP was estimated by the first questionnaire and their clinical features were assessed by the second questionnaire., Results: The estimated number of early CP patients was 5410 (95% confidence interval 3675-6945), with an overall prevalence of 4.2 per 100,000 persons. The number of patients who were newly diagnosed with early CP was estimated to be 1330 (95% confidence interval 1058-1602), with an annual incidence of 1.0 per 100,000 persons. Detailed clinical information was obtained in 151 patients in the second survey. The male-to-female sex ratio was 1.32:1. The mean age was 60.4 and the mean age at disease onset was 55.4. Idiopathic (47.7%) and alcoholic (45.0%) were the two most common etiologies. Proportions of female and idiopathic cases were higher in early CP than in definite CP. Hyperechoic foci without shadowing and stranding were the most common findings on endoscopic ultrasonography. The clinical profiles of early CP patients who showed lobularity with honeycombing on endoscopic ultrasonography or previous episodes of acute pancreatitis were similar to those of definite CP patients., Conclusions: We clarified the current status of early CP in Japan.

Extraskeletal chondroma is defined as a rare, benign, cartilaginous tumor arising from soft tissues such as tendons, tendon sheath synovia, and joint capsules with no continuity to the periosteum or bone cortex. In histopathologic findings, the tumor exhibits many lobular structures and some parts similar to hyaline cartilage. Therefore, it is sometimes difficult to differentiate this tumor from low-grade chondrosarcoma because of their similar histopathologic findings. In order to prevent recurrence, it is necessary to remove the tumor as a whole, including the capsule, so as not to leave any remnants of the tumor. In this article, we report our treatment experience with a case of extraskeletal chondroma in the index finger of a 63-year-old patient.

Recombinant proteins produced in insect cells and insects, unlike those produced in mammalian cells, have pauci-mannose-type N-glycans. In this study, we examined complex-type N-glycans on recombinant proteins via coexpression of human β-1,2-N-acetylglucosaminyltransferase II (hGnT II) and human β1,4-galactosyltransferase (hGalT I) in silkworm pupae, by using the Bombyx mori nucleopolyhedrovirus (BmNPV) bacmid system. The actin A3 promoter from B. mori and the polyhedrin promoter from Autographa californica multiple nucleopolyhedroviruses (AcMNPVs) were used to coexpress hGnT II and hGalT I. These recombinant BmNPVs were coexpressed with human IgG (hIgG), hGnT II and hGalT I in silkworm pupae. When hIgG was coexpressed with hGnT II, approximately 15% of all N-glycans were biantennary, with both arms terminally modified with N-acetylglucosamine (GlcNAc). In contrast, when hIgG was coexpressed with both hGnT II and hGalT I under the control of the polyhedrin promoter, 27% of all N-glycans were biantennary and terminally modified with GlcNAc, with up to 5% carrying one galactose and 11% carrying two. The obtained N-glycan structure was dependent on the promoters used for coexpression of hGnT II or hGalT I. This is the first report of silkworm pupae producing a biantennary, terminally galactosylated N-glycan in a recombinant protein. These results suggest that silkworms can be used as alternatives to insect and mammalian hosts to produce recombinant glycoproteins with complex N-glycans.

Background: This study aimed to elucidate the clinical features and prognostic factors of mesenchymal chondrosarcoma (MCS) and investigate optimal treatment strategies., Methods: Data from 57 patients with MCS were collected from a Japanese Musculoskeletal Oncology Group (JMOG) and retrospectively analyzed., Results: Data from 29 males and 28 females were collected. Primary tumor sites were the head and neck (7 patients), trunk (35 patients), and extremities (15 patients). The tumors originating in the trunk were significantly associated with a worse OS compared with those originating at the other sites in all patients and those with localized disease (P = 0.020 and P = 0.019, respectively). In patients with localized disease, the tumors originating in the head and neck were significantly associated with better OS and MFS compared with those originating in the trunk (P = 0.024 and P = 0.014, respectively). Positive surgical margin was significantly correlated with the worse LRFS (P = 0.018). Adjuvant chemotherapy exhibited a clear trend toward improved OS when MCS was localized in the trunk or extremities (P = 0.057)., Conclusions: Adequate surgery is considered to be the mainstay of treatment for localized MCS. Prognosis was different depending on the site of tumor origin., (© 2017 Wiley Periodicals, Inc.)

Pancreatic stellate cells (PSCs) play a pivotal role in pancreatic fibrosis associated with pancreatic ductal adenocarcinoma (PDAC). Kindlin-2 is a focal adhesion protein that regulates the activation of integrins. This study aimed to clarify the role of kindlin-2 in PSCs in pancreatic cancer. Kindlin-2 expression in 79 resected pancreatic cancer tissues was examined by immunohistochemical staining. Kindlin-2-knockdown immortalized human PSCs were established using small interfering RNA. Pancreatic cancer cells were treated with conditioned media of PSCs, and the cell proliferation and migration were examined. SUIT-2 pancreatic cancer cells were subcutaneously injected into nude mice alone or with PSCs and the size of the tumors was monitored. Kindlin-2 expression was observed in PDAC and the peritumoral stroma. Stromal kindlin-2 expression was associated with shorter recurrence-free survival time after R0 resection. Knockdown of kindlin-2 resulted in decreased proliferation, migration, and cytokine expression in PSCs. The PSC-induced proliferation and migration of pancreatic cancer cells were suppressed by kindlin-2 knockdown in PSCs. In vivo, co-injection of PSCs increased the size of the tumors, but this effect was abolished by kindlin-2 knockdown in PSCs. In conclusion, kindlin-2 in PSCs promoted the progression of pancreatic cancer., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: Myxofibrosarcoma has high frequency of local recurrence after surgery. To determine an optimal treatment for recurrent tumors, clinical features of recurrent cases should be characterized., Methods: We performed a retrospective analysis of 30 patients with recurrent myxofibrosarcoma who underwent surgery between 1999 and 2008., Results: A negative margin after surgery was achieved in only 12 patients (40.0%). The 5-year re-recurrence free-survival rate was 31.7%. The 5-year re-recurrence free survival for those with positive histological margin and those with negative margin were 9.8% and 62.3%, respectively, which indicated that a positive margin was the significant predictor of poor prognosis (P = 0.006). In 21 patients with recurrent myxofibrosarcoma in the extremities, 10 patients (47.6%) ultimately underwent amputation in the follow-up period and the 5-year amputation-free survival rate was 62.5%. The 5-year metastasis-free survival rates and the 5-year overall survival rates were 84.8% and 83.6%, respectively., Conclusions: In this study, the majority of recurrent cases could not achieve negative margins; notably, a positive margin is a significant poor prognostic indicator of local re-recurrence in patients with recurrent myxofibrosarcoma. To control local recurrence of myxofibrosarcoma was extremely difficult and amputation is often needed in the extremity cases., (© The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com)

Objective: Corticosteroid has been established as the standard therapy for autoimmune pancreatitis (AIP), but the requirement for maintenance corticosteroid therapy is controversial. We conducted a randomised controlled trial to clarify the efficacy of maintenance corticosteroid therapy in patients with AIP., Design: We conducted a multicentre, tertiary setting, randomised controlled trial. After the induction of remission with the initial oral prednisolone (PSL) treatment, maintenance therapy with PSL at 5-7.5 mg/day was continued for 3 years or withdrawn at 26 weeks. The primary endpoint was relapse-free survival over 3 years and the secondary endpoint was serious corticosteroid-related complications. All analyses were performed on an intention-to-treat basis., Results: Between April 2009 and March 2012, 49 patients with AIP were randomly assigned to the maintenance therapy group (n=30) or the cessation group (n=19). Baseline characteristics were not different between the two groups. Relapses occurred within 3 years in 11 out of 19 (57.9%) patients assigned to the cessation group, and in 7 of 30 (23.3%) patients in the maintenance therapy group. The relapse rate over 3 years was significantly lower in the maintenance therapy group than that in the cessation group (p=0.011). The relapse-free survival was significantly longer in the maintenance therapy group than that in the cessation group (p=0.007). No serious corticosteroid-related complications requiring discontinuation of PSL were observed., Conclusions: Maintenance corticosteroid therapy for 3 years may decrease relapses in patients with AIP compared with those who discontinued the therapy at 26 weeks., Trial Registration Number: UMIN000001818; Results., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Background: Osteoid osteoma accounts for approximately 10% of all benign bone tumors. The most common sites of osteoid osteoma are the subcortical shaft and metaphyses of long bones, but any other skeletal bone site can be involved. The acetabulum is a rare site according to past reports. This site presents challenges to optimal management because it is anatomically difficult to approach, and because its rarity leads to limited experience with therapeutic procedures. Here, we report for the first time a rare case of osteoid osteoma in the acetabulum that was successfully treated via resection of the nidus and ablation using a standard electrosurgical generator under computed tomographic guidance., Case Presentation: A 9-year-old Japanese girl presented at a clinic with left hip pain without apparent cause for 1 month. She was diagnosed as having coxitis simplex. However, her pain did not change for 1 year and she was admitted to another hospital where osteoid osteoma in her left acetabulum was suspected. She was then referred to our hospital approximately 1 year after first symptom presentation, where she presented with severe left hip pain and was completely unable to walk. Computed tomography examinations revealed a well-demarcated 5 mm mass with bone sclerosis in her left acetabulum. The mass was characterized by low intensity on T1 and high intensity on T2 magnetic resonance images. These findings were consistent with osteoid osteoma of left acetabulum. She underwent computed tomography-guided resection of nidus and ablation using a standard electrosurgical generator. A histological examination confirmed acetabular osteoid osteoma. Complete pain relief was achieved after the procedure and she experienced no complications. She could walk without any pain at the final follow-up 1 year post-treatment and no local recurrence was observed., Conclusions: We successfully treated acetabulum osteoid osteoma without any symptom recurrence by computed tomography-guided resection and ablation using a standard electrosurgical generator. In addition, we preserved our patient's sciatic nerve and triradiate cartilage. Our report provides evidence that a computed tomography-guided procedure should be considered the treatment of choice for osteoid osteoma of the acetabulum because it is a less invasive alternative to en bloc resection.

Background: Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma that has unusual ultrastructural and molecular features. However, unlike other soft tissue sarcomas, it does not have specific clinical symptoms or radiological features, which can make its diagnosis difficult. Nevertheless, extraskeletal myxoid chondrosarcoma has a rare gene fusion (EWSR1-NR4A3) that is useful for making a differential diagnosis., Case Presentation: A 43-year-old Japanese man presented with a soft tissue mass in his right thigh. A physical examination and radiography revealed a large soft tissue mass. During magnetic resonance imaging, the mass exhibited isointensity on T1-weighted images and high intensity on T2-weighted images, as well as gadolinium enhancement at the side edge of the partition structure. Thus, we considered a possible diagnosis of a malignant myxoid soft tissue tumor, such as myxoid liposarcoma, myxofibrosarcoma, or metastatic carcinomas, including myoepithelial tumor and neuroendocrine tumor, and performed an incisional biopsy to make a definitive diagnosis. The pathological findings revealed a lobulated tumor with a myxoid structure and atypical spindle-shaped cells that created eosinophilic cord-like forms. Immunohistochemistry revealed that the tumor was positive for S-100 and negative for synaptophysin, chromogranin A, and pan keratin (AE1/AE3). The percentage of Ki-67 was 10 % in the hot spot area. Based on these clinicopathological findings, we initially considered the possibility of a myxoid liposarcoma, although we did not observe any lipoblasts. Therefore, we considered the possibility of an extraskeletal myxoid chondrosarcoma. As this tumor is very rare, we searched for the EWSR1-NR4A3 gene fusion using fluorescence in situ hybridization, which confirmed the diagnosis of extraskeletal myxoid chondrosarcoma. Positron emission tomography-computed tomography did not identify any obvious metastases, and we performed radical resection of our patient's vastus medialis and femur with a 3 cm margin. After the resection, we treated his resected femur using liquid nitrogen, and reconstructed his femur using autogenous fibula and plate fixation. No local recurrence or metastasis was observed at the 1-year follow-up., Conclusion: Genetic testing is useful for diagnosing extraskeletal myxoid chondrosarcoma based on the presence of the EWSR1-NR4A3 gene fusion.

Background: Historically, local control of recurrent sarcomas has been limited to radiotherapy when surgical re-resection is not feasible. For metastatic carcinomas to the bone or soft tissue, radiotherapy and some interventional radiology treatment along with other systemic therapies have been widely advocated due to the possibility of disseminated disease. These techniques are effective in alleviating pain and achieving local control for some tumor types, but it has not been effective for prolonged local control of most tumors. Recently, cryoablation has been reported to have satisfactory results in lung and liver carcinoma treatment. In this study, we analyzed the clinical outcome of CT-guided cryoablation for malignant bone and soft tissue tumors to elucidate potential problems associated with this procedure., Methods: Since 2011, 11 CT-guided cryoablations in 9 patients were performed for locally recurrent or metastatic bone and soft tissue tumors (7 males and 2 females) at our institute. The patients' average age was 74.8 years (range 61-86) and the median follow up period was 24.1 months (range 5-48). Histological diagnosis included renal cell carcinoma (n = 4), dedifferentiated liposarcoma (n = 2), myxofibrosarcoma (n = 2), chordoma (n = 1), hepatocellular carcinoma (n = 1), and thyroid carcinoma (n = 1). Cryoablation methods, clinical outcomes, complications, and oncological outcomes were analyzed., Results: There were 5 recurrent tumors and 6 metastatic tumors, and all cases had contraindication to either surgery, chemotherapy or radiotherapy. Two and 3 cycles of cryoablation were performed for bone and soft tissue tumors, respectively. The average length of the procedure was 101.1 min (range 63-187), and the average number of probes was 2.4 (range 2-3). Complications included 1 case of urinary retention in a patient with sacral chordoma who underwent prior carbon ion radiotherapy, 1 transient femoral nerve palsy, and 1 minor wound complication. At the final follow up, 4 patients showed no evidence of disease, 2 were alive with disease, and 3 died of disease., Conclusions: Reports regarding CT-guided cryoablation for musculoskeletal tumors are rare and the clinical outcomes have not been extensively studied. In our case series, CT-guided cryoablation had analgesic efficacy and there were no cases of local recurrence post procedure during the follow-up period. Although collection of further data regarding use of this technique is necessary, our data suggest that cryoablation is a promising option in medically inoperable musculoskeletal tumors.

Background: Hibernoma is a rare, benign, soft tissue tumor arising from brown fat that cannot be distinguished from other lipogenic tumors on computed tomography and magnetic resonance imaging (MRI) scans. On the other hand, the image of hibernoma on 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG-PET) is different from that of other lipogenic tumors. However, fewer studies have investigated the typical features of hibernoma on PET scans. We present the case of a hibernoma that was incidentally detected on 18 F-FDG-PET., Case Presentation: A 48-year-old Asian man underwent 18 F-FDG-PET for routine follow-up of gastric cancer. The patient reported a painless soft mass on the upper arm that had persisted for several years. On 18 F-FDG-PET, the mass showed a maximum standardized uptake value of 9.6. The mass showed high intensity on T1- and T2-weighted MRI scans, and showed focally enhanced septa after gadolinium administration. The mass was surgically resected. Histopathologically, the mass was composed of brown adipose cells characteristic of hibernoma. His postoperative course was uneventful, and there was no local recurrence at the final 24-month follow-up., Conclusion: Hibernoma showed strong uniform accumulation on 18 F-FDG-PET, suggesting that 18 F-FDG-PET would be a useful modality for the differential diagnosis of hibernoma versus other lipogenic tumors.

Background: Trabectedin is reported to be particularly effective against translocation-related sarcoma. Recently, a randomized phase 2 study in patients with translocation-related sarcomas unresponsive or intolerable to standard chemotherapy was conducted, which showed clinical benefit of trabectedin compared with best supportive care (BSC). Extraskeletal myxoid chondrosarcoma (EMCS) and Mesenchymal chondrosarcoma (MCS) are very rare malignant soft tissue sarcomas, and are associated with translocations resulting in fusion genes. In addition, the previous in vivo data showed that trabectedin affect tumor necrosis and reduction in vascularization in a xenograft model of a human high-grade chondrosarcoma. The aim of the present analysis was to clarify the efficacy of trabectedin for EMCS and MCS subjects in the randomized phase 2 study., Methods: Five subjects with EMCS and MCS received trabectedin treatment in the randomized phase 2 study. Three MCS subjects were allocated to the BSC group. Objective response and progression-free survival (PFS) were assessed according to the Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 by central radiology imaging review., Results: The median follow-up time of the randomized phase 2 study was 22.7 months, and one subject with MCS was still receiving trabectedin treatment at the final data cutoff. The median PFS was 12.5 months (95 % CI: 7.4-not reached) in the trabectedin group, while 1.0 months (95 % CI: 0.3-1.0 months) in MCS subjects of the BSC group. The six-month progression-free rate was 100 % in the trabectedin group. One subject with MCS showed partial response, and the others in the trabectedin group showed stable disease. Overall survival of EMCS and MCS subjects was 26.4 months (range, 10.4-26.4 months) in the trabectedin group. At the final data cutoff, two of five subjects were still alive., Conclusions: This sub-analysis shows that trabectedin is effective for patients with EMCS and MCS compared with BSC. The efficacy results were better than previously reported data of TRS. These facts suggest that trabectedin become an important choice of treatment for patients with advanced EMCS or MCS who failed or were intolerable to standard chemotherapy., Trial Registration: The randomized phase 2 study is registered with the Japan Pharmaceutical Information Center, number JapicCTI-121850 (May 31, 2012).

Arterial transit time (ATT) is most crucial for measuring absolute cerebral blood flow (CBF) by arterial spin labeling (ASL), a noninvasive magnetic resonance (MR) perfusion assessment technique, in patients with chronic occlusive cerebrovascular disease. We validated ASL-CBF and ASL-ATT maps calculated by pulsed continuous ASL (pCASL) with multiple post-label delay acquisitions in patients with occlusive cerebrovascular disease. Fifteen patients underwent MR scans, including pCASL, and positron emission tomography (PET) scans with 15O-water to obtain PET-CBF. MR acquisitions with different post-label delays (1.0, 1.5, 2.0, 2.5 and 3.0 sec) were also obtained for ATT correction. The theoretical framework of 2-compartmental model (2CM) was also used for the delay compensation. ASL-CBF and ASL-ATT were calculated based on the proposed 2CM, and the effect on the CBF values and the ATT correction characteristics were discussed. Linear regression analyses were performed both on pixel-by-pixel and region-of-interest bases in the middle cerebral artery (MCA) territory. There were significant correlations between ASL-CBF and PET-CBF both for voxel values (r = 0.74 ± 0.08, slope: 0.87 ± 0.22, intercept: 6.1 ± 4.9) and for the MCA territorial comparison in both affected (R2 = 0.67, y = 0.83x + 6.3) and contralateral sides (R2 = 0.66, y = 0.74x + 6.3). ASL-ATTs in the affected side were significantly longer than those in the contralateral side (1.51 ± 0.41 sec and 1.12 ± 0.30 sec, respectively, p <0.0005). CBF measurement using pCASL with delay compensation was feasible and fairly accurate even in altered hemodynamic states.

Purpose: A cephalocele is a congenital anomaly involving the herniation of intracranial tissue from a skull defect. The sac containing the central nervous system (CNS) with the ventricle system is called the encephalocystocele. An atretic cephalocele is thought to be an abortive form of cephalocele, and the essential nature is still controversial., Case Report: Here, we report the case of a newborn boy with an occipital cephalocele containing a small cystic component which was composed of ependymal cells and the immature CNS tissue. A newborn boy was admitted to our hospital because of an occipital mass, which was about 2.5 cm in diameter, located at the posterior midline, and covered with alopetic skin without CSF leakage. He had a cleft palate. Magnetic resonance imaging (MRI) clearly showed an occipital cephalocele with a tiny cystic component connecting to the subarachnoid space. MRI also showed mild hydrocephalus, hypoplasia of the corpus callosum and tentorium cerebelli, dropping down of the bilateral occipital lobes and vermicular agenesis. We performed the extirpation of the subscalp module under general anesthesia and histologically examined the resected mass. On immunohistopathological examination, most part of the subscalp module was fibrous tissue with numerous vessels and meningeal origin cells. In a small part of the innermost layer, we found a small island consisting of CNS tissue and a tiny cyst lined with a single layer of ependymal cells., Conclusion: Based on radiological and immunohistopathological findings, we speculate that the cystic component at the base of the nodule seems to correspond to neural crest remnants but not to true herniation of the brain and cerebral ventricles.

Background and Study Aim: Self-expandable metallic stents (SEMSs) are used for palliation in patients with malignant perihilar biliary strictures. However, recurrent biliary obstruction occasionally causes cholangitis and jaundice. This study aimed to identify risk factors for recurrent biliary obstruction in such patients., Methods: Data from consecutive patients with malignant perihilar biliary strictures treated with endoscopic placement of SEMSs between 2007 and 2014 in Tohoku University Hospital were retrospectively reviewed. Risk factors for recurrent biliary obstruction were calculated using the Cox proportional hazards models (with hazard ratios [HRs] and 95 % confidence interval [95 %CIs]), and SEMS patency period was examined using the Kaplan - Meier method. SEMS patency was defined as the period between SEMS insertion and the development of recurrent biliary obstruction., Results: 104 patients were included. Median survival time was 281 days; and 85 patients died during a median follow-up period of 320 days. Recurrent biliary obstruction occurred in 35 patients. Median SEMS patency period was 549 days. Multivariable analyses showed that: compared with bile duct carcinoma, gallbladder carcinoma was associated with shorter SEMS patency (HR 8.18, 95 %CI 2.41 - 26.83); patency of left-sided SEMS was inferior to that of bilateral (HR 0.5, 95 %CI 0.32 - 0.93) and right-sided SEMS (HR 0.1, 95 %CI 0.02 - 0.65). Cholangitis before SEMS placement increased the risk of recurrent biliary obstruction (HR 11.44; 95 %CI 4.48 - 32.35) and reduced the SEMS patency period (746 vs. 210 days)., Conclusion: Gallbladder carcinoma, left-sided stent placement, and cholangitis before SEMS placement are risk factors for recurrent biliary obstruction after SEMS placement., (© Georg Thieme Verlag KG Stuttgart · New York.)

Percutaneous treatments for trigeminal neuralgia (TN) including glycerol rhizotomy (GR), radiofrequency thermocoagulation (RT), and balloon compression (BC) are effective for patients with medical comorbidities and risk factors of microvascular decompression (MVD). These procedures are usually performed under fluoroscopy. Surgeons advance the needle to the trigeminal plexus through the foramen ovale while observing landmarks of fluoroscopic images; however, it is sometimes difficult to appropriately place the needle tip in Meckel's cave. We present the technical details of percutaneous GR using a single-plane, flat panel detector angiography system to check the needle positioning. When the needle tip may be located near the trigeminal cistern, three-dimensional (3-D) bone images are taken with cone-beam computed tomography (CT). These images clearly show the position of the needle tip in Meckel's cave. If it is difficult to place it through the foramen ovale, surgeons perform cone beam CT to observe the actual position of the needle tip at the skull base. After confirming the positional relation between the needle tip and foramen ovale, surgeons can advance it in the precise direction. In 10 procedures, we could place the nerve-block needle in about 14.5 minutes on average without complications. We think that our method is simple and convenient for percutaneous treatments for TN, and it may be helpful for surgeons to perform such treatments.

Small cell glioblastoma (SCGBM) is a variant of glioblastomas characterized by a predominant population of small and monomorphic glial cells. The aim of the present study was to investigate clinical, neuroimaging, pathologic, and genetic features of SCGBM. The clinicopathologic and genetic features were evaluated in 14 patients with SCGBM. All cases were divided into multifocal and solitary type by MRI, and extent of microvascular proliferation, intratumoral necrosis, and perivascular lymphocytic accumulation were investigated. IDH1 mutations by immunohistochemistry (IDH1 R132H) and 1p 19q codeletion by fluorescence in situ hybridization were detected. Patients ranged from 23 to 92 years of age (median: 71 years), with three females and eleven males. The overall survival time of the patients ranged from 7 to 23 months (mean: 11 months). Nine patients (64%) were the multifocal type. Pathologic study revealed that the microvascular proliferation, necrosis, and lymphocytic infiltration were limited in SCGBM. Immunohistochemically, tumor cells were negative for IDH1 R132H in all patients. FISH analysis demonstrated that no SCGBM had 1p/19q codeletion in informative patients. Our investigation suggested that an elderly onset and multifocal lesions were characteristics of SCGBM associated with degradation of the immune response, infiltrative feature of tumor cells, and an unfavorable prognosis.

Objective: The purpose of this study was to examine the cardiovascular protective effects of candesartan in patients undergoing percutaneous coronary intervention (PCI) with drug-eluting stents (DESs)., Background: Candesartan has been reported to reduce cardiovascular events when therapy was started 6 months after PCI with bare-metal stents in patients who survived restenosis. Candesartan started immediately after PCI with DESs was also effective in preventing cardiovascular events., Methods: The 4C trial was a multicenter, prospective, randomized, open-label study. A total of 1145 patients at 39 centers in Japan were randomly assigned to receive candesartan plus standard medical treatment or standard medical treatment alone. The primary endpoints were all-cause death, and a composite of non-fatal myocardial infarction (MI), unstable angina pectoris (uAP), congestive heart failure (CHF), and non-fatal cerebrovascular events. The follow-up period was up to 3 years after the index PCI (ClinicalTrials.gov NCT00139386)., Results: The incidence of total death, one of the primary endpoints, was comparable between the two treatment groups (3.8% each, p=0.9702). Another primary endpoint, non-fatal major cardiovascular events, tended to occur more often in the control group than in the candesartan group (9.2% vs. 12.5%, p=0.0985). In contrast, candesartan significantly reduced one of the pre-specified secondary endpoints: cardiovascular events that included non-fatal MI, uAP, and CHF (4.4% vs. 6.7%, p=0.0136). Furthermore, candesartan significantly reduced another secondary endpoint that included cardiovascular events and cardiovascular death (5.0% vs. 7.7%, p=0.0493)., Conclusions: The 4C trial showed that candesartan administered immediately after PCI with DESs did not improve the prognosis after the index procedure, but did reduce some cardiac-related events for 3 years., (Copyright © 2015. Published by Elsevier Ltd.)

Background: Autologous bone flaps adequately preserved can be used successfully for repair of cranial bone defects with biological and economic advantages. However, the effect or advantages of cryopreservation of autologous bone flaps using cryoprotectant solutions have not been investigated. The aim of this study was to investigate the utility of autologous bone flaps frozen with glycerol in cranioplasty after decompressive craniectomy., Methods: We evaluated postoperative complications and clinical and aesthetic results in 40 consecutive patients who underwent delayed cranioplasty using autologous bone flaps frozen with glycerol following decompressive craniectomy between 2001 and 2010. Bone flaps removed at the time of external decompression were dipped in 20 % glycerol solution with a sterilized plastic vessel and stored at -84 °C in an ultra-low temperature freezer. The bone flaps were thawed in the vessel at room temperature for 12 h. The bone flaps were taken out of melting glycerol, replaced in their original positions, and fixed with silk sutures or titanium plates. Follow-up periods ranged from 35 to 3745 days (median, 558 days)., Results: Mild resorption of the bone flap occurred in one case. However, there were no cases with local infection and no cases requiring reoperation for complications during the follow-up period., Conclusions: Cryopreservation with glycerol is a simple and safe method for preservation of autologous bone grafts. The clinical and aesthetic results of delayed cranioplasty using autologous bone flaps frozen with glycerol solution were satisfactory.

α-tricalcium phosphate (α-TCP, α-Ca3(PO4)2) receives great attention for bone repairing due to its biodegradability and capability of transformation to human bone's main inorganic components, hydroxyapatite (HAp). α-TCP porous scaffold is easily procurable by sintering of the low-temperature polymorph of TCP, β-TCR Still, porous body of α-TCP is too brittle to being handled and shaped, limiting its clinical application as implant materials. To improve mechanical properties of α-TCP porous scaffold, the present study focused on coating of a type of polysaccharides on α-TCP scaffolds. Gellan gum was chosen as the polysaccharide for coating because of its biodegradability as well as the potential acting as substrate for HAp deposition during hydration of α-TCP after exposure to body fluid. After coating of gellan gum on α-TCP scaffolds with porosity of 75 vol%, the compressive strength increased from 0.45 MPa to around 2.00 MPa. Among the coated scaffold, the maximum compressive strength, 3.97 MPa, was obtained on the scaffold with porosity of 63 vol%. Improvement of mechanical properties of α-TCP/gellan gum composites was achieved to show easy handling performance for a bone substitute for tissue repairing. The dissolving rate of the coated scaffolds was also controlled by adjusting the concentration of GG solutions.

Fibrous dysplasia (FD) is a monostotic or polyostotic benign bone lesion with spindle-cell proliferation in woven bone and stroma. Rarely, cartilaginous differentiation can be seen in the lesions of FD. FD with massive cartilaginous differentiation is called fibrocartilaginous dysplasia (FCD) and is considered a rare variant of FD. Although pathological findings of FD show irregular immature bone formation without osteoblastic rimming in fibrous tissue, and rarely show very small amounts of cartilage, histological images of FCD are said to show that cartilage with a relatively high cell density is present in the majority and that FD-like findings are seen in parts of it. The most characteristic feature of FCD on images is calcification in the lesions reflecting cartilaginous tissue. On the other hand, typical radiographic findings of FD include shadows with a ground-glass appearance and thinning and bulging of the cortical bone, the observation if calcification is not usual. Therefore, in the diagnosis of FCD, differentiation from multiple enchondromatosis, Ollier disease, chondrosarcoma, and chondrosarcoma secondary to FD is necessary, and it seems important to make a careful diagnosis based not only on the pathological findings but also on imaging and clinical findings. Herein, we report on a case of FD of the proximal femur associated with intralesional extensive carti laginous differentiation in which a pathological fracture occurred during follow-up, with a review of the literature.

A 32-year-old woman was referred due to abdominal pain and elevated liver enzymes. Computed tomography and magnetic resonance imaging showed ectopic opening of the common bile duct (CBD) into the duodenal bulb. Esophagogastroduodenoscopy showed a hemispheric bulge in the duodenal bulb. Endoscopic retrograde cholangiopancreatography (ERCP) revealed the bulge to be cystic dilatation of the CBD. ERCP also showed no communication between the ventral and dorsal pancreatic ducts. We diagnosed the patient with ectopic opening of the CBD accompanied by choledochocele and pancreas divisum. Endoscopic incision was performed for the treatment of the choledochocele. The patient's symptoms and elevated liver enzymes improved after treatment.

Objective: To investigate the predictive factors for visual and ischemic complications after open surgery for paraclinoid aneurysms of the internal carotid artery (ICA)., Materials and Methods: Thirty-eight consecutive patients with unruptured paraclinoid aneurysms of ICA operated on between 2009 and 2013 were included in this study. The male:female ratio was 6:32 and the ages ranged from 33 to 81 (mean: 60 ± 2). Twenty cases were asymptomatic and 18 had ophthalmological symptoms. The sizes of the aneurysms ranged from 2 to 35 mm (mean: 10.6 ± 9 mm). Twenty-three patients were treated by clipping and 15 by trapping with bypass (high-flow bypass in 11, and low-flow in 4). Twenty-four patients underwent removal of the anterior clinoid process. Among them, 8 underwent en bloc anterior clinoidectomy with a high-speed drill, and 16 had piecemeal excision with a microrongeur or ultrasonic bone curette. Intraoperative monitoring was performed using motor-evoked potentials (MEP) and visual-evoked potentials (VEP) in 27 and 15 cases, respectively., Results: Complete obliteration of the aneurysm was achieved in 37 cases (97.4 %). The patency rate of bypass was 100 %. Postoperative worsening of visual acuity, including one case of blindness, was observed in six cases (11 %). Worsening of visual field defects occurred in 14 cases (38 %), but 10 of them were transient. Transient oculomotor nerve palsy occurred in six cases (15 %). Postoperative stroke was detected by diffusion-weighted imaging (DWI) in five cases (13 %), four of which were symptomatic. Statistical analysis showed that piecemeal anterior clinoidectomy was significantly safer than en bloc removal in preserving visual function. Trapping with high-flow bypass had a significantly greater risk of postoperative stroke than direct clipping., Conclusions: Intraoperative VEP monitoring might be useful for preventing postoperative worsening of visual function. Two-stage treatment with bypass and endovascular trapping might be safer than single-stage trapping alone.

Background: Standard imaging modality for the follow-up after prosthetic replacements for musculoskeletal tumor patients has been conventional radiography. This technique is effective in detecting subtle changes in bone adjacent to metal implants, but in many cases, radiographs do not lead to definitive diagnosis of postoperative adverse events such as acute infection, local recurrence of soft tissue tumor or soft tissue local recurrence of osseous sarcoma. Conventional MRI sequences have not been effective due to metal artifacts. In this study, we tried to elucidate the effectiveness of metal artifact suppression using novel sequence, multiacquisition variable-resonance image combination (MAVRIC), after musculoskeletal tumor surgeries., Methods: We retrospectively analyzed 5 cases of malignant bone and soft tissue sarcoma patients who were reconstructed with metal prosthesis after wide resection of tumors. Images obtained using MAVRIC and short tau inversion recovery (STIR) were compared side by side. The paired MAVRIC and STIR images were qualitatively compared independently by two specialists for 4 parameters: visualization of bone - implant interface, visualization of surrounding soft tissues, image blurring, and overall image quality. Quantitatively, paired images were reviewed to identify the slice where the metal artifact was maximal, and a region of interest encompassing the implant and surrounding artifact was drawn using Advantage Workstation (GE Healthcare, Japan)., Results: There were no local recurrences that were detected. By utilizing MAVRIC, visualization of the bone - implant interface and visualization of the surrounding soft tissue were significantly improved in MAVRIC compared to STIR. Although blurring was worse on the MAVRIC acquisitions, the overall image quality was still better on MAVRIC. Quantitatively, the area of metal artifact measured using MAVRIC was markedly less compared to STIR (61.4 cm(2) vs 135.9 cm(2))., Conclusion: Despite the relatively small number of cases in the present study, our observation strongly suggests that MAVRIC is able to improve the quality of images by decreasing the artifact caused by endoprosthesis, frequently utilized in reconstruction of musculoskeletal tumor patients. Further installments of conventional imaging sequences with the addition of gadolinium - enhancement will enable increased accuracy in diagnosing local recurrences of sarcoma patients.

Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed.

A 50-year-old male was referred to our hospital for the evaluation of hyperproteinemia. Fluorodeoxyglucose positron emission tomography revealed high fluorodeoxyglucose uptake in the pancreas, bilateral lacrimal glands, submandibular glands, parotid glands, bilateral pulmonary hilar lymph nodes, and kidneys. Laboratory data showed an elevation of hepatobiliary enzymes, renal dysfunction, and remarkably high immunoglobulin (Ig) G levels, without elevated serum IgG4. Abdominal computed tomography revealed swelling of the pancreatic head and bilateral kidneys. Endoscopic retrograde cholangiopancreatography showed an irregular narrowing of the main pancreatic duct in the pancreatic head and stricture of the lower common bile duct. Histological examination by endoscopic ultrasonography-guided fine-needle aspiration revealed findings of lymphoplasmacytic sclerosing pancreatitis without IgG4-positive plasma cells. Abnormal laboratory values and the swelling of several organs were improved by the treatment with steroids. The patient was diagnosed as having type 1 autoimmune pancreatitis (AIP) based on the International Consensus Diagnostic Criteria. Therefore, we encountered a case of compatible type 1 AIP without elevated levels of serum IgG4 or IgG4-positive plasma cells. This case suggests that AIP phenotypes are not always associated with IgG4.

Epithelioid sarcoma is a rare soft tissue sarcoma and usually resistant to chemotherapy. It has high rates of local recurrence and distant metastasis, and the prognosis after metastasis is poor. We report a case of multiple lung metastases of an epithelioid sarcoma originating in the inguinal area that we treated with the multikinase inhibitor pazopanib. The patient was a 38-year-old male who began to experience discomfort in his left inguinal area. Magnetic resonance imaging showed a tumor extended from the medial aspect of the wing of the left ilium along the iliopsoas muscle to its site of insertion on the femur. The histopathological diagnosis with a biopsy was proximal-type epithelioid sarcoma. Although a positron emission tomography examination showed fluorodeoxyglucose accumulation in the left inguinal tumor, there was no distant metastasis. Wide resection by a combined iliac resection procedure was performed. Twelve months after surgery, computed tomography revealed multiple nodules and a diagnosis of bilateral multiple lung metastases was made. Treatment with pazopanib 800 mg was started. After 2.5 months of treatment, a clear reduction in the size of the pulmonary metastases was shown. Thirty months after the start of pazopanib treatment, most of the metastases have disappeared, and no development of new lesions has been seen. Therefore, it appeared that pazopanib was capable of serving as one of the choices of therapeutic agents that should be taken into consideration for the treatment of advanced epithelioid sarcoma.

Background: Spontaneous spinal epidural hematoma (SSEH) is relatively rare. SSEH with anticoagulants including warfarin and rivaroxaban (Factor Xa inhibitor) have been reported; however, SSEH with Factor X deficiency has not been described yet., Methods: Case report., Findings: An 82-year-old woman with acquired Factor X deficiency complained of sudden onset of severe posterior neck pain. Magnetic resonance imaging demonstrated an epidural hematoma from C3 to T3 levels. Because she showed tetraparesis on the third hospital day, we performed surgery. Just before surgery, her prothrombin time-international normalized ratio was 2.49, which was immediately reversed by infusion of prothrombin complex concentrate. The patient safely underwent an emergency laminectomy from C3 to T2, in which the epidural hematoma was evacuated. Post-operatively, the patient recovered completely without rebleeding. Hematologists found acquired deficiency of Factor X in this patient with systemic amyloid light-chain amyloidosis., Conclusion: To our knowledge, this is the first report of a case of SSEH with Factor X deficiency. A blood coagulation disorder should be considered in patients with SSEH.

The i-motif structures are formed by oligonucleotides containing cytosine tracts under acidic conditions. The folding of the i-motif under physiological conditions is of great interest because of its biological role. In this study, we investigated the effect of the intra-strand cross-link on the stability of the i-motif structure. The 4-vinyl-substituted analog of thymidine (T-vinyl) was incorporated into the 5'-end of the human telomere complementary strand, which formed the intra-strand cross-link with the internal adenine. The intra-strand cross-linked i-motif displayed CD spectra similar to that of the natural i-motif at acidic pH, which was transformed into a random coil with the increasing pH. The pH midpoint for the transition from the i-motif to random coil increased from pH 6.1 for the natural one to pH 6.8 for the cross-linked one. The thermodynamic parameters were obtained by measuring the thermal melting behaviors by CD and UV, and it was determined that the intra-strand cross-linked i-motif is stabilized due to a favorable entropy effect. Thus, this study has clearly indicated the validity of the intra-strand cross-linking for stabilization of the i-motif structure., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Single preoperative biliary drainage for malignant perihilar biliary stricture occasionally fails to control jaundice and cholangitis. Multiple biliary drainage is required in such cases, but their clinical background is unclear. We determined the clinical characteristics associated with the requirement for multiple biliary drainage., Methods: The consecutive 122 patients with malignant perihilar biliary stricture were enrolled in a single-center retrospective study. Preoperative biliary drainage was initially performed on the future remnant hepatic lobe. Additional drainage was performed if jaundice failed to improve or cholangitis developed in undrained hepatic lobes. Detailed clinical characteristics and the number of preoperative biliary drainage procedures required before operation were analyzed., Results: Thirty-one patients (25.4%) initially underwent multiple biliary drainage. However, 69 (56.7%) required multiple biliary drainage by the time of the operation. In the univariate analysis, the initial serum bilirubin level, cholangitis, percutaneous portal vein embolization, history of inserted endoscopic biliary stenting, length of preoperative period, operative procedure, and Bismuth classification were significant factors. In the multivariate analysis using these factors, Bismuth classification was independently associated with the requirement for multiple biliary drainage. The number of patients who required multiple biliary drainage was higher in those with Bismuth-II (91.9%), Bismuth-IIIa (65.7%), and Bismuth-IV (92.9%) than in those with Bismuth-I (22.2%) and Bismuth-IIIb (18.2%)., Conclusions: Patients with Bismuth-II, Bismuth-IIIa, and Bismuth-IV are at higher risk for multiple biliary drainage. A strategy based on the Bismuth classification for performing preoperative biliary drainage is important for patients with malignant perihilar biliary stricture.

According to the newest World Health Organization (WHO) classification for soft tissue tumors in 2013, malignant fibrous histiocytoma (MFH) has been gone. Most sarcomas called MFH were reclassified to be high-grade pleomorphic forms of leiomyosarcoma, liposarcoma, rhabdomyosarcoma, and other sarcomas by recent molecular technologies. However, about 10% to 15% of sarcomas called MFH before, still cannot be given a precise classification, and these are now called undifferentiated pleomorphic sarcoma or are still called MFH. Further molecular approaches including proteomic approaches are imperative to classify these unclassified sarcomas for improving clinical outcomes of the patients with soft tissue sarcomas. This article is part of a Special Issue entitled: Medical Proteomics., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background and Objective: Intrathecal baclofen (ITB) pump implantation for cerebral palsy (CP) patients is usually performed in the lateral position; however, it might be difficult for some patients with severe deformity to take a lateral position during surgery., Method: We report a case of ITB pump implantation in the prone position for a CP patient who exhibited uncontrollable opisthotonus with severe scoliosis., Result: ITB therapy effectively controlled her opisthotonus., Conclusion: Our findings suggest that ITB therapy may be useful for CP patients with uncontrollable spasticity, dystonia, or opisthotonus who are not able to take a lateral position for pump implantation due to deformities of their extremities and spine., (© 2014 International Neuromodulation Society.)

We report a rare case of cerebral amyloid angiopathy (CAA) causing large contralateral hemorrhage during surgery for lobar hemorrhage. A 62-year-old woman presented with lobar hemorrhage in the left frontal and parietal lobes recurring over the previous 1 month. Because we could not detect the origin of the lobar hemorrhage, we performed a biopsy around the lobar hemorrhage site with the removal of a hematoma. During the surgery, we identified acute brain swelling without bleeding from the operative field. Intraoperative computed tomography demonstrated new large lobar hemorrhage of the right parietal lobe, which we could promptly remove. Specimens around hematomas on both sides were pathologically diagnosed as CAA on immunohistochemical examination. After the surgery, she suffered from lobar hemorrhage three times in the space of only 3 months. To the best of our knowledge, there has been no reported case of CAA causing intracranial hemorrhage of another lesion during surgery. Neurosurgeons should know a possibility of intraoperative hemorrhage in surgeries for lobar hemorrhage caused by CAA., (Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Although cadaver dissections are important for skull base surgeons to acquire anatomical knowledge and techniques, their opportunities are limited in Japan. The Autopsy Imaging Center of the University of Fukui Hospital has both a CT scanner and an MR unit solely for deceased patients. The authors applied the postmortem imaging to cadaver dissections and evaluated its usefulness in surgical education. Ten sides of five formalin-fixed cadaver heads were dissected by ten neurosurgeons. Five neurosurgeons were young, three were moderately experienced, and two were experts in skull base surgery. They performed orbitozygomatic, anterior transpetrosal, posterior transpetrosal, and transcondylar approaches. CT bone images were taken before and after dissections, and MR images were taken before dissection to merge with the CT bone images. The usefulness of the images for each neurosurgeon and for each skull base approach was evaluated. The postmortem imaging system was useful for all neurosurgeons, especially in anterior transpetrosal, posterior transpetrosal, and transcondylar approaches. They could find the insufficiency or excessiveness of their drilling of specific bony structures with the images. Even the experts in skull base surgery could identify regions in which they could add drilling safely to widen the surgical field more. The postmortem imaging system was useful for skull base cadaver dissections. This system is expected to be utilized for education and research on surgical anatomy.

Described herein is the technique of performing assisting low-speed and high-speed extra-intracranial bypass, followed by a clinical case report concerning successful application of this technique in trapping of a giant aneurysm of the cavernous portion of the internal carotid artery in a female patient after endured transitory ischaemic attack during the test with temporary balloon-mediated occlusion of the internal carotid artery.

Objective: Myxofibrosarcoma (MFS) is characterized by a high frequency of local recurrence after surgery because of infiltrative growth of the tumor cells. This infiltrative growth creates a characteristic 'tail-like' pattern on magnetic resonance imaging (MRI), and it has been reported that this pattern is especially obvious on gadolinium-enhanced MRI (Gd MRI). However, the relationship between the tail-like pattern seen on Gd MRI and clinicopathological features of MFS is still not clear. In this study, we performed a retrospective analysis to identify clinicopathological factors related to the tail-like pattern of the MRI findings in patients with MFS., Materials and Methods: We retrospectively analyzed 50 patients with MFS to identify factors related to the tail-like pattern., Results: On Gd MRI, 32 of the 50 patients presented the tail-like pattern, whereas 18 presented a solid pattern. The clincopathological factors related to the tail-like pattern were evaluated by chi-squared test. A superficial origin (p = 0.0009) was most significantly related to the tail-like pattern. The 5-year recurrence-free survival (RFS) rate was 75.6 % for patients showing the tail-like pattern and 90.9 % for those showing the solid pattern. The corresponding 5-year disease-free survival (DFS) rates were 64.7 and 79.3 %, respectively. Thus in terms of both 5-year RFS and DFS, patients with the tail-like pattern tended to have a poorer outcome., Conclusion: A superficial origin of MFS is significantly related to a tail-like pattern on Gd MRI. The tail-like pattern is associated with poorer prognosis. Further studies of tumor depth and the tail-like pattern on Gd MRI are needed.

Background: Intraoperative morphological and functional monitoring is essential for safe neurosurgery. Functional monitoring is based on electroencephalography (EEG), which uses silver electrodes. However, these electrodes generate metal artifacts as silver blocks X-rays, creating white radial lines on computed tomography (CT) images during surgery. Thick electrodes interfere with surgical procedures. Thus, thinner and lighter electrodes are ideal for intraoperative use., Methods: The authors developed thin brain electrodes using carbon nanotubes that were formed into thin sheets and connected to electrical wires., Results: The nanotube sheets were soft and fitted the curve of the head very well. When attached to the head using paste, the impedance of the newly developed electrodes was 5 kΩ or lower, which was similar to that of conventional metal electrodes. These electrodes can be used in combination with intraoperative CT, magnetic resonance imaging (MRI), or cerebral angiography. Somatosensory-evoked potentials, auditory brainstem responses, and visually evoked potentials were clearly identified in ten volunteers. The electrodes, without any artifacts that distort images, did not interfere with X-rays, CT, or MR images. They also did not cause skin damage., Conclusions: Carbon nanotube electrodes may be ideal for neurosurgery.

Objectives: A nationwide epidemiological survey of acute pancreatitis (AP) was performed to estimate the number of patients with AP in 2011 in Japan and to clarify the clinical features., Methods: The first survey was performed by sending a questionnaire to randomly selected 4175 departments to determine the number of hospitalized patients with AP during 2011. The second survey was conducted by sending a questionnaire to departments in which hospitalized patients with AP were treated based on the first questionnaire. Evaluation of the AP severity was based on the revised severity scoring system of AP of the Japanese Ministry of Health, Labor and Welfare (2008)., Results: The estimated total number of patients with AP in 2011 was 63,080 (95% confidence interval, 57,678-68,484), with an overall prevalence rate of 49.4 per 100,000 population. The male-to-female ratio was 1.9. The mean age was 58.5 in male and 65.3 in female patients. Alcoholic AP was the most common in male and gallstone AP was the most common in female patients. The overall mortality of AP was 2.6%, and in severe AP, 10.1%., Conclusions: The number of patients with AP is still increasing. The revised severity scoring system provided a more precise prediction of prognosis.