Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized primarily by developmental anomalies of the anterior segment of the eye and systemic manifestations, including craniofacial abnormalities, dental anomalies, and neurological involvement. Although genetic mutations in the forkhead box C1 (FOXC1) or paired-like homeodomain transcription factor 2 (PITX2) have been implicated, the full extent of associated neurological features remains underexplored. We present the case of a 2-year-old boy diagnosed with Axenfeld-Rieger syndrome who exhibited mild facial dysmorphism and required surgical intervention for glaucoma. Genetic testing identified a FOXC1 mutation, and family history revealed that his father and paternal grandfather were also affected. Brain magnetic resonance imaging scans revealed periventricular white matter lesions, dilated perivascular spaces, and vertebrobasilar artery dolichoectasia. This case underscores the potential for significant neurological findings in patients with Axenfeld-Rieger syndrome and highlights the clinical value of comprehensive neuroradiological evaluation in such cases.

Cardiac angiosarcoma is a malignant tumor derived from vascular endothelium with a dismal prognosis. The imaging findings of cardiac angiosarcoma are nonspecific and endomyocardial and pericardial biopsies have insufficient accuracy. For these reasons, the diagnosis is sometimes difficult. Primary and metastatic tumors tend to bleed easily, causing hemoptysis and neurological symptoms. Brain metastases are not often known to be fatal when they cause hemorrhage. We report a 27-year-old man diagnosed with right atrium angiosarcoma, with metastases in the lung, brain, and bone. The patient had only respiratory symptoms at the first visit and did not show any symptoms derived from brain metastases yet died after 27 days due to hemorrhage from brain metastases. If brain metastasis from angiosarcoma is suspected based on imaging findings, urgent radiotherapy should be considered before histological examination for a definitive diagnosis.

This study investigated the influence of iterative reconstruction (IR) methods on computed tomography (CT) images when training convolutional neural network (CNN) models to diagnose pulmonary emphysema. To evaluate the influence of the IR algorithm on CNN, the present study comprised two steps: the comparison of noise reduction by IR algorithms using phantom examinations and the change in performance of CNN with IR algorithms using patient data. We retrospectively analyzed 97 patients. Raw CT data were reconstructed using the filtered back-projection (FBP) and adaptive statistical iterative reconstruction V (ASIR-V) algorithms with blending levels of 30

BACKGROUND International guidelines recommend brachytherapy for patients with dysphagia from esophageal cancer, whereas brachytherapy is infrequently used to palliate dysphagia in some countries. To clarify the availability of palliative treatment for dysphagia from esophageal cancer and explain why brachytherapy is not routinely performed are unknown, this study investigated the use of brachytherapy and external beam radiotherapy for dysphagia from esophageal cancer. METHODS Japanese Radiation Oncology Study Group members completed a survey and selected the treatment that they would recommend for hypothetical cases of dysphagia from esophageal cancer. RESULTS Of the 136 invited facilities, 61 completed the survey (44.9%). Four (6.6%) facilities performed brachytherapy of the esophagus, whereas brachytherapy represented the first-line treatment at three (4.9%) facilities. Conversely, external beam radiotherapy alone and chemoradiotherapy were first-line treatments at 61 and 58 (95.1%) facilities, respectively. In facilities that performed brachytherapy, the main reason why brachytherapy of the esophagus was not performed was high invasiveness (30.2%). Definitive-dose chemoradiotherapy with (≥50 Gy) tended to be used in patients with expected long-term survival. CONCLUSIONS Few facilities routinely considered brachytherapy for the treatment of dysphagia from esophageal cancer in Japan. Conversely, most facilities routinely considered external beam radiotherapy. In the future, it will be necessary to optimize external beam radiotherapy.

Abstract Evidence regarding postoperative radiation therapy (PORT) for metastases to the long bones is lacking. Characterizing the current practice patterns and identifying factors that influence dose-fractionation schedules are essential for future clinical trials. An internet-based survey of the palliative RT subgroup of the Japanese Radiation Oncology Study Group was performed in 2017 to collect data regarding PORT prescription practices and dose-fractionation schedules. Responders were also asked to recommend dose-fractionation schedules for four hypothetical cases that involved a patient with impending pathological fractures and one of four clinical features (poor prognosis, solitary metastasis, radio-resistant primary tumor or expected long-term survival). Responders were asked to indicate their preferred irradiation fields and the reasons for the dose fractionation schedule they chose. Responses were obtained from 89 radiation oncologists (67 institutions and 151 RT plans) who used 22 dose-fractionation schedules, with the most commonly used and recommended schedule being 30 Gy in 10 fractions. Local control was the most common reason for preferring longer-course RT. High-dose fractionated schedules were preferred for oligometastasis, and low-dose regimens were preferred for patients with a poor prognosis; however, single-fraction RT was not preferred. Most respondents recommended targeting the entire orthopedic prosthesis. These results indicated that PORT using 30 Gy in 10 fractions to the entire orthopedic prosthesis is preferred in current Japanese practice and that single-fraction RT was not preferred. Oligometastasis and poor prognosis influenced the selection of high- or low-dose regimens.

Purpose This study aimed to assess whether a Monte Carlo (MC)-based algorithm reflects the influence of totally implantable venous access ports (TIVAPs) in external radiation therapy. Materials and Methods The present study comprised two steps: experimental measurements of depth doses and surface doses with and without TIVAPs and calculation with an MC-based algorithm. Results The TIVAP-associated maximum dose reduction compared with the dose at the same depths without TIVAPs was 7.8% at 4 MV, 6.9% at 6 MV, and 5.7% at 10 MV in measurement, and 7.4% at 4 MV, 6.6% at 6 MV, and 5.5% at 10 MV in calculation. Relative surface doses were higher with TIVAPs made of titanium, due to a higher fluence of backscattered electrons from the TIVAPs, than with plastic TIVAPs. There were no significant differences in the relative differences between the measured and calculated doses of the titanium TIVAP group and the plastic TIVAP group at 4 MV ( p = 0.99), 6 MV ( p = 0.67), and 10 MV ( p = 0.54). Conclusion TIVAPs caused target dose reductions and dose increase near the TIVAP, especially when made of titanium. The influences are reflected in the MC-based algorithm.

We evaluated the evolving structure of radiation oncology in Japan in terms of equipment, personnel, patient load and geographic distribution to identify and overcome any existing limitations. From March 2012 to August 2015, the Japanese Society for Radiation Oncology conducted a questionnaire based on the Japanese national structure survey of radiation oncology in 2011. Data were analyzed based on the institutional stratification by the annual number of new patients treated with radiotherapy per institution. The estimated annual numbers of new and total (new plus repeat) patients treated with radiation were 211 000 and 250 000, respectively. Additionally, the estimated cancer incidence was 851 537 cases with approximately 24.8% of all newly diagnosed patients being treated with radiation. The types and numbers of treatment devices actually used included linear accelerator (LINAC; n = 836), telecobalt (n = 3), Gamma Knife (n = 46), 60Co remote afterloading system (RALS; n = 24), and 192Ir RALS (n = 125). The LINAC system used dual-energy functions in 619 units, 3D conformal radiotherapy functions in 719 and intensity-modulated radiotherapy (IMRT) functions in 412. There were 756 JRS or JASTRO-certified radiation oncologists, 1018.5 full-time equivalent (FTE) radiation oncologists, 2026.7 FTE radiotherapy technologists, 149.1 FTE medical physicists, 141.5 FTE radiotherapy quality managers and 716.3 FTE nurses. The frequency of IMRT use significantly increased during this time. To conclude, although there was a shortage of personnel in 2011, the Japanese structure of radiation oncology has clearly improved in terms of equipment and utility.

ABSTRACT This paper describes the ongoing structure of radiation oncology in Japan in terms of equipment, personnel, patient load and geographic distribution to identify and overcome any existing limitations. From March 2013 to August 2016, the Japanese Society for Radiation Oncology conducted a questionnaire based on the Japanese national structure survey of radiation oncology in 2012. Data were analyzed based on the institutional stratification by the annual number of new patients treated with radiotherapy per institution. The estimated annual numbers of new and total (new plus repeat) patients treated with radiation were 213 000 and 251 000, respectively. Additionally, the estimated cancer incidence was 865 238 cases with ~24.6% of all newly diagnosed patients being treated with radiation. The types and numbers of treatment devices actually used included linear accelerator (LINAC; n = 864), telecobalt (n = 0), Gamma Knife (n = 44), 60Co remote afterloading system (RALS; n = 23) and 192Ir RALS (n = 130). The LINAC system used dual-energy functions in 651 units, 3D conformal radiotherapy functions in 759 and intensity-modulated radiotherapy (IMRT) functions in 466. There were 792 Japan Radiological Society/Japanese Society for Radiation Oncology-certified radiation oncologists, 1061.6 full-time equivalent (FTE) radiation oncologists, 2124.2 FTE radiotherapy technologists, 181.3 FTE medical physicists, 170.9 FTE radiotherapy quality managers and 841.5 FTE nurses. The frequency of IMRT use significantly increased during this time. In conclusion, the Japanese structure of radiation oncology has clearly improved in terms of equipment and utility although there was a shortage of personnel in 2012.

Although postoperative radiation therapy (RT) (PORT) for patients with long bone metastases has become common, reliable evidence is lacking. The optimal dose fractionation and irradiation field of PORT are unknown. This study aimed to characterize current patterns of practice regarding PORT and factors that affect dose fractionation. Members of the Japanese Radiation Oncology Study Group (JROSG) completed an internet-based survey. The survey was composed of questions to determine the prescription of various dose fractionations in each institution in 2017, describe dose fractionations in four hypothetical cases in which patients were treated with PORT for impending pathological fractures in a lower limb, and indicate irradiation fields each member would prefer. Case 1 was a patient with limited prognosis of non-small-cell lung cancer. Case 2 was a patient with a single oligometastasis. Case 3 was identical to case 1, except for the presence of a radio-resistant primary tumor, renal cell carcinoma. Case 4 was a patient with breast cancer with expected long-term prognosis. Radiation oncologists who recommended long-course RT in case 1 were asked to explain why they considered long-course RT over short-course RT (i.e., 8 Gy in 1 fraction or 20 Gy in 5 fractions). In total, 89 radiation oncologists from 68 institutions (50% of JROSG institutions) responded. Of the patients treated at these institutions, 151 were eligible for this study, except for 1 patient with an unknown prescribed dose fractionation. Among 22 different dose fractionations prescribed, the most common was 30 Gy in 10 fractions (n=75; 50%), followed by 20 Gy in 5 fractions (n=29; 19%). In all the hypothetical cases, the commonest prescribed regimen was 30 Gy in 10 fractions. For case 1, short-course RT (i.e., 8 Gy in 1 fraction or 20 Gy in 5 fractions) was preferred by 20% of the respondents (n=18). The following factors were most often cited as reasons for preferring long-course RT（i.e., >20 Gy in 5 fractions）: “local control” (54%), “incidence of re-irradiation” (34%), and “time until first increase in pain” (19%). Zero percent of the respondents for case 2, 8% for case 3, and 5% for case 4 prescribed short-course RT. Fractionated dose regimens of >30 Gy in 10 fractions were preferred in patients with oligometastasis (53%) compared to those with radio-resistant tumors (34%) and those with expected long-term prognosis (25%). For the irradiation field, 74% (n=66) of respondents recommended involvement of “the entire orthopedic prosthesis” for PORT. For PORT of long bone metastases, 30 Gy in 10 fractions for the entire orthopedic prosthesis is preferred currently in Japan. Higher dose regimens are preferred for patients with oligometastasis. Our results will guide the designing future clinical trials or research for bone metastases.

To determine the current practice of stereotactic irradiation (STI) for brain metastases in Japan by a questionnaire survey.

We evaluated the evolving structure of radiation oncology in Japan in terms of equipment, personnel, patient load, and geographic distribution to identify and overcome any existing limitations. From March 2011 to June 2013, the Japanese Society for Radiation Oncology conducted a questionnaire based on the Japanese national structure survey of radiation oncology in 2010. Data were analyzed based on the institutional stratification by the annual number of new patients treated with radiotherapy per institution. The estimated annual numbers of new and total (new plus repeat) patients treated with radiation were 211 000 and 251 000, respectively. Additionally, the estimated cancer incidence was 805 236 cases, with ~26.2% of all newly diagnosed patients being treated with radiation. The types and numbers of treatment devices actually used included linear accelerator (LINAC; n = 829), telecobalt (n = 9), Gamma Knife (n = 46), 60Co remote afterloading system (RALS; n = 28), and 192Ir RALS (n = 131). The LINAC system used dual-energy functions in 586 units, three-dimensional conformal radiotherapy functions in 663, and intensity-modulated radiotherapy (IMRT) functions in 337. There were 564 JASTRO-certified radiation oncologists, 959.2 full-time equivalent (FTE) radiation oncologists, 1841.3 FTE radiotherapy technologists, 131.3 FTE medical physicists, 121.5 FTE radiotherapy quality managers, and 649.6 FTE nurses. The frequency of IMRT use significantly increased during this year. To conclude, although there was a shortage of personnel in 2010, the Japanese structure of radiation oncology has clearly improved in terms of equipment and utility.

Purpose Whereas whole-brain radiotherapy (WBRT) has been the standard treatment of brain metastases (BMs), stereotactic radiosurgery (SRS) is increasingly preferred to avoid cognitive dysfunction; however, it has not been clearly determined whether treatment with SRS is as effective as that with WBRT or WBRT plus SRS. We thus assessed the noninferiority of salvage SRS to WBRT in patients with BMs. Patients and Methods Patients age 20 to 79 years old with performance status scores of 0 to 2-and 3 if caused only by neurologic deficits-and with four or fewer surgically resected BMs with only one lesion > 3 cm in diameter were eligible. Patients were randomly assigned to WBRT or salvage SRS arms within 21 days of surgery. The primary end point was overall survival. A one-sided α of .05 was used. Results Between January 2006 and May 2014, 137 and 134 patients were enrolled in the WBRT and salvage SRS arms, respectively. Median overall survival was 15.6 months in both arms (hazard ratio, 1.05; 90% CI, 0.83 to 1.33; one-sided P for noninferiority = .027). Median intracranial progression-free survival of patients in the WBRT arm (10.4 months) was longer than that of patients in the salvage SRS arm (4.0 months). The proportions of patients whose Mini-Mental Status Examination and performance status scores that did not worsen at 12 months were similar in both arms; however, 16.4% of patients in the WBRT arm experienced grade 2 to 4 cognitive dysfunction after 91 days postenrollment, whereas only 7.7% of those in the SRS arm did ( P = .048). Conclusion Salvage SRS is noninferior to WBRT and can be established as a standard therapy for patients with four or fewer BMs.

Chondral tumors in soft tissue are referred to as soft-tissue chondromas or extraskeletal chondromas, or as synovial chondromatosis if they arise in synovial tissue. We report the case of a 29-year-old man with synovial chondromatosis, also called synovial osteochondromatosis, which appeared in a solitary and extra-articular form. On magnetic resonance imaging (MRI) and computed tomography, the central portion of the tumor showed similar characteristics to bone marrow, despite the absence of any connection to adjacent bone. T2-weighted imaging displayed marked peripheral hyperintensity consistent with a cartilaginous area. These findings suggested the presence of enchondral ossification and were similar to those of skeletal osteochondroma, with the exception of the absence of attachment to bone. MRI is useful for distinguishing solitary synovial chondromatosis from other lesions, such as myositis ossificans, extraskeletal chondrosarcoma, and parosteal osteosarcoma.

Some recent studies reported that radiation dose of subventricular zone (SVZ) is associated with recurrence pattern and survival of malignant glioma patients. Glioma cells are believed to originate from cancer stem cells (CSCs) which are transformed neural stem cells in SVZ. Our previous study showed that SVZ-contiguous tumors at initial presentation are prone to recur from SVZ-contiguous region, and high dose radiation therapy is associated with decreased possibility of SVZ-contiguous recurrence. The purpose of this study was to evaluate the prognostic value of radiation dose to SVZ in malignant glioma patients. Consecutive 74 histologically proven malignant glioma patients who received radiation therapy at our hospital from 1992 to 2013 were included. Fifty-four of 74 patients' dosimetries were accessible. Radiation oncologists contoured SVZ on computed tomography to calculate the delivered radiation dose. Univariate and multivariate analyses were performed to examine the prognostic factors for progression free survival (PFS) and overall survival (OS). In multivariate analysis, we included 9 covariates into Cox proportional hazards model: age, sex, histology, extent of resection, ipsilateral SVZ dose, clinical target volume (CTV) volume, Karnofsky Performance Status (KPS), localization of the lesion, and MIB-1 index. Patients' median age was 60.5 years old (range, 5-83). There were 46 male and 28 female patients. There were 58 glioblastoma and 16 anaplastic astrocytoma patients. Fifty-seven patients received surgery. Prescription doses were 60 Gy and 80 Gy in 55 and 18 patients, respectively. Eighteen patients have no evidence of recurrence at the time of analysis. Median follow-up, PFS, and OS periods were 666, 428 and 802 days, respectively. The median of mean ipsilateral SVZ, contralateral SVZ, and CTV doses were 58.2, 44.1, and 60.4 Gy, respectively. On multivariate analysis only MIB-1 index was significant to OS and extent of surgery was significant to PFS. In a subgroup analysis of 58 glioblastoma patients, none of 8 factors was significant to OS while higher ipsilateral SVZ dose and less than total resection were associated with shorter PFS. Higher radiation doses to ipsilateral SVZ did not affect PFS or OS in malignant glioma patients on multivariate analysis. Higher radiation doses were associated with decreased PFS in glioblastoma patients.

IMPORTANCE It remains uncertain whether treatment with stereotactic radiosurgery (SRS) alone can be safely applied to all patient populations with 1 to 4 brain metastases (BMs) exhibiting heterogeneous prognoses. OBJECTIVE To investigate the feasibility of SRS alone for patients with different prognoses determined by the diagnosis-specific Graded Prognostic Assessment (DS-GPA). DESIGN, SETTING, AND PARTICIPANTS A secondary analysis (performed in September 2014) of the Japanese Radiation Oncology Study Group (JROSG) 99-1, a phase 3 randomized trial, comparing SRS alone and whole-brain radiotherapy (WBRT) + SRS conducted in 1999 to 2003. Among a total of 132 patients, 88 with non-small-cell lung cancer (NSCLC) and 1 to 4 BMs were included and poststratified by DS-GPA scores to avoid potential bias from BMs from different primary cancer types. The median follow-up time was 8.05 months. INTERVENTIONS The WBRT schedule was 30 Gy in 10 fractions over 2 to 2.5 weeks. The mean SRS dose was 21.9 Gy in SRS alone and 16.6 Gy in WBRT + SRS. MAIN OUTCOMES AND MEASURES The primary end point was overall survival (OS), and the secondary end points included brain tumor recurrence (BTR), salvage treatment, and radiation toxic effects. RESULTS Forty-seven patients had a favorable prognosis, with DS-GPA scores of 2.5 to 4.0 (26 SRS-alone and 21 WBRT + SRS [DS-GPA 2.5-4.0 group]), and 41 had an unfavorable prognosis, with DS-GPA scores of 0.5 to 2.0 (19 SRS-alone and 22 WBRT + SRS [DS-GPA 0.5-2.0 group]). Significantly better OS was observed in the DS-GPA 2.5-4.0 group in WBRT + SRS vs the SRS alone, with a median survival time of 16.7 (95% CI, 7.5-72.9) months vs 10.6 (95% CI, 7.7-15.5) months (P = .04) (hazard ratio [HR], 1.92; 95% CI, 1.01-3.78). However, no such difference was observed in the DS-GPA 0.5-2.0 group (HR, 1.05; 95% CI, 0.55-1.99) (P =.86). This benefit could be explained by the differing BTR rates, in that the prevention against BTR by WBRT had a more significant impact in the DS-GPA 2.5-4.0 group (HR, 8.31; 95% CI, 3.05-29.13) (P < .001) vs the DS-GPA 0.5-2.0 group (HR, 3.57; 95% CI, 1.02-16.49) (P = .04). CONCLUSIONS AND RELEVANCE Despite the current trend of using SRS alone, the important role of WBRT for patients with BMs from NSCLC with a favorable prognosis should be considered. Our findings should be validated through appropriately designed prospective studies.

A 68-year-old man came to our hospital because he got edema in his left leg. Abdominal CT scan revealed multiple liver metastasis and pelvic CT scan showed marked thickening of the rectal wall and the border with the bladder became indistinct. Colonoscopic examination revealed stenosis of the rectum without mucosal abnormality. Histological examination of boring biopsies from this lesion led to the suspicion adenocarcinoma. His condition turned worse and died 1 month later. From the autopsy findings we diagnosed advanced bladder cancer with direct rectal invasion. Because this metastasis form is very rare, we report on this case along with a review of the literature.

Purpose: To perform an individual patient data (IPD) meta-analysis of randomized controlled trials evaluating stereotactic radiosurgery (SRS) with or without whole-brain radiation therapy (WBRT) for patients presenting with 1 to 4 brain metastases.Method and Materials: Three trials were identified through a literature search, and IPD were obtained. Outcomes of interest were survival, local failure, and distant brain failure. The treatment effect was estimated after adjustments for age, recursive partitioning analysis (RPA) score, number of brain metastases, and treatment arm.Results: A total of 364 of the pooled 389 patients met eligibility criteria, of whom 51% were treated with SRS alone and 49% were treated with SRS plus WBRT. For survival, age was a significant effect modifier (P=.04) favoring SRS alone in patients <= 50 years of age, and no significant differences were observed in older patients. Hazard ratios (HRs) for patients 35, 40, 45, and 50 years of age were 0.46 (95% confidence interval [CI] = 0.24-0.90), 0.52 (95% CI = 0.29-0.92), 0.58 (95% CI = 0.35-0.95), and 0.64 (95% CI = 0.42-0.99), respectively. Patients with a single metastasis had significantly better survival than those who had 2 to 4 metastases. For distant brain failure, age was a significant effect modifier (P=.043), with similar rates in the 2 arms for patients <= 50 of age; otherwise, the risk was reduced with WBRT for patients >50 years of age. Patients with a single metastasis also had a significantly lower risk of distant brain failure than patients who had 2 to 4 metastases. Local control significantly favored additional WBRT in all age groups.Conclusions: For patients <= 50 years of age, SRS alone favored survival, in addition, the initial omission of WBRT did not impact distant brain relapse rates. SRS alone may be the preferred treatment for this age group. (C) 2015 Elsevier Inc.

This article reviews roles of imaging examinations in the management of community-acquired pneumonia（CAP）, imaging diagnosis of specific CAP and discrimination between CAP and noninfectious diseases. Chest radiography is usually enough to confirm the diagnosis of CAP, whereas computed tomography is required to suggest specific pathogens and to discriminate from noninfectious diseases. Mycoplasma pneumoniae pneumonia, tuberculosis, Pneumocystis jirovecii pneumonia and some cases of viral pneumonia sometimes show specific imaging findings. Peribronchial nodules, especially tree-in-bud appearance, are fairly specific for infection. Evidences of organization, such as concavity of the opacities, traction bronchiectasis, visualization of air bronchograms over the entire length of the bronchi, or mild parenchymal distortion are suggestive of organizing pneumonia. We will introduce tips to effectively make use of imaging examinations in the management of CAP.