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The coronavirus disease (COVID-19) pandemic negatively affected the diagnosis and treatment of several cancer types. However, this pandemic's exact impact and extent on bone and soft tissue sarcomas need to be clarified. We aimed to investigate the effect of the COVID-19 pandemic and emergency declaration by the local government on consultation behavior and clinical stage at diagnosis of bone and soft tissue sarcoma. A total of 403 patients diagnosed with bone and soft tissue sarcoma who initially visited three sarcoma treatment hospitals between January 2018 and December 2021 were included. The monthly number of newly diagnosed soft tissue sarcoma patients was reduced by 25%, and the proportion of soft tissue patients with stage IV disease at diagnosis significantly increased by 9% during the COVID-19 pandemic compared to before the COVID-19 pandemic. Furthermore, the monthly number of new primary bone and soft tissue sarcoma patients significantly decreased by 43% during the state of emergency declaration. The COVID-19 pandemic had a negative impact on soft tissue sarcoma patients' consultation behavior and increased the proportion of advanced-stage patients at initial diagnosis. An emergency declaration by the local government also negatively affected primary bone and soft tissue sarcoma patients' consultation behavior. [ABSTRACT FROM AUTHOR]

Purpose: The present study aimed to determine whether concomitant extrahepatic bile duct resection (EHBDR) improves the prognosis of patients with T2 gallbladder cancer (GBC). Methods: Between 2014 and 2018, 4947 patients with GBC were registered in the National Biliary Tract Cancer Registry in Japan. This included 3804 patients (76.9%) who underwent curative‐intent surgical resection; 1609 of these patients had pT2 GBC with no distant metastasis. Of the 1609 patients with GBC, 520 underwent EHBDR and 1089 did not. We compared the patients' backgrounds and disease‐specific survival rates between the groups. Results: The frequency of lymph node metastasis was significantly higher in the EHBDR group than in the non‐EHBDR group (38.2% vs. 20.7%, p <.001). In the entire cohort, however, there was no significant difference in disease‐specific survival between the two groups (76% vs. 79%, p =.410). The EHBDR group had a significantly higher incidence of postoperative complications (Clavien–Dindo classification grade = 3) (32.4% vs. 11.7%, p <.001). When we focused on the survival of only T2N1 patients who underwent gallbladder bed resection, the prognosis was significantly improved for the EHBDR group (5‐year survival rate: 64% vs. 54%, p =.017). The non‐EHBDR group was subcategorized into two groups: D2 dissection and D1 dissection or sampling, and survival curves were compared between these subgroups. Although the EHBDR group tended to have a favorable prognosis compared to the D2 group, this difference was not significant (p =.167). However, the EHBDR group had a significantly greater prognosis than the D1 dissection or sampling group (5 year‐survival rate: 64 vs. 49%, p =.027). Conclusions: The EHBDR may improve the prognosis of patients with T2 gall bladder cancer with lymph node metastases; however, its indication should be carefully determined because of the increased risk of postoperative complications. [ABSTRACT FROM AUTHOR]

Giant cell tumour of bone (GCTB) is genetically characterised by an H3F3A mutation. GCTB is treated with curettage or resection, and denosumab may be administered. Herein, we retrospectively analysed a large cohort of GCTB and identified a previously uncharacterised distinct blue matrix. Among 127 archival GCTB cases positive for the H3F3A G34 mutation, a blue lacy matrix was observed in 10 cases (7.9%). Five patients were previously treated with denosumab. Although a focal observation, the matrix was multifocal or relatively conspicuous in a subset. It appeared as blue mottles or bands, in which a delicate meshwork of basophilic, focally calcified, lacy material intricately surrounded the H3.3 G34W-positive mononuclear cells. The matrix was associated with depletion of osteoclast-like giant cells and fascicular proliferation of spindle cells, regardless of the history of denosumab therapy. Recognising this unique matrix will help avoid confusion with other bone tumours with different clinical management. [ABSTRACT FROM AUTHOR]

Purpose: Undifferentiated pleomorphic sarcoma (UPS) is associated with poor prognosis. Recently, signal regulatory protein alpha (SIRPα), which is the immune checkpoint of macrophages, and T cell immunoreceptor with immunoglobulin and immunoreceptor tyrosine-based inhibition motif domains (TIGIT), which is the immune checkpoint of T cells and natural killer cells, have been considered as potential targets for cancer immunotherapy. This study aimed to assess the value of SIRPα and TIGIT as prognostic factors of UPS. Materials and methods: The cBio Cancer Genomics Portal was used to analyze mRNA expression data of 50 UPS cases in the Cancer Genome Atlas. We retrieved 49 UPS cases and performed immunohistochemistry (IHC) to detect programmed death ligand 1 (PD-L1), SIRPα, CD68, CD163, TIGIT, CD155, and CD8. Results: SIRPα was positively associated with CD163 (Pearson's r = 0.51, p = 0.0002) as per open access data and IHC of the cohort (p = 0.002), which revealed that SIRPα-positive macrophage infiltration was higher in UPS cells with ≥ 1% PD-L1 expression than that in UPS cells with < 1% PD-L1 expression (p = 0.047). TIGIT was positively correlated with PD-L1 (r = 0.54, p < 0.0001) and CD8A (r = 0.98, p < 0.0001). In 35 of 49 cases, IHC revealed high levels of TIGIT expression on tumor cells. Furthermore, TIGIT expression on tumor cells was negatively correlated with CD155-positive (p = 0.0144) and CD8-positive (p = 0.0487) cell infiltration. Survival analysis showed that the high degree of SIRPα-positive macrophage infiltration was associated with poor overall survival and metastasis (p < 0.0001, p = 0.0006, respectively). Conclusion: SIRPα-positive macrophages infiltrated UPS cells, which predicted poor prognosis. High TIGIT expression on tumor cells was associated with decreased levels of tumor-infiltrating macrophages in UPS. [ABSTRACT FROM AUTHOR]

Myxoid liposarcoma (MLS) accounts for 20%‐30% of liposarcoma and the round cell component (RCC) is believed to be a specific poor prognostic factor. However, the RCC assessment criteria are vaguely defined and, therefore, are inconsistently employed by pathologists. In this study, we modified and applied two established grading systems to evaluate nuclear atypia (namely, the World Health Organization/International Society of Urological Pathology and the Fuhrman grading in renal cell carcinoma) in 64 MLS cases. Detailed software‐based assessments of the morphology and the cellularity were performed. DNA mutation analysis, comprehensive mRNA expression analysis, and immunohistochemistry were also performed. Our findings revealed that the high–nuclear‐grade group according to the modified Fuhrman grading system exhibited a significantly poor disease‐free survival (hazard ratio: 4.43; 95% confidence interval: 0.9‐22.6; p = 0.047). On the other hand, the cellularity was significantly higher in the modified Fuhrman high‐grade group (p = 0.010 at the percentage of the hypercellular area; p = 0.003 at the maximum cell density) but did not qualify per se as a poor prognostic factor in the survival analyses. Furthermore, the modified Fuhrman high‐grade group significantly expressed the cell cycle–related genes (such as FOXM1, PLK1, and CDK1). In conclusion, our analyses suggest that an evaluation focusing on nuclear morphology (rather than on cellular density) can be more reliable in predicting the MLS prognosis. [ABSTRACT FROM AUTHOR]

Intimal sarcoma is one of the most common and well-known primary malignant neoplasms of the aorta and heart. The authors reviewed cases of intimal sarcoma from histological, immunohistochemical and genetic perspectives. Twenty cases of intimal sarcoma were retrieved. Immunohistochemistry and FISH of MDM2 and PDGFRA genes were performed. All 20 tumours were composed of spindle-shaped, stellate, oval or polygonal tumour cells with irregular hyperchromatic nuclei arranged in a haphazard pattern, accompanied by nuclear pleomorphism and frequent mitotic figures. Other histological findings were as follows: abnormal mitosis in 10 cases (50%), necrosis in 15 cases (75%), myxoid stroma in 12 cases (60%), cartilaginous formation in 1 case (5%), haemorrhage in 12 cases (60%) and fibrinous deposition in 14 cases (70%). The tumours were positive for MDM2 in 16 cases (80%), ERG in 4 cases (20%), alpha-smooth muscle actin in 6 cases (30%), desmin in 5 cases (25%) and AE1/AE3 in 4 cases (20%). Immunohistochemical positivity was focal in each case. Loss of H3K27me3 expression was noted in 2 cases (10%). MDM2 and PDGFRA gene amplifications were detected in 11 cases (55%) and 1 case (5%), respectively. Fisher's exact test revealed a significant correlation between MDM2 gene amplification and myxoid stroma (p = 0.0194). No parameters showed any association with the anatomical location of the tumours. It was suggested that myxoid histology of intimal sarcoma may be associated with MDM2 gene amplification and that intimal sarcoma may be divided into myxoid and non-myxoid types. [ABSTRACT FROM AUTHOR]

Malignant peripheral nerve sheath tumor (MPNST) is a very aggressive peripheral nerve sheath-derived sarcoma, which is one of the most difficult tumors to diagnose due to its wide spectrum of histological findings and lack of specific immunohistochemical markers. Recently, it has been reported that losses of expression of H3K27me3 and H3K27me2 caused by PRC2 dysfunction may be useful diagnostic markers for MPNST, but there is no consensus on their clinicopathological significance. Here, we investigated the relationship between loss of H3K27 methylation and various parameters and clarified the clinicopathological significance of such loss. We analyzed the clinicopathological and immunohistochemical features in 84 MPNST cases. Complete losses of H3K27me3 and H3K27me2 were observed in 37 (44%) and 29 (35%) cases, respectively. Losses of H3K27me3 and H3K27me2 were significantly correlated with myogenic immunopositivity (H3K27me3 vs. desmin, P = 0.0051; H3K27me3 vs. myogenin, P = 0.0009; H3K27me2 vs. myogenin, P = 0.042). Meanwhile, there were significant correlations between preservation of immunohistochemical neurogenic markers and intact H3K27me3 and H3K27me2 (H3K27me3 vs. S-100 protein, P = 0.0019; H3K27me3 vs. SOX10, P = 0.014; H3K27me2 vs. S-100 protein, P = 0.0011; H3K27me2 vs. SOX10, P = 0.0087). In multivariate analysis, local recurrence, distant metastasis, high FNCLCC grade, and loss of SOX10 expression were independent prognostic factors for overall survival. H3K27me3 and H3K27me2 expression was retained in all 26 cases of rhabdomyosarcoma non-alveolar subtype. In conclusion, we suggest that H3K27me3 and H3K27me2 immunonegativity is useful but not definitive for diagnosing MPNST. Complete loss of H3K27 methylation may be involved in aggressive transdifferentiation from neural differentiation to skeletal muscle differentiation in MPNST. [ABSTRACT FROM AUTHOR]

We report the peculiar case of a parosteal osteosarcoma arising beneath the periosteum in a 12-year-old boy. He complained of difficulty in left knee flexion. Plain radiography showed a uniformly dense mineralized mass in the bone cortex and parosteal ossified nodules at the metaphysis and diaphysis of the left distal femur. Periosteal reaction was not evident. Uniquely, plain radiography had a smooth outline and revealed gradually thickening mass toward the center. Histologically, the tumor showed a proliferation of spindle-shaped cells with parallel-oriented dense bone trabeculae and hyaline cartilaginous tissue disclosing mild atypia. The periosteum was inverted along the polypoid mass, but there was no periosteum at the top. Immunohistochemically, the spindle cells, including those at the top of the polypoid mass, and cartilaginous cells were positive for MDM2 and CDK4. MDM2 gene amplification was detected in these cells by fluorescence in situ hybridization. Despite the peculiar feature of plain radiography, the lesion was diagnosed as parosteal osteosarcoma. This case report presents a case of parosteal osteosarcoma arising beneath the periosteum, although it is postulated to arise in the outer layer of the periosteum. The unique radiographic findings in this case suggest an association of parosteal osteosarcoma with vigorous bone growth before closure of the growth plate. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is an intermediate malignant bone tumor that is locally aggressive and rarely metastasizes. Denosumab, which is a receptor activator of nuclear factor kappa B ligand (RANKL) inhibitor, can be used to treat GCTB. We focused on potential immunotherapy for GCTB and investigated the tumor microenvironment of GCTB. Programmed death-ligand 1 (PD-L1) and indoleamine 2,3-dioxygenase 1 (IDO1) expression and signal-regulatory protein alpha (SIRPα), forkhead box P3 (FOXP3), and cluster of differentiation 8 (CD8) infiltration were assessed by immunohistochemical studies of 137 tumor tissues from 96 patients. Of the naive primary specimens, 28% exhibited PD-L1 expression and 39% exhibited IDO1 expression. There was significantly more SIRPα+, FOXP3+, and CD8+ cell infiltration in PD-L1- and IDO1-positive tumors than in PD-L1- and IDO1-negative tumors. The frequency of PD-L1 expression and SIRPα+ cell infiltration in recurrent lesions treated with denosumab was significantly higher than in primary lesions and recurrent lesions not treated with denosumab. PD-L1 expression and higher SIRPα+ cell infiltration were significantly correlated with shorter recurrence-free survival. PD-L1 and SIRPα immune checkpoint inhibitors may provide clinical benefit in GCTB patients with recurrent lesions after denosumab therapy. [ABSTRACT FROM AUTHOR]

Background: Undifferentiated pleomorphic sarcoma (UPS) is a sarcoma with a poor prognosis. A clinical trial, SARC028, revealed that treatment with anti-PD-1 drugs was effective against UPS. Studies have reported that UPS expresses PD-L1, sometimes strongly (≥ 50%). However, the mechanism of PD-L1 expression in UPS has remained unclear. CKLF-like MARVEL transmembrane domain containing 6 (CMTM6) was identified as a novel regulator of PD-L1 expression. The positive relationship between PD-L1 and CMTM6 has been reported in several studies. The aim of this study was thus to examine CMTM6 expression in UPS and evaluate the relationship between PD-L1 and CMTM6 in this disease. Materials and methods: Fifty-one primary UPS samples were subjected to CMTM6 and PD-L1 immunostaining. CMTM6 expression was assessed using proportion and intensity scores. CMTM6 gene copy number was also evaluated using a real-time PCR-based copy number assay. We also analyzed the mRNA expression and copy number variation of PD-L1 and CMTM6 in The Cancer Genome Atlas (TCGA) data. Results: TCGA data indicated that the mRNAs encoded by genes located around 3p22 were coexpressed with CMTM6 mRNA in UPS. Both proportion and intensity scores of CMTM6 positively correlated with strong PD-L1 expression (≥ 50%) (both p = 0.023). CMTM6 copy number gain increased CMTM6 expression. Patients with UPS with a high CMTM6 intensity score had a worse prognosis for overall survival. Conclusions: UPS showed variation in CMTM6 copy number and CMTM6 expression. CMTM6 expression was significantly correlated with PD-L1 expression, especially with strong PD-L1 expression. [ABSTRACT FROM AUTHOR]

The yips, an involuntary movement impediment that affects performance in skilled athletes, is commonly described as a form of task-specific focal dystonia or as a disorder lying on a continuum with focal dystonia at one end (neurological) and chocking under pressure at the other (psychological). However, its etiology has been remained to be elucidated. In order to understand sensorimotor cortical activity associated with this movement disorder, we examined electroencephalographic oscillations over the bilateral sensorimotor areas during a precision force task in athletes with yips, and compared them with age-, sex-, and years of experience-matched controls. Alpha-band event-related desynchronization (ERD), that occurs during movement execution, was greater in athlete with yips as compared to controls when increasing force output to match a target but not when adjusting the force at around the target. Event-related synchronization that occurs after movement termination was also greater in athletes with yips. There was no significant difference in task performance between groups. The enhanced ERD is suggested to be attributed to dysfunction of inhibitory system or increased allocation of attention to the body part used during the task. Our findings indicate that sensorimotor cortical oscillatory response is increased during movement initiation in athletes with yips. [ABSTRACT FROM AUTHOR]

Purpose: Therapies targeting the immune checkpoint molecules programmed death ligand 1 (PD-L1) and indoleamine 2,3-dioxygenase 1 (IDO1) have been explored in various malignant tumours. In this study, we examined the relationship between PDL-1, IDO1 and JAK2 expression and the roles of these signal pathways in soft tissue leiomyosarcoma (LMS). Methods: The next-generation sequencing data of 53 patients with LMS were obtained from an online public database and were used to assess PD-L1, IDO1 and JAK2 gene amplification and mRNA expression. Then, we determined the relationship between JAK–STAT pathway activation and PD-L1 and IDO1 expression in a LMS cell line. In addition, immunohistochemical staining of 69 cases of LMS was performed for PD-L1, IDO1, TDO2 and phosphorylated JAK2 (pJAK2). Results: Comprehensive gene expression analysis using microarray and RNA-Seq data revealed that PD-L1 and IDO1 mRNA expression positively correlated with JAK2 and STAT1 mRNA expression. Two of the 53 cases exhibited PD-L1 and JAK2 gene amplification; however, they were not related to their gene expression. LMS cell line analysis revealed that IFN-γ supplementation induced IDO1 and PD-L1 expression; these effects were suppressed by JAK inhibition. Immunohistochemical analysis of the resected specimens revealed that TDO2 expression positively correlated with pJAK2 (P = 0.0490) and IDO1 expression (P < 0.0001). PD-L1-positive specimens tended to express pJAK2; however, the relationship did not reach statistical significance (P = 0.1477). Conclusion: The results suggest the possible feasibility of the combined inhibition of PD-1/PD-L1 or IDO1 with IFN-γ–JAK–STAT pathway inhibition to treat soft tissue LMS. [ABSTRACT FROM AUTHOR]

Purpose: Programmed death ligand 1 (PD-L1) and indoleamine 2,3-dioxygenase 1 (IDO1) are immunosuppressive proteins known to be associated with poor prognosis in various cancers. However, their expression and clinical relevance in osteosarcoma remain unknown. In this study, the relationships of PD-L1 and IDO1 expression with clinicopathological features and prognosis were explored. Methods: The expression of PD-L1, IDO1, CD3, CD4, and CD8 in 112 formalin-fixed, paraffin-embedded tumor tissues collected by biopsy or surgical resection from 56 osteosarcoma patients was evaluated immunohistochemically. Moreover, four osteosarcoma cell lines were evaluated for the effects of IFNγ on PD-L1 and IDO1 mRNA expression by real-time reverse-transcription polymerase chain reaction. Results: In pre-neoadjuvant chemotherapy (NAC) primary specimens, 10 cases (17%) showed PD-L1 expression and 12 (21%) showed IDO1 expression. Six of ten cases (60%) with PD-L1 positivity co-expressed IDO1. In post-NAC metastatic lesions, the frequency of immunoexpression of PD-L1 and IDO1 was increased compared with that in pre-NAC specimens. PD-L1 and/or IDO1 expression was not associated with poor prognosis. PD-L1 immunoexpression was significantly associated with the infiltration of CD3+ T cells, CD4+ T cells, and CD8+ T cells; while, IDO1 immunoexpression was significantly associated with the infiltration of CD3+ T cells and CD4+ T cells. In all osteosarcoma cell lines, PD-L1 and IDO1 expression was upregulated by stimulation with IFNγ. Conclusion: Our results suggest that the PD-L1 and IDO1 immune checkpoint inhibitors may provide clinical benefit in osteosarcoma patients with metastatic lesions after conventional chemotherapy. [ABSTRACT FROM AUTHOR]

Data on FOLFIRINOX as a second-line chemotherapy for advanced pancreatic cancer are limited. In the JASPAC06 study—a nationwide, multicenter, observational study—FOLFIRINOX for patients with unresectable or recurrent pancreatic cancer as any line of treatment showed favorable efficacy and safety in Japanese clinical practice. We performed exploratory analyses of patients with unresectable or recurrent pancreatic cancer who received FOLFIRINOX as the second-line chemotherapy in Japanese clinical settings. Of the 399 evaluable patients, 44 were eligible for inclusion in the analysis. The patients' characteristics were as follows: median age, 62 years; men, 26 (59%); Eastern Cooperative Oncology Group-Performance status 0/1, 30 (68%)/14 (32%); disease status, recurrent/local/metastatic: 4 (9%)/8 (18%)/32 (73%). The initial dose was reduced in 28 (64%) patients. The median time to treatment failure and number of cycles were 4.5 (range, 0.2–19.1) months and 6 cycles (range, 1–13 or more), respectively. The major grade 3/4 adverse events were neutropenia in 29 (66%), leucopenia in 17 (39%), anorexia in 7 (16%), febrile neutropenia in 5 (11%), and anemia in 5 (11%) patients. The median overall survival, progression-free survival, and 1-year survival rates were 10.3 (95% confidence interval [CI], 7.2–13.3), 4.1 (95% CI, 2.6–5.5) months, and 30%, respectively. Our findings suggest that FOLFIRINOX as a second-line chemotherapy for advanced pancreatic cancer was effective in patients with a good performance status. It displayed toxicity similar to that observed with its use as a first-line treatment. [ABSTRACT FROM AUTHOR]

Background: Lymph node metastasis is an important prognostic factor for distal bile duct cancer. The number of lymph node metastases was adopted for nodal classification. However, different cutoff values have been proposed, ranging from two to five. Methods: A total of 1748 cases who underwent curative surgery with pancreatoduodenectomy for distal bile duct cancer registered in the nationwide biliary tract cancer registry in Japan from 2008 to 2013 were included. Univariate Cox regression was performed to assess the effect of prognostic lymph node metastasis counts on mortality and to determine cutoff values. Results: The overall survival rate after resection was 47.4% at 5 years. Univariate and multivariate analysis found prognostic factors to include lymph node metastasis. The cutoff point was set to two lymph node metastases using the Cox model. There were significant differences in pairwise comparisons between three groups by the number of metastatic lymph node (P <.001 for 0 vs 1‐2 and P =.003 for 1–2 vs ≥3). Conclusion: Our data suggest lymph node classification as N0 (patients without lymph node metastases), N1 (metastasis in 1–2 regional lymph nodes), and N2 (metastases in ≥3 regional lymph nodes). [ABSTRACT FROM AUTHOR]

Background: We present a case of pancreatic and splenic metastases following alveolar soft part sarcoma (ASPS), which was successfully treated by surgery. Case presentation: A 41-year-old male was referred to our hospital in 2012. Computed tomography (CT) showed the presence of a pancreatic tumor. In 2002, the patient had undergone surgical resection of an ASPS of the anal region. In 2009, during follow-up, CT revealed lung metastases, which prompted surgical resection of the lung, followed by resection of the head skin in 2011. Abdominal ultrasonography (US) revealed the presence of isodense masses sized 34 mm in the pancreatic head and 60 mm within the spleen. The contrast-enhanced US revealed a solitary lesion with enhancement. Contrast-enhanced CT revealed solitary lesions with enhancement within the pancreatic head, spleen, and liver. The patient underwent metastasectomies from the pancreas, spleen, and liver. The patient was discharged on postoperative day 22 without recurrence for 18 months after metastasectomy. Twelve years after primary resection and 2 years after metastasectomy, the patient died as a consequence of multiple metastases. Conclusions: We have presented a rare case of pancreatic and spleen metastases from ASPS. Resection by radical metastasectomy was successful without morbidity. Thus, for improved survival of patients with multiple metastases from ASPS, metastasectomy may be indicated. If multiple metastases are resectable, surgical approaches may be the preferred treatment. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is a locally aggressive bone tumor that frequently shows local recurrence and occasionally shows malignant transformation to high-grade sarcoma. Histologically, conventional GCTB is composed mainly of three types of cells: mononuclear neoplastic cells with an osteoblastic precursor phenotype, mononuclear histiocytic cells, and osteoclast-like multinucleated giant cells. These cells interact with each other via the RANKL-RANK axis and other mechanisms for tumor formation. The vast majority of GCTBs were recently revealed to harbor H3F3A p.G34W mutation, and a minor subset have H3F3A p.G34L, p.G34M, p.G34R, or p.G34V mutation. H3.3 G34W mutant-specific immunohistochemistry is a highly sensitive and specific surrogate marker for H3F3A p.G34W mutation in GCTB and thus useful for differential diagnoses of histological mimics. H3.3 mutant-specific immunohistochemistry has also contributed to the understanding of the bone-forming ability of neoplastic cells of GCTB and the remarkable new bone formation after treatment with denosumab, an inhibitor of RANKL. In primary and secondary malignant GCTBs, the H3F3A gene allele can be preserved or lost with malignant transformation. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumor (SFT) is a soft-tissue neoplasm of intermediate malignant potential, presenting a wide histopathological spectrum. Poorer prognosis of hemangiopericytoma of the central nervous system (CNS), hypoglycemic SFT, and dedifferentiation are well-known characters of SFT, but their clinical significance were not demonstrated enough by large-sized study. Here, the clinicopathological features of SFTs are reviewed and the relationship between genetics and clinicopathological features is examined using 145 SFT cases. All cases were STAT6 IHC-positive and/or NAB2-STAT6 fusion gene-positive. Tumor location was classified into three categories: 30 pleuropulmonary, 96 non-pleuropulmonary/non-central nervous system (CNS), and 18 CNS tumors. The tumor developed recurrence in 21 of 93 available cases (22.5%), metastasis in 11 of 93 (11.8%), and tumor death in 9 of 93 (9.6%). Hypoglycemia occurred in 2 primary tumors and 1 metastatic tumor among 63 reviewable cases, and dedifferentiation occurred in 10 cases (6.8%) including 6 primary tumors, 2 recurrent tumors, and 2 metastatic tumors. Recurrence was positively associated with CNS location (p = 0.0109) and hypoglycemia (p = 0.001); metastasis was positively associated with CNS location (p = 0.0231), hypoglycemia (p < 0.0001), and dedifferentiation (p < 0.0001), while metastasis was negatively correlated with pleural location (p = 0.0471). Tumor death was positively associated with male sex (p = 0.0154), larger size (p = 0.0455), hypoglycemia (p < 0.0001), and dedifferentiation (p < 0.0001). Multivariate analysis revealed independent statistical significance of dedifferentiation for overall survival (p = 0.0467). Exon variant of the fusion gene had no statistical correlation with clinical outcome. In conclusion, dedifferentiation is a major prognostic factor of SFT, and specific location such as cerebromeningeal and intra-abdominal site and hypoglycemia also had a high risk for unfavorable prognosis. [ABSTRACT FROM AUTHOR]

Undifferentiated sarcoma (US) is a frequent soft tissue sarcoma. Although the 10-year survival rate is around 60%, advanced US is highly resistant to chemo/radiotherapy. The tumor microenvironment (TME) is closely associated with tumor progression. However, few studies of infiltrated immune cells in US have been published. In this study, we evaluated tumor-associated macrophages (TAMs) and CD8-positive cytotoxic T lymphocytes (CTLs) in 28 cases of US. Iba1, CD163, and CD204 were used as markers for TAMs. The density of CTLs was positively correlated with the density of TAMs. However, a negative correlation was seen between the density of CTLs and the percentage of CD204-positive TAMs. We found no significant association between the density of Iba1-/CD204-/CD8-positive cells and clinicopathological factors. No significant correlation between immune cell infiltration and clinical outcome was observed. Although we found no significant association between immune cells and clinicopathological factors, these findings may provide new insight into the characterization of immune cells in the TME of US. [ABSTRACT FROM AUTHOR]

Studies the low-temperature crystallization of hydrogenated amorphous silicon films in contact with evaporated aluminum electrodes. Potential applications of hydrogenated amorphous silicon; Details on the experimental procedure; Discussion on the experimental results.

Presents a study that investigated the effects of the radio frequency glow discharge parameters on the deposition rate of hydrogenated amorphous silicon for solar cells from pure silicon hydride (SiH[sub4]) plasma. Methodology; Analysis of the deposition rate and SiH[sub4] usage efficiency dependence on SiH[sub4]; Impact of pressure and SiH[sub4] flow rate on deposition rate.

In the original article published, the data of AJCC stage. [ABSTRACT FROM AUTHOR]

Aims: Phosphaturic mesenchymal tumour, mixed connective tissue variant (PMT‐MCT), is a tumour of uncertain differentiation, characterised by ‘smudgy/grungy’ calcification and vitamin D‐resistant phosphaturic osteomalacia. Fibroblast growth factor (FGF)23 is recognised as a reliable marker of PMT‐MCT, but quantitative evaluation has never been performed. We reviewed cases of tumour‐associated osteomalacia or histologically definitive PMT‐MCT without osteomalacia using histological, immunohistochemical and genetic methods and evaluated the diagnostic significance of these findings. Methods and results: A total of 19 tumours from 14 cases diagnosed previously as PMT‐MCT were retrieved, on which immunohistochemical staining, reverse transcription–polymerase chain reaction (RT–PCR) and fluorescence in‐situ hybridisation (FISH) analysis were performed. Histologically, fibrous capsule, calcification and giant cell reaction tended to be observed in soft‐tissue PMT‐MCT, while PMT‐MCT of bone and multiple PMT‐MCT showed an infiltrative growth pattern. The immunohistochemical results were as follows: the tumour cells were positive for FGF23 (nine of 12, 75%), FGFR1 (11 of 11, 100%), CD56 (12 of 14, 85.7%) and E26 oncogene homologue (ERG) (5 of 13, 38.4%). The sole malignant tumour was positive for p53. FGF23 mRNA was detected in seven of 14 formalin‐fixed paraffin‐embedded (FFPE) specimens and all five frozen specimens by RT–PCR. The level of FGF23 mRNA, which was determined by real‐time PCR, varied among the phosphaturic cases. Two of 17 tumours were positive for FGFR1 gene rearrangement. Conclusions: It was considered that PMT‐MCT is a histopathological entity with or without phosphaturia, with varying levels of FGF23 mRNA, and with or without fibronectin 1 (FN1)–FGFR1 fusion gene. The authors propose that the histology of PMT‐MCT differs depending on its location, such as bone or soft tissue, which could complicate the differential diagnosis. [ABSTRACT FROM AUTHOR]

Presents a study which investigated evaporated aluminum and copper films as metal diffusion sources for series connection between hydrogenated amorphous silicon p-i-n photodiodes. Experimental procedure; Results; Discussion; Conclusion.

Background: The present study analyzed biliary tract cancer patients registered from 2008 to 2013 in Japan and evaluated the outcomes of biliary tract cancer. Methods: A total of 18,606 patients were registered from 2008 to 2013. Cases were analyzed with regard to patient survival according to contiguous extent of the primary tumor (T), node metastasis, and tumor stage using the 3rd English edition of the Japanese classification of the biliary tract cancers. Results: Five ‐ year survival rates were 39.8% for gallbladder cancer, 24.2% for perihilar bile duct cancer, 39.1% for distal bile duct cancer, and 61.3% for ampullary region cancer. Significant differences were observed between newly introduced subdivisions in the new Japanese classification for all tumoral sites except gallbladder cancer. The survival rate in patients with #13a metastasis was significantly higher than in patients with distant lymph node metastasis. Conclusions: The new Japanese classification adopted the 7th edition of staging system developed by the Union for International Cancer Control staging system. However, numerous aspects of these classification systems remain unvalidated. The present analysis demonstrated the significance of a proportion of T factor subdivisions and classifications of regional lymph nodes in cases of gallbladder cancer in the new Japanese classification. Highlight Ishihara and colleagues conducted a registry study to determine the outcomes of biliary tract cancer and to validate the new Japanese classification based on the UICC staging system 7th edition. Of particular note, survival rates were significantly higher in patients with #13a metastasis than in those with distant lymph node metastasis. [ABSTRACT FROM AUTHOR]

Purpose: Hepatectomy of segments 4a and 5 (S4a+5) is the recommended treatment for pT2 gallbladder cancer. However, gallbladder bed resection is also occasionally used. Using nationwide data from the Japanese Biliary Tract Cancer Registry and a questionnaire survey, we retrospectively compared these 2 methods of treatment. Method: The study involved 85 patients with pT2, pN0 gallbladder cancer (55 treated with gallbladder bed resection, and 30, with S4a+5 hepatectomy). The prognosis and mode of tumor recurrence following treatment were analyzed retrospectively, with overall survival as the endpoint. Results: The 5-year survival rate did not differ significantly between the 2 groups. Univariate analysis showed that bile duct resection and perineural tumor invasion were significant prognostic factors, but the extent of hepatectomy, location of the major intramural tumor, regional lymph node excision, and histological type were not. Multivariate analysis identified perineural tumor invasion as a significant prognostic factor. Recurrence occurred most frequently in both lobes than S4a+5 of the liver following gallbladder bed resection. Conclusion: In the present study of cases of Japanese Biliary Tract Cancer Registry, it was not possible to conclude that S4a+5 hepatectomy was superior to gallbladder bed resection. [ABSTRACT FROM AUTHOR]

Background: In the field of gastroenterological surgery, laparoscopic surgery has advanced remarkably, and now accounts for most gastrointestinal operations. This paper outlines the current status of and future perspectives on robot-assisted laparoscopic pancreatectomy. Methods: A review of the literature and authors' experience was undertaken. Results: The da Vinci Surgical System is a robot for assisting laparoscopy and is safer than conventional endoscopes, thanks to the 3-dimensional hi-vision images it yields, high articular function with the ability to perform 7 types of gripping, scaling function enabling 2:1, 3:1, and 5:1 adjustment of surgeon hand motion and forceps motions, a filtering function removing shaking of the surgeon's hand, and visual magnification. By virtue of these functions, this system is expected to be particularly useful for patients requiring delicate operative manipulation. Conclusions: Issues of importance remaining in robot-assisted laparoscopic pancreatectomy include its time of operation, which is longer than that of open surgery, and the extra time needed for application of the da Vinci compared with ordinary laparoscopic surgery. These issues may be resolved through accumulation of experience and modifications of the procedure. Robot-assisted laparoscopic pancreatectomy appears likely to become a standard procedure in the near future. [ABSTRACT FROM AUTHOR]

To apply duodenum-preserving pancreatic head resection (DPPHR) as radical procedure for benign or low-grade malignant tumors, it needs the reconciliation of complete pancreatic head resection and preservation of the bile duct and peripancreatic vessels. Several modifications have been introduced and applied to remove these lesions, however, the techniques have not been made clear in the management of the peripancreatic vessels and the bile duct. The long-term outcomes of the DPPHR have been reported as extremely rare in comparison with pylorus preserving pancreatoduodenectomy (PPPD) in these pancreatic head tumors. The angiograms by multi-detector row CT (MD-CT) can be reconstructed more physiologically than selective angiography. The anterior arcade is predominant in 43% of 64 patients. Therefore, we modified the DPPHR to include a complete resection of the pancreatic head and the preservation of both anterior and posterior arterial arcades. The bile duct is covered by the pancreatic parenchyma in various ways. The techniques of the preservation of the bile duct are also introduced. We performed 21 DPPHRs and 19 PPPDs in the patients with benign or low-grade malignant pancreatic head tumor. There was no significant difference in operative factors. The postoperative death was one patient in PPPD, but none in DPPHR. The postoperative complications of PPPD were more often than that of DPPHR. There is no postoperative recurrence in DPPHR in the follow-up period from 2 to 216 months. Both exocrine and endocrine function and the long-term results following DPPHR were superior to those following PPPD. The DPPHR should be favored over the PPPD in benign or low-grade malignant tumors of the head of the pancreas if there is no compromise with oncologic radicality. [ABSTRACT FROM AUTHOR]

It is widely accepted that congenital choledochal cyst is associated with pancreaticobiliary maljunction (PBM). But, PBM is an independent disease entity from choledochal cyst. PBM is synonymous with “abnormal junction of the pancreaticobiliary ductal system”, “anomalous arrangement of pancreaticobiliary ducts”, “anomalous union of bilio-pancreatic ducts”, etc. Cases with PBM not associated with biliary duct dilatation are often found, and these cases are frequently complicated gallbladder cancer. The Japanese Study Group of Pancreaticobiliary Maljunction was started in 1983, and defined diagnostic criteria and nationwide registration system of PBM cases was started. PBM is defined as a union of the pancreatic and biliary ducts which is located outside the duodenal wall. Bile and pancreatic juice reflux and regurgitate mutually. The most bothersome problem is biliary carcinogenesis. Gallbladder cancers arise in 14.8% and bile duct cancers arise in 4.9%. The incidence of the gallbladder carcinoma of PBM without bile duct dilatation is 36.1%. Many investigators have tried to clarify the carcinogenic process, from various aspects. The biliary epithelia are injured by harmful substances, and in the course of repair, multiple alterations of oncogenes and tumor suppressor genes are followed, and they lead to carcinoma through multistage interaction. In the biliary epithelia of PBM, incidence and degree of hyperplasia are characteristic. K-ras gene mutations are observed in the cancerous as well as noncancerous lesions of biliary tract of PBM patients. Mutations of p53 gene and overexpression of p53 protein are also found in the cancerous and noncancerous lesions. These changes are called “hyperplasia–carcinoma sequence”. Total excision of the extrahepatic bile duct with gallbladder followed by hepaticojejunostomy, Roux-en-Y, or end-to-side hepaticoduodenostomy are treatment of choice, even for cases with not dilated bile duct, because the incidence of cancer in the nondilated bile duct is not negligible, and genetic changes are seen in a nondilated bile duct. [ABSTRACT FROM AUTHOR]

Complete surgical resection of biliary tract carcinoma remains the best treatment. The Japanese Society of Biliary Surgery has organized a registry project and established a classification of biliary tract carcinoma. We report here the status of biliary surgery in Japan. For hilar bile duct carcinoma, major hepatectomy is needed to increase the resection rate, and total caudate lobectomy is required for curative resection. The 5-year survival rate was 39.1%. Middle and distal bile duct carcinomas were treated with pancreatoduodenectomy (PD) or pylorus-preserving PD (PPPD) or bile duct resection alone. The 5-year survival rate was 44.0%. The treatment of gallbladder carcinoma with pT1 lesions is cholecystectomy. The treatment of pT2 lesions is extended cholecystectomy or various hepatectomy with or without extrahepatic bile duct resection along with lymphadenectomy. Treatment of pT3 and pT4 lesions includes hepatectomy with or without bile duct resection, combined with vascular resection, extended lymphadenectomy, and autonomic nerve dissection. Several groups in Japan have performed hepatopancreatoduodenectomy. The 5-year survival rate of pT1, pT2, pT3, and pT4 were 93.7, 65.1, 27.3, and 13.8%. PD or PPPD is the standard operation for carcinoma of the papilla of Vater. The 5-year survival rate was 57.5%. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Adenocarcinoma (ADC) is the most frequent histological type of lung cancer and comprises the majority of lung cancers in non-smokers. Thus, genetic factors responsible for ADC susceptibility need to be determined to establish efficient ways of preventing the disease. The OGG1 gene, encoding a glycosylase for 8-hydroxyguanine, an oxidatively damaged promutagenic base, has the polymorphism Ser326Cys, and OGG1-326Cys protein was indicated to have a lower ability to prevent mutagenesis than the OGG1-326Ser protein. Case-control studies to date suggest that the OGG1-326Cys allele is associated with a higher risk for several types of cancers, including overall lung cancer. However, the contribution of this polymorphism to lung ADC risk is unclear. In the present study, the OGG1-Ser326Cys polymorphism was assessed for association with lung ADC risk using a case-control study of a Japanese population consisting of 1097 cases and 394 controls. Odds ratios (OR) of the 326Cys allele carriers increased in a dose-dependent manner with allele number ( P for the trend test = 0.04). The OR of homozygotes for the 326Cys allele was increased significantly when homozygotes for the 326Ser allele were used as a reference (OR = 1.5, 95% confidence interval [CI] = 1.0–2.1, P = 0.04). Furthermore, the overall OR for lung ADC of the Cys/Cys homozygotes out of a total of 1925 ADC patients and 3449 controls from six case-control studies reported up to the present were 1.43 (95% CI = 1.11–1.84, P = 0.0045). These results indicate that OGG1-326Cys functions as a risk allele for lung ADC development. ( Cancer Sci 2006; 97: 724–728) [ABSTRACT FROM AUTHOR]

Using helical computed tomography (CT), we evaluated cystic pancreatic lesions in 11 patients and compared the imaging and the histopathologic findings. Helical CT allowed us to assess the tumor vasculature. Contrastenhanced images showed satisfactory details of the cysts, cyst walls, and intracystic structures. Helical CT is extremely useful for the evaluation of cystic pancreatic lesions. [ABSTRACT FROM AUTHOR]

A 76-year-old woman underwent combined resection of the gallbladder plus partial hepatectomy for early gallbladder cancer. From the pathology results, the surgical treatment was deemed to have been curative. However, 3 years later, the patient was readmitted to the hospital with an elevated carbohydrate antigen (CA) 19-9 level. Percutaneous transhepatic cholangiography demonstrated irregularity of the common hepatic duct and the left intrahepatic bile duct, and percutaneous transhepatic cholangioscopy revealed two separate papillary tumors at these sites. A diagnosis of multiple carcinomas of the bile duct was made and left hepatic lobectomy and resection of the extrahepatic bile duct was performed; reconstruction was carried out with a right hepatico-jejunostomy with Roux-en-Y anastomosis. Microscopic study revealed that both of the lesions were papillary adenocarcinomas, and normal biliary mucosa was confirmed to exist between them. [ABSTRACT FROM AUTHOR]

Discusses the visual accommodation and subject performance during a stereographic object task using liquid crystal shutters. Human response to virtual reality; Accommodation to a stereogram; Stereoscopic vision; Task performance; Fatigue symptoms.

We report a 66-year-old man who had a cystic intraductal papillary adenocarcinoma containing a papillary adenoma, in the head of the pancreas and a coexistent invasive, well differentiated solid tubular adenocarcinoma in the tail of the pancreas. He was hospitalized with acute epigastralgia. Computed tomography demonstrated a multilocular cystic mass in the head of the pancreas and a solid tumor in the tail. Endoscopic retrograde pancreatography showed mucin secretion from an enlarged papilla of Vater, marked dilatation of the main pancreatic duct in the head and body, cystic dilatation of the uncinate branch, and irregular narrowing of the main pancreatic duct in the tail. Total pancreatectomy was performed. Between the cystic tumor and the solid tumor there was a distance of 4.8 cm of normal pancreatic parenchyma and duct, recognized both grossly and microscopically. The patient died 35 months after the operation. At autopsy, peritonitis carcinomatosa was found in the abdominal cavity. Microscopically, disseminated nodules were also well differentiated tubular adenocarcinoma. The apparent anatomic separation of these two tumors within the pancreas is extremely unusual. [ABSTRACT FROM AUTHOR]

Presents a study that investigated the deposition rate dependence of hydrogenated amorphous silicon prepared by capacitively coupled radio frequency glow discharge upon substrate temperature. Background on the required substrate temperature for high-quality hydrogenated amorphous silicon; Analysis of plasma emission intensity and rate of deposition; Implications of the results.

Although methemoglobinemia is an uncommon disorder, it should always be considered in the differential diagnosis of cyanosis. Major causes of acquired methemoglobinemia are nitrates, aniline, and analgesics, though rare cases have been reported to have been caused by automobile exhaust fumes. A 24-year-old man had inhaled a large amount of automobile exhaust fumes, intending to commit suicide. He had become unconscious, with dilated pupils and symptoms of cyanosis. Arterial hemoglobin oxygen saturation (SpO 2) was 86%, with a methemoglobin level of 44.3% and a carboxyhemoglobin level of 0%, while electrolytes, blood urea nitrogen, creatine, and glucose measurement results were normal. He was treated with methylene blue 250 mg (approximately 4 mg/kg) through a nasogastric tube. Four hours after the treatment, because the methemoglobin level was slightly above normal (2.2%), we added 180 mg of methylene blue. The results of final arterial blood gas analysis were a methemoglobin level of 0.4% and a carboxyhemoglobin level of 0.8%. He recovered uneventfully and returned home by himself the next day. To summarize, we successfully treated, with methylene blue given through a nasogastric tube, a young man who had developed severe methemoglobinemia from inhaling automobile exhaust fumes. [ABSTRACT FROM AUTHOR]

The rotational temperature of a nitrogen molecule (N2) was observed to increase proportionally with the substrate-holder temperature in a capacitively coupled rf glow discharge system. The rotational temperature, equivalent to the gas temperature, from the second positive system of the emission spectrum of N2 increased with increasing the mixing ratio of hydrogen, which correlates with the deposition rate, optical band gap, and etching rate of silicon nitride (SiNx). This technique can be used for monitoring the temperature of a SiNx surface during glow discharge processing without any damage to the plasma. [ABSTRACT FROM AUTHOR]