For the secondary prevention of cardiovascular disease, comprehensive cardiac rehabilitation is required. This involves optimal medical therapy, education on nutrition and exercise therapy, and smoking cessation. Of these, efficient exercise therapy is a key factor. A highly effective training protocol is therefore warranted, which requires a high rate of compliance. Although moderate-intensity continuous training has been the main training regimen recommended in cardiac rehabilitation guidelines, high-intensity interval training has been reported to be more effective in the clinical and experimental setting from the standpoint of peak oxygen uptake and central and peripheral adaptations. In this review, we illustrate the scientific evidence for high-intensity interval training. We then verify this evidence and discuss its significance and the remaining issues.

Objective The purpose of our study was to differentiate the imaging findings of patients with spontaneous intramural intestinal hemorrhage (SIIH) from those with acute mesenteric ischemia (AMI) after abdominal computed tomography (CT) survey in the emergency department.

Methods We retrospectively included 83 patients diagnosed with SIIH or AMI after abdominal CT.

Results The mean ages of 30 SIIH patients and 53 AMI patients were 74.4±14.6 years and 75.8±11.2 years, respectively. Patients with SIIH had significantly thicker maximal intestinal wall thickening (14.8±3.9 vs. 10.9 ±4.1, p<0.001), a lower rate of ileum involvement (26.7% vs. 77.4%, p<0.001) and a higher rate of ascites (96.7% vs. 64.2%, p<0.001) compared with patients with AMI. Neither pneumatosis intestinalis (p<0.001) nor portomesenteric gas (p<0.01) were detected in SIIH patients but were observed in AMI patients. A receiver-operating characteristic (ROC) curve analysis showed that the optimal cut-off value for maximal intestinal wall thickening between groups was 10.4 mm and the area under the ROC curve between groups was 0.752 (p<0.0001). A multiple logistic regression analysis showed that the independent predictors of SIIH were non-involvement of the ileum (odds ratio, OR, 6.998; p=0.001), maximal intestinal wall thickening ≥10.4 mm (OR, 5.748; p=0.040) and ascites (OR, 13.348; p=0.023). The area under the ROC curve for the model was 0.854 (p<0.001).

Conclusion The independent predictors of SIIH from AMI after abdominal CT in acute abdominal patients include non-involvement of the ileum, intestinal wall thickening ≥10.4 mm, and ascites.

Objective The purpose of this study was to investigate the relationship between circulating soluble fibrinogen-like protein 2 (sFGL2) concentrations and the severity of coronary artery disease (CAD) in patients who underwent first-time angiography for suspected CAD.

Methods Serum sFGL2 concentrations were measured in 102 consecutive patients by an enzyme-linked immunosorbent assay (ELISA). The number of circulating CD4⁺CD25⁺CD127^low T regulatory cells (Tregs) was determined by flow cytometry and effecter cytokines, including transforming growth factor-β1 and interleukin-10 (IL-10), were also evaluated by an ELISA. Associations between sFGL2 and Tregs with angiographic indexes of the severity of CAD (i.e., number of diseased vessels and the modified Gensini score) were estimated.

Results The sFGL2 levels in patients with angiographically confirmed CAD were significantly lower than those in patients with normal coronary arteries (26.95±8.53 vs. 9.88±5.46 ng/mL, p<0.001). Significant correlations were observed between the serum sFGL2 level and number of diseased vessels (r=-0.860, p<0.001) and modified Gensini score (r=-0.833, p<0.001). Using a multivariate analysis, the serum sFGL2 level was independently associated with the presence and severity of CAD.

Conclusion The serum sFGL2 levels are significantly lower in the presence of CAD and correlate with the severity of the disease. Further clinical studies are needed to confirm the use of sFGL2 as a biomarker for the detection and extent of CAD.

Objective The recognition of clinical symptoms is critical to developing an effective therapeutic strategy for aortic valve stenosis (AS). Although AS is common, little is known about the factors influencing the natural history of AS patients who are 80 years of age older in advanced aging societies. We investigated the natural history and indications for valve procedures in AS patients of 80 years of age or older.

Methods The medical records of 108 consecutive AS patients (moderate grade or higher) who are 80 years of age or older (mean age, 84.2±3.9 years; female, 65 patients) were reviewed to investigate their symptoms, the development of congestive heart failure, the incidence of referral for aortic valve replacement and death. The median duration of follow-up was 9 months (interquartile range, 2 to 25 months).

Results The probability of remaining free of events (valve replacement and death) was 29±13% in all patients. There was no significant difference in the aortic valve area of the symptomatic and asymptomatic patients (0.85±0.28 cm² vs. 0.88±0.25 cm², p=0.59). The aortic valve (AV) velocity and AV area index were predictors of subsequent cardiac events (p<0.05).

Conclusion The severity of AS was the only factor to affect the prognosis of AS patients who were 80 years old of age or older. It is necessary to frequently monitor the subjective symptoms of such patients and to objectively measure the AV area.

Objective Ischemia-mediated oxidative stress and inflammation have been reported to be important contributors to the pathogenesis of polycystic ovary syndrome (PCOS). Ischemia-modified albumin (IMA) is a novel marker generated under ischemic and oxidative conditions and may reflect disease activity in distinct disease states. Therefore, we investigated whether the serum IMA levels are affected in infertile PCOS patients.

Methods Forty-six patients with infertile PCOS, 30 patients with unexplained infertility, and 31 age- and body mass index (BMI)-matched controls were included in this cross-sectional study. Biochemical parameters, serum IMA levels, and their correlations with serum testosterone and insulin resistance were determined for each subject.

Results In patients with infertile PCOS, the serum IMA levels were significantly elevated (p=0.003) compared with unexplained infertility patients and controls. A correlation analysis suggested that the IMA levels only correlated with the serum free testosterone levels in PCOS patients (r=0.43, p=0.028).

Conclusion Elevations in the serum IMA levels in infertile PCOS patients may suggest a possible additional role of oxidative stress mechanisms in disease pathophysiology. Moreover, correlation between serum IMA and testosterone levels may influence the quality of oocytes via alterations in the balance of critical follicular fluid factors in the follicular microenvironment.

Objective There is no definite consensus regarding the treatment and guidance for individuals with high-normal fasting plasma glucose levels (FPG;100-109 mg/dL). The present study aimed to determine the risk factors for future diabetes in Japanese people with high-normal FPG.

Methods Retrospective cohort studies were conducted from 2008 to 2012, including 15,097 individuals who underwent medical examinations. First, the participants were divided into normal FPG (n=13,065) and high-normal FPG (n=2,032) groups to compare the diabetes incidence. Second, the high FPG group was divided into diabetes onset (n=133) and non-diabetes onset (n=1,899) groups to compare the baseline values. Third, to determine the risk factors for future diabetes in the high-normal FPG group, multivariate analyses were conducted.

Results The cumulative incidence during the mean follow-up of 4 years was 94/13,065 (0.72%) and 133/2,032 (6.55%) in the normal FPG and high-normal FPG groups, respectively. Within the high-normal FPG group, the baseline body mass index, waist circumference, triglycerides, FPG, alanine aminotransferase (ALT), and gamma-glutamyl transferase were significantly higher and high-density lipoprotein cholesterol (HDL-C) was significantly lower in the diabetes onset group than in the non-diabetes onset group. Obesity, abdominal obesity, hypertriglyceridemia, low HDL-C, and high ALT were significant risk factors for diabetes according to a multivariate analysis.

Conclusion The high-normal FPG group had a higher risk of diabetes than the normal FPG group, particularly when accompanied with obesity, abdominal obesity, hypertriglyceridemia, low HDL-C, and high ALT. Thus, this high risk group should receive appropriate guidance for lifestyle changes to avoid developing diabetes at an early stage.

Objective The long-term clinical course and prognosis of patients with chronic eosinophilic pneumonia (CEP) including factors predictive of the relapse of CEP have not been fully investigated. The aim of the present study was to investigate these issues.

Methods We retrospectively investigated the rate of relapse and prognosis in 73 patients diagnosed as having CEP.

Results Systemic corticosteroid therapy was administered at a prednisolone dose of 29.4±7.6 mg/day. During a median follow-up period of 1,939 days, 27 patients suffered from relapse of CEP. Two patients developed steroid-induced diabetes mellitus, and 1 patient developed pulmonary nontuberculous mycobacteriosis. Five patients died; however, none died of CEP. A history of smoking was the only independent negative risk factor for relapse of CEP [hazard ratio, 0.37 (0.14-0.98)].

Conclusion Patients with CEP frequently relapse. During the follow-up, metabolic and infectious complications under prolonged corticosteroid therapy are problematic. A history of smoking was a negative factor for predicting the risk of CEP relapse.

Objective The objective of this study was to explore the perspective of hematologists and their patients regarding the management of adult chronic immune thrombocytopenia (ITP).

Methods This was a multi-center, questionnaire-based, cross-sectional study conducted between 2012 and 2013 throughout Japan.

Patients Hematologists, members of the Japanese Society of Hematology in 171 institutions, and their patients were invited to participate in this study. The hematologists were mainly asked about their treatment strategies, while patients were asked about their opinion of the applied treatments, treatment effect, impact on their quality of life (QOL), and treatment satisfaction.

Results Questionnaires from 204 hematologists and 213 patients were collected. One hundred sixty hematologists (78.4%) started treatment based on the patient's platelet count. Corticosteroids were considered to be the most effective treatment (44.1%). Forty-six percent of hematologists responded that treatment would be started after the platelet count fell below 20×10⁹/L with bleeding symptoms, compared to 62.9% for patients with no bleeding symptoms. A platelet count of 50×10⁹/L or lower was acceptable for 94.0% of hematologists and 66.8% of patients. Fatigue was most frequently experienced by patients (44.6%). Patients also experienced psychological symptoms (feeling of anxiety or depressive mood: 29.1%, labyrinthitis: 23.5%). While 70.6% of hematologists assumed that the patient QOL was impaired to a moderate to substantial degree, the QOL was impaired in 34.3% of patients.

Conclusion A substantial gap which exists between hematologists and their patients highlights a need for better understanding of potential conflicts for establishing effective strategies for ITP management.

Objective We evaluated the short-term effects of smoking cessation therapy with varenicline on the lung function.

Methods In this study, 81 subjects received 12 weeks of smoking cessation therapy with varenicline. No changes were made to any previously prescribed medications. A physical examination, blood sampling, and spirometry were performed at the first and last visit. Spirometric lung ages were calculated by a formula based on height and the forced expiratory volume in 1 second. The success group comprised 62 subjects who attained 4-week continuous abstinence confirmed by exhaled carbon monoxide testing; whereas the failure group comprised 19 subjects who did not attain this result. However, the number of cigarettes consumed per day was reduced in all subjects of the failure group.

Results The spirometric lung ages significantly improved over the 12-week period in the success group (69.8±24.7 vs. 66.9±24.1, p<0.01); however, spirometric lung ages significantly deteriorated in the failure group (70.5±25.5 vs. 73.7±26.9, p<0.01). The effect sizes (Cohen's d) of spirometric lung age in the success and failure groups were 0.37 and 0.81, respectively. The post-hoc statistical power of the spirometric lung age in the success and failure groups was 0.83 and 0.91, respectively. According to a multiple regression analysis, success in smoking cessation exhibited an independent association with the difference in spirometric lung age between the last visit and baseline (p<0.01).

Conclusion These findings suggest that successful smoking cessation therapy with varenicline improves the spirometric lung age in the short term.

Objective A significant number of Japanese cancer patients refuse to have central venous (CV) ports implanted. The aim of this study is to investigate the experiences of patients prior to and after CV port implantation, as well as their expectations regarding the use of CV ports.

Methods This study was carried out at Osaka Medical Center for Cancer and Cardiovascular Diseases from October 20, 2014, to January 16, 2015. Data were collected using a questionnaire developed by the researchers, and various statistical analyses were performed.

Results Among the 50 patients who participated in this study, the CV port was implanted due to poor venous access in 18 (36%). The proportion of patients who were anxious before the port implantation was significantly higher among the patients in whom CV ports were implanted due to poor venous access than among those in whom CV ports were implanted for other reasons. All patients exhibited high satisfaction levels, regardless of the reason for CV port implantation. CV port-related discomfort was most commonly associated with seat belts.

Conclusion The patients exhibited high satisfaction levels regardless of the reason for CV port implantation. However, the patients that exhibited poor venous access often experienced anxiety before the implantation of the port, so it is important to provide such patients with sufficient information prior to port implantation. In order to improve the quality of life of patients with CV ports, medical staff should give special consideration to discomfort experienced by patients that are wearing seat belts.

We herein describe a rare case of a 24-year-old man who presented with severe epigastralgia after consuming a considerable amount of broiled meat. Computed tomography revealed a cystic lesion adjacent to the distal stomach, with high intensity on T2-weighted magnetic resonance imaging. Upper endoscopy showed a cystic mass measuring 6 cm in diameter, mimicking a submucosal tumor adjacent to the pyloric valve, with duodenum invagination, characteristic of ball valve syndrome. Endoscopic ultrasonography showed that the lesion was contiguous through the first to the third layer of the stomach. Therefore, we performed distal gastrectomy. Pathology showed that the lesion was a gastric duplication cyst without malignancy.

A 59-year-old man was admitted to our hospital for treatment of a 45 mm pancreatic mass found during a medical examination. Endoscopic ultrasound-guided fine-needle aspiration cytology showed polygonal cells with pseudopapillary structures. The tumor cells were positive for nuclear/cytoplasmic β-catenin and CD10, and negative for chromogranin A. After a tentative diagnosis of a solid pseudopapillary neoplasm, middle pancreatectomy was performed. Histologically, polygonal cells with abundant eosinophilic cytoplasm formed in the trabeculae and were immunohistochemically positive for HepPar1 and protein induced by vitamin K absence or antagonist-II. The tumor was finally diagnosed to be pancreatic hepatoid carcinoma. No recurrence occurred for 12 months, even without adjuvant chemotherapy.

The ratio of the number of patients with non-alcoholic steatohepatitis (NASH) to the total number of patients with liver dysfunction has increased in many countries around the world. Liver dysfunction is also caused by multiple blood transfusions in patients with leukemia and other hematological diseases, with liver dysfunction often accompanied by secondary hemochromatosis. This study describes a 25-year-old man with secondary hemochromatosis combined with NASH. Magnetic resonance imaging was useful for visualizing the distributions of both iron and fat in the liver of this patient in order to make a differential diagnosis and to evaluate the effect of treatment.

A 76-year-old woman was hospitalized repeatedly due to unexplained heart failure. On admission, she had hypertensive acute heart failure. Her symptoms disappeared promptly after the initial treatment; however, her systolic blood pressure remained at over 160 mmHg despite her taking three antihypertensive drugs. Closer examination revealed hemodynamically significant right renal artery stenosis and a lack of left kidney function. We performed percutaneous transluminal renal angioplasty using CO₂ angiography in combination with iodinated contrast agents. The patient's renal function and blood pressure improved, however, CO₂ gas-induced mild ischemic colitis occurred. We discuss the possibility of the use of combined iodinated contrast angiography and CO₂ angiography to avoid contrast-induced nephropathy and the complications peculiar to CO₂ angiography.

We herein report a case of a 52-year-old woman who presented with a history of recurrent palpitations that occurred during swallowing solid food. On a Holter electrocardiogram, paroxysmal atrial tachycardias (PATs) were detected while eating. We mapped the right atrium (RA) with a multipolar mapping catheter while she swallowed a rice ball and it was revealed that the earliest endocardial breakthrough was on the anterior septal side near the superior vena cava junction of the RA. We successfully eliminated PAT at both the site in the RA and the adjacent right superior pulmonary vein ostium. After ablation, no PAT was documented while eating.

Cardiopulmonary abnormalities are often present in patients with liver diseases. We herein report a case of congenital portosystemic shunts complicated by hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). A 57-year-old woman complained of dyspnea and was subsequently diagnosed with HPS and PoPH caused by congenital portosystemic shunts. Although shunt closure by transcatheter embolization was successfully performed, her dyspnea worsened and pulmonary artery pressure and pulmonary vascular resistance elevated. Conventional vasodilator therapy was started, resulting in an improvement of pulmonary hypertension (PH). In some patients with congenital portosystemic shunts, shunt closure could exacerbate PH, and vasodilator therapy may be effective.

A 40-year-old man presented with Cushing's syndrome due to bilateral adrenal hyperplasia with multiple nodules. Computed tomography scan results were atypical demonstrating an enlargement of the bilateral adrenal glands harboring multiple small nodules, but the lesion was clinically diagnosed to be primary pigmented nodular adrenocortical disease (PPNAD) based on both endocrinological test results and his family history. We performed bilateral adrenalectomy and confirmed the diagnosis histologically. An analysis of the patient and his mother's genomic DNA identified a novel mutation in the type Iα regulatory subunit of protein kinase A (PRKAR1A) gene; p.E17X (c.49G>T). This confirmed the diagnosis of PPNAD which is associated with Carney Complex.

A 73-year-old woman was diagnosed with pulmonary Mycobacterium avium complex (MAC) infection and received no treatment. Disease progression was evident one year later with the development of myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) titers and systemic symptoms of a fever, polyarthritis, purpura, and rapidly progressive glomerulonephritis. Her symptoms did not improve with antibiotic treatment. A renal biopsy revealed crescentic glomerulonephritis with immunodeposition. According to these findings, she was diagnosed with ANCA-associated vasculitis (AAV) superimposed on infection-related glomerulonephritis (IRGN). Although there was a risk of aggravating an underlying infection, the combination therapy of corticosteroid and antibiotics improved AAV, IRGN, and even the lung radiological findings. To the best of our knowledge, this is the first case of AAV and IRGN secondary to pulmonary MAC infection.

Acute kidney injury (AKI) is caused by diverse pathologies, although it may occasionally result from concurrent renal efflux disturbances. We herein describe a case of AKI in a patient complicated by renal cell carcinoma (RCC) with renal vein and inferior vena cava (IVC) involvement. A neoplastic thrombus which disrupted the blood flow in the renal vein appeared to play a role in the rapid decline in the renal function. Such a scenario has rarely been mentioned in the previous literature describing the cases of RCC complicated by AKI. Concerns regarding the diagnostic and therapeutic strategies for RCC are also discussed.

Clinicians generally suspect pulmonary tumor embolism (PTE) with uncontrolled carcinomas which often spread to lungs. We, however, experienced an autopsy case of diffuse microscopic PTE despite controlled liver metastases of transitional cell carcinoma (TCC). A 66-year-old man with progressing respiratory symptoms showed almost normal chest findings on computed tomography. Although liver metastases were successfully shrunk by chemotherapy, the patient died from aggressive respiratory failure. An autopsy revealed small pulmonary vessels showing diffuse tumor emboli. TCC can cause PTE even if liver metastases are controlled. We must therefore be aware that PTE can manifest as respiratory symptoms without any computed tomography findings.

We herein report a case of a 67-year-old woman previously treated with erlotinib for adenocarcinoma with an epidermal growth factor receptor (EGFR) mutation in exon 19, which rapidly developed to progressive symptomatic leptomeningeal carcinomatosis. The primary tumor and lung metastases also worsened and the performance status (PS) score declined to 3. With a re-biopsy from the pulmonary metastases, the T790M mutation was detected by the cobas EGFR mutation test, but not the cycleave test, although an exon 19 deletion was detected by both of the tests. A week after afatinib initiation, the neurological symptoms disappeared and the PS improved to 1 with a radiological response in all disease sites. Chest physicians should consider the use of afatinib for patients with leptomeningeal carcinomatosis from 1st-generation EGFR-TKI resistant adenocarcinoma, regardless of the PS score and the presence of the T790M mutation in the extracranial lesion.

Desmoplastic small round cell tumor (DSRCT) is an aggressive mesenchymal tumor which primarily affects the abdomen. Even a multimodal approach rarely achieves durable remission and the optimal therapy for extended disease is unknown. We herein describe a rare case of DSRCT arising from the pleura in a 32-year-old man. Initial therapy, which included chemotherapy, surgery and radiotherapy, achieved a partial response for only two months. Although salvage chemotherapies had no effect, pazopanib treatment shrank the tumors and was well-tolerated on an outpatient basis. From the viewpoint of quality of life, pazopanib may therefore be a good therapeutic option for this aggressive disease.

We herein report a case of IgG4-related lung disease (IgG4-RLD) associated with autoimmune hemolytic anemia (AIHA). A 73-year-old Japanese female visited our hospital for an examination following an abnormal chest X-ray in 1999. She was diagnosed with bronchiolitis and AIHA, and treatment with prednisolone was started. After seven years, she visited our department due to a cough. Chest computed tomography (CT) demonstrated focal consolidation with ground-glass attenuations and thickened bronchial walls in the bilateral lungs. She was clinically diagnosed and treated for bronchial asthma. CT findings had shown no changes, and a lung biopsy was performed using video-assisted thoracic surgery at eleven years from the first diagnosis of AIHA. The pathological findings demonstrated the presence of peribronchovascular lymphoplasmacytic infiltrates with stromal fibrotic changes, admixed with many IgG4-positive plasma cells. Furthermore, the patient's serum IgG4 level was high, and her CT findings did not show any obvious abnormal findings in the any organs other than the lungs. She was diagnosed with IgG4-RLD based on the findings. We believe that this case report of IgG4-RLD associated with AIHA is clinically helpful for a better understanding of these diseases, although there are five reported cases of IgG4-related disease associated with AIHA.

A 47-year-old woman with ulcerative colitis (UC) was admitted to our hospital for renal dysfunction and progressive anemia. Colonoscopy revealed intestinal lesions and pathological findings showed intravascular large B-cell lymphoma (IVLBCL). According to the polymerase chain reaction analysis of sequential rectal specimens, we concluded that she suffered from intestinal BCL, not UC. After chemotherapy, her renal function progressed to nephrotic syndrome. The pathological findings of renal biopsy specimens indicated membranoproliferative glomerulonephritis (MPGN). Chemotherapy was continued and led to the remission of BCL and MPGN. We herein describe the first case of intestinal IVLBCL mimicking UC with secondary MPGN.

Patients with cluster headaches occasionally fail to respond to conventional preventive treatments. We herein report a case of a patient with a cluster headache in which the symptoms were refractory to conventional preventive treatments except for high-dose glucocorticoids. The headache attacks occurred daily while sleeping, thus the patient suffered from insomnia. Ramelteon, a selective melatonin receptor agonist and a member of a new class of insomnia therapies, completely suppressed the attacks during sleep and provided rapid relief from insomnia. This is the first English case report to describe the efficacy of ramelteon as a preventive treatment for cluster headaches.

The nationwide introduction of a Japanese encephalitis (JE) vaccine has contributed to a reduction in the annual infection rate of JE in Japan. However, the current neutralizing antibody prevalence ratio in Japan is approximately 20% in children 3-4 years of age and in people in their forties and fifties. We herein report a man with JE who was definitively diagnosed by multi-virus real-time polymerase chain reaction employing biopsied brain tissue and serological examinations. JE should be kept in mind when a patient has severe encephalitis of unknown etiology. In order to protect the susceptible population from JE, vaccination is recommended, especially for children and middle-aged people.

A 57-year-old Japanese woman with a 5-year history of rheumatoid arthritis (RA) was admitted to our hospital for an evaluation of nephrotic range proteinuria (4.8 g/day). A renal biopsy led to the diagnosis of amyloidosis according to strong positivity for Congo red staining and the detection of microfibrillar structures on electron microscopy that were negative for AA and positive for kappa light chain. Combination therapy with high-dose melphalan and autologous stem cell transplantation was performed according to the regimen for AL amyloidosis. Her proteinuria and RA subsided, but relapsed after 3 years. This is the first report regarding kappa light chain amyloidosis in an RA patient.

Multifocal fibrosclerosis (MFS), which causes systemic and chronic connective tissue inflammation, has been associated with IgG4 and regarded as an identical entity with "IgG4-related disease (IgG4-RD)". Although a few cases of MFS mimicking IgG4-RD histopathologically, despite the absence of a serum IgG4 elevation and IgG4-positive plasma cell infiltration, have been reported, there is, so far, little information regarding such exceptional cases. We herein demonstrate a case of non-IgG4-related MFS presenting with periaortitis and parotiditis, whose histological findings were consistent with IgG4-RD despite the absence of elevated serum and tissue IgG4 levels.

We herein report a case of Penicillium marneffei infection (PMI) in a Japanese man who was infected with human immunodeficiency virus-1 (HIV-1), who was diagnosed on the basis of a bone marrow culture and who was effectively treated with itraconazole. Our review of the PMI cases reported in Japan suggests that increased serum (1→3)-β-D-glucan levels are a useful diagnostic tool in cases of suspected PMI.

We herein encountered a case of abdominal wall dermatofibrosarcoma protuberans (DFSP) that developed pulmonary and pancreatic metastases 5 years after complete resection. Because specific rearrangements of the platelet-derived growth factor beta (PDGFB) locus by a novel fluorescence in situ hybridization method was detected, the patient was treated with imatinib mesylate at 400 mg/day. A partial response was achieved by imatinib without any specific toxicity. Although metastatic DFSP is an extremely rare disease, an evaluation of PDGFB fusion is essential and imatinib mesylate should be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP.