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Micronodular thymoma with lymphoid stroma (MNT) is a rare thymic epithelial neoplasm subtype characterized by a micronodular tumor cell growth pattern and abundant lymphoid stroma. Micronodular thymic carcinoma with lymphoid stroma (MNCA) is considered as a malignant counterpart of MNT and exhibits a growth pattern similar to that of MNT but has histologic features reminiscent of thymic squamous cell carcinoma, such as cytologic atypia and CD5 and CD117 immunoexpression. Although both MNT and MNCA are characterized by abundant lymphoid stroma, it remains unknown whether there are differences in infiltrating lymphocytes between MNT and MNCA. We analyzed the immune microenvironment profile in eight MNT and three MNCA cases. The cell density of CD8-positive T cells was significantly higher in MNT than in MNCA, whereas that of FOXP3-positive T cells was significantly higher in MNCA than in MNT. There was no significant difference in the cell density of programmed death protein 1-positive T cells and programmed death ligand 1 expression between the MNT and MNCA cases. Our findings indicated that the immune microenvironment of MNCA differed from that of MNT and, compared with the T-cell profile of MNT, that of MNCA was more suppressive to patients' antitumor immune response.

Background Lung fibrosis is a serious life-threatening condition whose manifestation varies according to the localization and characteristics of fibroblasts, which are considered heterogeneous. Therefore, to better understand the pathology and improve diagnosis and treatment of this disease, it is necessary to elucidate the nature of this heterogeneity and identify markers for the accurate classification of human lung fibroblast subtypes. Methods We characterized distinct mouse lung fibroblast subpopulations isolated by fluorescence-activated cell sorting (FACS) and performed microarray analysis to identify molecular markers that could be useful for human lung fibroblast classification. Based on the expression of these markers, we evaluated the fibroblast-like cell subtype localization in normal human lung samples and lung samples from patients with idiopathic pulmonary fibrosis (IPF). Results Mouse lung fibroblasts were classified into Sca-1high fibroblasts and Sca-1low fibroblasts by in vitro biological analyses. Through microarray analysis, we demonstrated CD248 and integrin alpha-8 (ITGA8) as cell surface markers for Sca-1high fibroblasts and Sca-1low fibroblasts, respectively. In mouse lungs, Sca-1high fibroblasts and Sca-1low fibroblasts were localized in the collagen fiber-rich connective tissue and elastic fiber-rich connective tissue, respectively. In normal human lungs and IPF lungs, two corresponding major fibroblast-like cell subtypes were identified: CD248highITGA8low fibroblast-like cells and CD248lowITGA8high fibroblast-like cells, localized in the collagen fiber-rich connective tissue and in the elastic fiber-rich connective tissue, respectively. Conclusion CD248highITGA8low fibroblast-like cells and CD248lowITGA8high fibroblast-like cells were localized in an almost exclusive manner in human lung specimens. This human lung fibroblast classification using two cell surface markers may be helpful for further detailed investigations of the functions of lung fibroblast subtypes, which can provide new insights into lung development and the pathological processes underlying fibrotic lung diseases.

Objectives: Using a murine model of systemic Kawasaki disease (KD)–like vasculitis induced by Candida albicans cell–wall–derived mannan · β–glucan · protein complexes, the objective was to elucidat...

Background/Objective To examine tenascin C (TN-C) expression in coronary artery lesions (CALs) and myocardial lesions (MLs) in Kawasaki disease (KD). Methods and Results Twenty-five KD autopsy cases (post-KD-onset range of 6 days to 17 years) were examined in this study. Time-course analysis based on the disease day was performed of the histological findings for the CALs and MLs, as well as the localization and intensity of expression of TN-C. TN-C expression was observed to coincide with the areas where inflammatory cell infiltration was present in both coronary arteries and myocardium during the acute stage of KD, and the intensity of its expression correlated with the degree of inflammation. Obvious TN-C expression persisted in the thickened intima and media of CALs even after Disease Day 27. However, in spite of the presence of inflammatory cell infiltration, TN-C expression became weaker in the adventitia and surrounding connective tissue. After 8 months or more, TN-C was not expressed in the vasculitis scars of most cases, but expression was observed around newly formed vessels in the thickened intima and around recanalized vessels after thrombotic occlusion. Conclusions The findings suggest a correlation between the degree of inflammation and TN-C expression in the cardiovascular lesions of acute–stage Kawasaki disease.

Granulocyte-colony stimulating factor (G-CSF) producing tumors are rare diseases and occur in various organs. However, due to the rarity of these tumors, the precise biologic characteristics and optimal therapeutic strategies are largely unknown. Previous studies have shown the extremely high malignant potential and aggressive clinical features of G-CSF producing tumors, regardless of the histologic type and tumor location. We present the case of a 59-year-old female who had large tumor in extrahepatic bile duct. She underwent surgery and adjuvant chemotherapy. Then, she also received gemcitabine and cisplatin combination chemotherapy for metastatic lesions. The therapy showed a certain effect and she survived for 17 months after surgery. To the best of our knowledge, this is the first report of multidisciplinary treatment with surgery and systemic chemotherapy for G-CSF producing extrahepatic bile duct cancer. This study highlights the potential of multidisciplinary treatment even for this lethal disease condition.

Purpose The differential diagnosis of interstitial lung diseases (ILDs), particularly idiopathic pulmonary fibrosis (IPF) versus other non-IPF ILDs, is important for selecting the appropriate treatment. This retrospective study aimed to explore the utility of gremlin-1 for the differential diagnosis. Methods Serum gremlin-1 concentrations were measured using an ELISA in 50 patients with IPF, 42 patients with non-IPF ILD, and 30 healthy controls. The baseline clinical data, including pulmonary functions, prognosis, and three serum biomarkers (Krebs von den Lungen-6 [KL6], surfactant protein-D [SP-D], and lactate dehydrogenase [LDH]), were obtained through a medical record review for analyzing their associations with serum gremlin-1 concentrations. To evaluate the origin of gremlin-1, we performed immunostaining on lung sections. Results Serum gremlin-1 concentrations were significantly higher in patients with IPF (mean concentration, 14.4 ng/mL), followed by those with non-IPF ILD (8.8 ng/mL) and healthy controls (1.6 ng/mL). The area under the curve for IPF versus non-IPF ILDs was 0.759 (95% confidence interval, 0.661–0.857), which was superior to that of KL6/SP-D/LDH. The sensitivity and specificity for gremlin-1 (cutoff, 10.4 ng/mL) was 72 and 69%, respectively. By contrast, serum gremlin-1 concentrations were not associated with the pulmonary functions nor the prognosis in all patients with ILDs. In immunostaining, the gremlin-1 was broadly upregulated in IPF lungs, particularly at myofibroblasts, bronchiolar/alveolar epithelium, and CD163-positive M2-like macrophages. Conclusions Gremlin-1 may be a useful biomarker to improve the diagnostic accuracy for IPF compared to non-IPF ILDs, suggesting a role of this molecule in the pathogenesis of IPF.

We present an extremely rare case of an alpha-fetoprotein (AFP)-producing gastric so-called carcinosarcoma with enteroblastic differentiation in a 74-year old man. After detecting a type 1 (polypoid) tumor (40 × 35 mm) in the upper stomach, the patient underwent total gastrectomy and lymphadenectomy. Microscopically, the tumor comprised a fetal gut-like adenocarcinoma component, common tubular adenocarcinoma component, and sarcomatous component. The fetal gut-like adenocarcinoma component was positive for AFP, GPC3, SALL4, and CLDN6, whereas the common tubular adenocarcinoma component was positive for SALL4, but negative for AFP, GPC3, and CLDN6. The sarcomatous component was composed of atypical spindle cells and was negative for AE1/3, AFP, and CLDN6, but part of the sarcomatous component was positive for SALL4 and GPC3. Multiple liver metastases appeared, and the patient died fifteen months after the surgery. To our knowledge, this is the first report describing the immunohistochemical characteristics of gastric carcinosarcoma with enteroblastic differentiation.

Background: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. Objective: We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. Methods: A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. Results: Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. Conclusion: Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.

Background Spontaneous pneumothorax is a complication that occurs in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD); however, few studies on the clinical implications of pneumothorax for patients with CTD-ILD have been performed. Objectives This study aimed to investigate the incidence and prognostic significance of pneumothorax and the risk factors for its onset in patients with CTD-ILD. Methods This study included 140 consecutive patients with CTD-ILD. Clinical characteristics, laboratory findings, pulmonary function test results, and chest high-resolution computed tomography (HRCT) images were retrospectively evaluated. Results A total of 18 patients (12.9%) developed pneumothorax during their clinical course. The cumulative incidence of pneumothorax from the time of CTD-ILD diagnosis was 6.5%, 8.7%, and 11.3% at 1, 3, and 5 years, respectively. The 10-year survival rate was significantly lower in patients with pneumothorax (29.6%) than that in those without pneumothorax (81.3%). The development of pneumothorax was significantly associated with poor prognosis (HR 22.0; p < 0.010). Furthermore, a lower body mass index, greater extent of reticular abnormalities on HRCT, and administration of methylprednisolone pulse therapy were significantly associated with the development of pneumothorax. Conclusion Pneumothorax is a serious complication in the clinical course of patients with CTD-ILD and the onset of pneumothorax predicts a poor outcome.

A 64-year-old man was admitted to our hospital with an abnormal chest shadow. The patient was a current-smoker and had a past illness of autoimmune pancreatitis with a high serum level of IgG4, 348 mg/dL. Chest CT showed upper-lobe emphysema, and lower-lobe reticulation with honeycombing, suggestive of combined pulmonary fibrosis with emphysema (CPFE). Surgical lung biopsy was revealed a usual interstitial pneumonia pattern with marked infiltration of IgG4-positive plasma cells. The patient was diagnosed with IgG4 related disease (IgG4-RD) presenting with CPFE. Pulmonary manifestation was improved by corticosteroid therapy. IgG4-RD may be an underlying condition in patient with CPFE. Keywords: Combined pulmonary fibrosis and emphysema (CPFE), IgG4-related disease (IgG4-RD), Usual interstitial pneumonia (UIP)

Although differentiation of lung fibroblasts into α-smooth muscle actin (αSMA)-positive myofibroblasts is important in the progression of idiopathic pulmonary fibrosis (IPF), few biomarkers reflecting the fibrotic process have been discovered. We performed microarray analyses between FACS-sorted steady-state fibroblasts (lineage (CD45, TER-119, CD324, CD31, LYVE-1, and CD146)-negative and PDGFRα-positive cells) from untreated mouse lungs and myofibroblasts (lineage-negative, Sca-1-negative, and CD49e-positive cells) from bleomycin-treated mouse lungs. Amongst several genes up-regulated in the FACS-sorted myofibroblasts, we focussed on Ltbp2, the gene encoding latent transforming growth factor-β (TGF-β) binding protein-2 (LTBP2), because of the signal similarity to Acta2, which encodes αSMA, in the clustering analysis. The up-regulation was reproduced at the mRNA and protein levels in human lung myofibroblasts induced by TGF-β1. LTBP2 staining in IPF lungs was broadly positive in the fibrotic interstitium, mainly as an extracellular matrix (ECM) protein; however, some of the αSMA-positive myofibroblasts were also stained. Serum LTBP2 concentrations, evaluated using ELISA, in IPF patients were significantly higher than those in healthy volunteers (mean: 21.4 compared with 12.4 ng/ml) and showed a negative correlation with % predicted forced vital capacity (r = −0.369). The Cox hazard model demonstrated that serum LTBP2 could predict the prognosis of IPF patients (hazard ratio for death by respiratory events: 1.040, 95% confidence interval: 1.026–1.054), which was validated using the bootstrap method with 1000-fold replication. LTBP2 is a potential prognostic blood biomarker that may reflect the level of differentiation of lung fibroblasts into myofibroblasts in IPF.

Pathological differential diagnoses of pleuroparenchymal fibroelastosis (PPFE) include usual interstitial pneumonia (UIP) and pulmonary apical cap (PAC); however, there are no specific immunostaining makers to distinguish between these diseases. We performed immunohistochemistry using several pleural mesothelial cell-related markers, including cytokeratin-5/6, CAM5.2, WT-1, calretinin, desmin and podoplanin, for PPFE (n = 4), UIP (n = 10) and PAC (n = 3) lung sections. Among the examined markers, in PPFE and PAC lungs podoplanin commonly showed positivity for spindle cells both in thickened pleura and subpleural fibroelastosis lesions; these cells were also stained with α-smooth muscle actin, a marker of myofibroblasts. However, even in elastic fibre-rich cases, UIP lungs did not show such podoplanin-positive myofibroblasts in pleura/subpleura and fibroblastic foci. These findings were also verified using immunofluorescence. By contrast, immunohistochemically as well as morphologically, the difference between PPFE and PAC was not apparent. The presence of podoplanin-positive myofibroblasts could be a pathological hallmark of PPFE, suggesting a pathogenic process distinct from UIP but common to PAC.

Background and objective Pneumothorax is a co-morbidity in patients with idiopathic pulmonary fibrosis (IPF). However, its incidence, risk factors and prognostic significance in IPF remain unclear. The aim of this study was to clarify the incidence and prognostic significance of pneumothorax in patients with IPF, and to further investigate the risk factors for its onset. Methods Eighty-four consecutive patients with IPF based on the consensus guideline were included in this study. We retrospectively reviewed the medical records, pulmonary function tests and chest high-resolution computed tomography images, and determined the incidence of pneumothorax. The prognostic significance of pneumothorax was evaluated using the Cox proportional hazards model analysis with time-dependent covariates. We also assessed the cumulative incidence and the risk factors for pneumothorax. Results Of the 84 patients, 17 (20.2%) developed pneumothorax. The cumulative incidence of pneumothorax was 8.5%, 12.5% and 17.7% at 1, 2 and 3 years, respectively. Univariate analysis demonstrated that pneumothorax was significantly related to poor prognosis (hazards ratio, 2.99; P = 0.002). Multivariate analysis, adjusting for sex, age and forced vital capacity (% predicted), revealed that pneumothorax was an independent predictor of poor outcome in IPF (hazards ratio, 2.85; P = 0.006). Lower BMI and the presence of extensive reticular abnormalities were significantly associated with developing pneumothorax. Conclusion These results confirm that patients with IPF often develop pneumothorax during their clinical course and that the onset of pneumothorax predicts a poor outcome.

Objective.Interstitial lung disease (ILD) is involved in polymyositis/dermatomyositis (PM/DM), a disease associated with poor prognoses. Chitinase-3-like-1 protein (YKL-40) has pleiotropic biological activities involved in inflammation, cell proliferation, and tissue remodeling; however, the clinical application of YKL-40 remains limited. We investigated the clinical significance of YKL-40 in PM/DM–ILD.Methods.Sixty-nine consecutive patients with PM/DM–ILD and 34 healthy controls were analyzed. We measured baseline and followup serum YKL-40 using an ELISA, evaluated the association of YKL-40 with clinical variables and survival, and examined YKL-40 expression in lung specimens from patients with PM/DM–ILD using immunohistochemistry.Results.Serum YKL-40 levels were significantly greater in patients with PM/DM–ILD compared with healthy controls (p < 0.0001). Serum YKL-40 was correlated with arterial oxygen pressure (r = –0.40, p < 0.001) and percent-predicted DLCO (r = –0.41, p = 0.01) in patients with PM/DM–ILD. Multivariate Cox hazard analysis demonstrated that higher serum YKL-40 and lower percent-predicted forced vital capacity were independently associated with a poor prognosis. Immunohistochemistry analysis demonstrated that YKL-40 expression was enhanced in aggregated intraalveolar macrophages and hyperproliferative alveolar epithelial cells in patients with PM/DM–ILD.Conclusion.YKL-40 is a promising biomarker for evaluating PM/DM–ILD activity/severity and predicting disease prognosis. Insights into YKL-40 might help elucidate the pathogenesis of PM/DM–ILD.

Background: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features, and a relatively poor prognosis. However, patinets have various clinical presentations with a heterogeneous clinical course. Cluster analysis modeling is a valuable tool in identifying distinct clinical phenotypes in heterogeneous conditions. Methods: Using cluster analysis, novel PPFE phenotypes were identified among subjects from multicenter cohort, and outcomes were stratified according to phenotypic clusters. Results: Among subjects with complete data for baseline variables (N = 84), four clusters were identified. Cluster 1 (younger male subjects with coexistent non-UIP pattern) had the highest baseline FVC and diffusion capacity of the lung for carbon monoxide (DLCO). Cluster 2 (elderly female subjects non-smokers with lowest BMI) had intermediate FVC and DLCO. Cluster 3 (elderly male smokers with coexistent UIP pattern) had the lowest baseline FVC. Cluster 4 (younger male smokers without lower lobe lesion) had relatively higher baseline FVC and DLCO. In survival outcome, patients in Cluster 3 had significantly poor prognosis than Cluster 1, 2, and 4 (p Conclusions: Among diverse progressive idiopathic PPFE patients, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might predict survival outcome.

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a devastating condition that frequently occurs in the advanced stage of IPF. However, the clinical features in AE of connective tissue disease-associated interstitial pneumonia (AE-CTD-IP) have not been well-established. The aim of this study was to clarify the clinical features of AE-CTD-IP and to compare them with those of AE-IPF. Fifteen AE-CTD-IP patients and 48 AE-IPF patients who were diagnosed and treated at our hospital were retrospectively studied. Compared with AE-IPF patients, AE-CTD-IP patients had a significantly higher %FVC (median, 94.8 vs. 56.3%; p < 0.001) and a lower extent of honeycombing on HRCT ( p = 0.020) within 1 year before AE. At AE, AE-CTD-IP patients showed higher white blood cell counts (12.0 vs. 9.9 × 103/μL; p = 0.023), higher CRP (10.2 vs. 6.7 mg/dL; p = 0.027), and longer period from admission to the beginning of AE treatment (4 vs. 1 days; p = 0.003) than AE-IPF patients. In addition, patients with AE-CTD-IP had poor prognosis as in those with AE-IPF (log-rank; p = 0.171). In conclusion, AE-CTD-IP occurred even in the early stage of IP and had more inflammatory status than in AE-IPF.

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features among idiopathic interstitial pneumonias (IIPs). Affected patients have a variety of clinical presentations with heterogeneous clinical courses. Cluster analysis is a valuable tool for identifying distinct clinical phenotypes under heterogeneous conditions. This study aimed to identify the phenotypes of patients with idiopathic PPFE. Using cluster analysis, novel PPFE phenotypes were identified among subjects from our multicenter cohort, and outcomes were stratified according to phenotypic clusters. Among the subjects with baseline data (N = 84), four clusters were identified. Cluster 1 included younger male subjects with coexisting non-UIP-like patterns. Cluster 2 included elderly female nonsmokers with low body mass index (BMI). Cluster 3 included elderly male smokers with a coexisting IP-like pattern. Cluster 4 included younger male smokers without lower lobe lesions. Patients in cluster 3 had significantly worse survival outcomes than those in clusters 1, 2, and 4 (p &lt, 0.001, p = 0.0041, and p = 0.0155, respectively). Among idiopathic PPFE patients, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might predict survival outcomes.

Objectives Although acute exacerbation of pre-existing interstitial lung disease (AE-ILD) associated with cytotoxic chemotherapy has been recognized as a severe complication in lung cancer treatment, its risk factors have not been fully studied. Materials and methods Among lung cancer patients receiving cytotoxic chemotherapy, patients with pre-existing ILD were identified based on the pretreatment high-resolution computed tomography (HRCT) findings. Chemotherapy-associated AE-ILD was defined as deterioration or development of dyspnea and HRCT findings of new bilateral ground-glass attenuations with/without non-segmental consolidation superimposed on pre-existing interstitial shadows, without evidence of pulmonary infection, congestion, or pulmonary embolism, within four weeks after the last administration of chemotherapy. Baseline characteristics were reviewed and the risk factors for chemotherapy-associated AE-ILD were evaluated by logistic regression analyses. Results Among 85 patients identified as having pre-existing ILD, chemotherapy-associated AE-ILD occurred in 26 patients (30.6%); 8 patients died and 11 patients had a severely deteriorated general condition despite intensive treatment. Compared with those without AE-ILD, patients with AE-ILD had significantly lower forced vital capacity (FVC) (median: 91.1% versus 76.6%, P =0.01). Univariate and multivariate logistic regression analyses identified baseline lower FVC and non-small cell lung cancer (NSCLC) as the risk factors for this severe event (odds ratio of FVC: 0.97, 95% confidence interval: 0.94–0.99; odds ratio of NSCLC: 4.65, 95% confidence interval: 1.10–19.76). Conclusion Chemotherapy-associated AE-ILD was a frequent and lethal complication in lung cancer treatment for patients with pre-existing ILD. Spirometric assessment of pulmonary function may be useful to predict the event.

Aim Although methicillin-resistant Staphylococcus aureus (MRSA) is commonly isolated from respiratory specimens in healthcare-associated pneumonia (HCAP), it is difficult to determine the causative pathogen because of the possibilities of contamination/colonization. The present study aimed to identify clinical predictors of the true pathogenicity of MRSA in HCAP. Methods Patients with HCAP with positive MRSA cultures in the sputum or endotracheal aspirates who were admitted to Seirei Mikatahara General Hospital, Hamamatsu, Japan, from 2009 to 2014 were enrolled. According to the administered drugs and the treatment outcomes, patients with true MRSA pneumonia (MP) and those with contamination/colonization of MRSA (false MP) were identified. Baseline characteristics were compared between groups, and clinical predictors of true MP were evaluated by logistic regression analyses. Results A total of 93 patients (mean age 78.7 ± 12.6 years) were identified and classified into the true MP (n = 16) or false MP (n = 77) groups. Although baseline characteristics were broadly similar between groups, the true MP group had significantly more patients with PaO2 ≤ 60 Torr/pulse oximetry saturation ≤90% and those with MRSA single cultivation. Both variables were significant predictors of true MP in multivariate analysis (odds ratio of PaO2 ≤ 60 Torr/pulse oximetry saturation ≤90%: 5.64, 95% confidence interval 1.17–27.32; odds ratio of MRSA single cultivation: 4.76, 95% confidence interval 1.22–18.60). Conclusions Poor oxygenation and MRSA single cultivation imply the true pathogenicity of MRSA in HCAP with positive respiratory MRSA cultures. The present results might be helpful for the proper use of anti-MRSA drugs in this population. Geriatr Gerontol Int 2017; 17: 456–462.

Langerhans cell (LC) histiocytosis (LCH) and LC sarcoma (LCS) are proliferative processes consisting of cells having morphologic and phenotypic features of Langerhans cells (LCs), although the latter may have lost some of these features. Because neoplastic nature of LCH as well as LCS is more likely by recent studies, a category of LC hyperplasia can be better characterized. LCH and LCS are rarely seen in daily pathology practice, but it is important to accurately characterize these lesions. For this purpose, an outline covering proliferations of LC and related cells was constructed. The scheme of this outline is based especially on evaluating borderline lesions, neoplastic trans-differentiation, and degree of similarity with the normal counter-parts. In addition, the organization and update of the current classification scheme for histiocytic and dendritic-cell proliferations is presented.

Case Report, We report a case of a large (diameter>20 cm) mucinous cystic neoplasm (MCN) treated with laparoscopic distal pancreatectomy (LDP) using a double-balloon catheter. A 70-year-old woman presented to our hospital with epigastric pain and abdominal distention. Imaging studies revealed a large cystic mass in the left upper abdominal quadrant. No mural nodule was observed in the cyst. On the basis of these findings, we diagnosed a large pancreatic MCN. There was no evidence of malignancy, and LDP was considered to be the most appropriate treatment for this patient. Under pneumoperitoneum of 10 mmHg CO2, 5 trocars were inserted. Laparoscopic findings revealed that the cystic lesion occupied the left upper abdominal quadrant and that the surgical fields were limited. After dissecting the anterior surface of the cystic wall, a double-balloon catheter was used for intracorporeal aspiration of cystic fluid, as the initial step of surgery. In total, 3000 ml of serous brown fluid was aspirated, and no fluid leaked into the abdominal cavity during aspiration. A satisfactory laparoscopic view was obtained after aspiration, and LDP was performed safely. Pathologic findings revealed pancreatic mucinous cystic adenoma with ovarian-type stroma. The patient's postoperative course was uneventful, and she was discharged 10 days after surgery. Use of a double-balloon catheter for large MCNs is extremely important because it is safe laparoscopically and prevents leakage of cystic fluid into the abdominal cavity.

Change in body composition with skeletal muscle wasting, a major component of pulmonary cachexia, is associated with mortality in chronic obstructive pulmonary disease and cancer. However, its relevance in interstitial lung diseases (ILDs) remains unclear. We hypothesized changes in body composition would be associated with mortality in ILDs. We measured the cross-sectional-area (ESMCSA) and muscle attenuation (ESMMA) of erector-spinae muscles, as determined by CT-imaging, in patients with idiopathic pulmonary fibrosis (IPF; n = 131) and idiopathic pleuroparenchymal fibroelastosis (iPPFE; n = 43) and controls. Subsequently, implications with prognosis were evaluated. The ESMCSA of ILD patients, but not ESMMA, was significantly smaller than that in controls. Lower ESMCSA with decreased BMI were recorded in iPPFE patients versus IPF patients, whilst IPF patients had decreased ESMCSA without BMI decline. Lower ESMCSA in IPF patients were associated with poorer prognoses. Conversely, decreased ESMMA were associated with worse survival in iPPFE patients. Multivariate analyses showed that ESMCSA in IPF and ESMMA in iPPFE were independent risk factors for mortality. Distinct changes in body composition had prognostic significance among patients with IPF and iPPFE. Lower ESMCSA and ESMMA were independently associated with poor prognosis in IPF and iPPFE, respectively. These results suggest values to measure body composition changes in managing patients with IPF and iPPFE.

Background Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has an extremely poor prognosis. The role of ferritin in the pathogenesis of AE-IPF is not well known while serum ferritin is a key prognostic indicator for patients with clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia. Objective To elucidate the clinical importance of serum ferritin in patients with AE-IPF. Methods Thirty-seven patients (48 episodes), who were diagnosed with AE-IPF and treated at our hospital between 1997 and 2015, were retrospectively studied. Results Patients with AE-IPF had significantly higher levels of serum ferritin than baseline levels at the first diagnosis of IPF (P = 0.0017). Receiver operating characteristic analysis showed the cut-off value 174 ng/mL for the separation of AE (area under the curve, 0.700). No patients with AE-IPF were positive for anti- melanoma differentiation-associated gene 5 antibody. Patients with AE-IPF and higher ferritin (≥174 ng/mL) had lower %FVC and %DLCO before AE, and those with much higher ferritin (≥500 ng/mL) had significantly worse prognosis than those with lower ferritin (log-rank, P = 0.024). Immunohistochemical staining in autopsy specimens showed alveolar macrophages that were producing ferritin. Finally, in multivariate Cox proportional hazards analyses, serum ferritin level of ≥500 ng/mL was a significant worse prognostic factor (hazard ratio 5.280, P = 0.046). Conclusion Higher serum ferritin may be related to a worse prognosis in patients with AE-IPF.

BACKGROUND Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial pneumonia and sometimes coexists with a histologic usual interstitial pneumonia (UIP) pattern. This study aimed to describe the distinct clinical features of PPFE with UIP pattern compared with idiopathic pulmonary fibrosis (IPF). METHODS We conducted a retrospective review of the medical records of 110 consecutive patients with IPF with a histologic UIP pattern on surgical lung biopsy specimen. Patients meeting radiologic criteria for the diagnosis of PPFE based on high-resolution CT scan and with a histologic UIP pattern were included. RESULTS Nine of eleven patients meeting radiologic criteria for the diagnosis of PPFE were histologically confirmed as having PPFE with UIP pattern. The PPFE with UIP pattern group showed a significantly higher residual volume (1.8 L vs 1.3 L, P 2 (44.6 mm Hg vs 41.7 mm Hg, P = .04), and higher complication rate of pneumothorax and pneumomediastinum than the 99 patients with IPF/UIP. The ratio of anteroposterior to transthoracic diameter in patients with PPFE with UIP pattern was significantly lower than that in patients with IPF/UIP ( P = .04). Survival time tended to be shorter in patients with PPFE with UIP pattern. CONCLUSIONS The results support the view that PPFE with UIP pattern is a disease entity distinct from IPF/UIP and may well be classified as PPFE.