Publications by authors named "Jun Sugaya"

Objective: To clarify the association between skin autofluorescence of advanced glycation end products (AGEs) and clinical outcomes and pain in patients with degenerative cervical myelopathy (DCM).

Methods: Consecutive patients with DCM were prospectively enrolled. AGEs assessed by skin autofluorescence (the AGE score) were examined at the middle fingertip in eligible patients.

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Patients with dialysis are at high risk of reoperation after lumbar spinal fusion surgery. However, the risk of reoperation after short-segment (≤2 fusion levels) lumbar spinal fusion surgery in this patient group has not been completely investigated. This study aimed to compare the risk of reoperation after short-segment lumbar spinal fusion surgery between patients with dialysis and matched controls without dialysis.

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Article Synopsis
  • A multicenter study in Japan compared clinical outcomes for patients with pelvic bone sarcomas treated with surgery versus particle beam therapy (carbon-ion and proton).
  • The study included 116 patients, with 57 receiving surgery and 59 undergoing particle beam therapy, revealing differences in median age at diagnosis and tumor size between the groups.
  • Overall survival, local control, and metastasis-free survival rates were similar between the groups, but in chordoma patients, the particle beam therapy showed significantly better local control rates compared to surgery.
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Pleomorphic sarcoma (PS) is a heterogeneous group of malignant mesenchymal tumors without a specific histological lineage of differentiation. PS is genetically characterized by genetic instability and diversity and histologically characterized by morphological pleomorphism. PS is one of the most common soft tissue sarcomas.

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Article Synopsis
  • This study analyzed the clinical outcomes of 187 patients with pelvic and retroperitoneal bone and soft tissue sarcoma (BSTS) treated at specialized centers in Japan.
  • The 3-year overall survival (OS) rate was 71.7%, while local control (LC) and progression-free survival (PFS) rates were 79.1% and 48.6%, respectively, with osteosarcoma showing significantly worse outcomes.
  • Key poor prognostic factors identified included larger tumor size, soft tissue tumors, presence of distant metastasis, and age, impacting overall survival, local control, and progression-free survival.
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Synovial sarcoma (SS) is a rare and aggressive mesenchymal malignancy driven by a unique chromosomal translocation that generates the expression of the SS18:SSX fusion protein. It occurs at almost any anatomical site and most commonly in young adults. The standard curative treatment for primary SS is a wide surgical resection combined with radiotherapy and/or neoadjuvant chemotherapy.

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Dedifferentiated liposarcoma (DDLPS) is a highly aggressive subtype of liposarcoma that is morphologically defined as a transition from a well-differentiated lipomatous component to a non-lipogenic one. Curative therapy for DDLPS is complete resection, and the benefits of current systemic chemotherapy remain marginal. Although DDLPS is molecularly characterized by co-amplification of MDM2 and CDK4 (12q14-15) and detailed genomic analyses have been conducted by multiple research groups, the effects of molecular targeted drugs are marginal, and novel therapeutic modalities are required.

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Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by a germline mutation of the TP53. The lifetime risk of cancer in individuals with LFS is ≥ 70% for men and ≥ 90% for women. Undifferentiated pleomorphic sarcoma (UPS) is one of the core cancers associated with LFS.

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Myxofibrosarcoma (MFS) is a highly aggressive malignancy with complex karyotypes and a postoperative recurrence tendency, owing to its strong invasiveness. Although systemic chemotherapy is considered in patients with unresectable MFS, the efficacy of conventional chemotherapy is hitherto unclear. Recently, drug screening analysis using a large number of tumor cell lines has been attempted to discover novel therapeutic candidate drugs for common cancers.

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Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of mesenchymal tumors that arise from the sheath of peripheral nerves. MPNSTs exhibit strong metastatic potential, leading to poor clinical outcomes. The clinical utility of radiation therapy and chemotherapy is marginal with respect to overall survival and effective systematic therapies for MPNSTs are still needed to improve patient outcome.

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Undifferentiated pleomorphic sarcoma (UPS), previously termed malignant fibrous histiocytoma, is one of the most aggressive sarcomas with no identifiable line of differentiation. Although the molecular mechanism of oncogenesis in UPS has not been clarified, radiation exposure is considered to be a risk factor in the development of UPS. In the treatment of UPS, surgical treatment remains the most important modality.

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Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma genetically characterized by the presence of the FUS-CREB3L2 gene fusion. While LGFMS exhibits indolent features during its early stages, the rates of recurrence, metastasis, and death from the disease are high. Presently, the role of FUS-CREB3L2 gene fusions in the unique features of LGFMS is not clear, and there is no modality to improve the clinical outcomes of patients with LGFMS; thus, extensive studies on LGFMS are required.

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Myxofibrosarcoma (MFS) is an aggressive sarcoma with a highly complex karyotype. Complete resection is the only curative treatment for MFS because it is refractory to chemotherapy. To improve clinical outcomes, it is critical to develop novel treatments for MFS.

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Giant cell tumor of bone (GCTB) is a locally aggressive and rarely metastasizing tumor. GCTB is characterized by the presence of unique giant cells and a recurrent mutation in the histone tail of the histone variant H3.3, which is encoded by H3F3A on chromosome 1.

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Spindle cell/sclerosing rhabdomyosarcoma (ssRMS) is a rare subtype of rhabdomyosarcoma (RMS) that has fascicular spindle cell and/or sclerosing morphology. SsRMS has a diverse molecular background and is categorized into three groups: congenital/infantile ssRMS with a gene fusion involving the NCOA2 and VGLL2, ssRMS with the MYOD1 mutation, and ssRMS with no recurrent identifiable genetic alterations. Because ssRMS is a newly defined disease concept of RMS, the optimal treatment methods have not been determined.

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Myxofibrosarcoma (MFS) is one of the most aggressive sarcomas with highly complex karyotypes and genomic profiles. Although a complete resection is required in the treatment of MFS, it is often not achieved due to its strong invasive nature. Additionally, MFS is refractory to conventional chemotherapy, leading to poor prognosis.

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Dedifferentiated liposarcoma (DDLPS) is a highly malignant subtype of liposarcoma, with characteristic amplification of MDM2 and CDK4 (12q14-15). It is caused by the dedifferentiation of well-differentiated liposarcoma. DDLPS is refractory to conventional chemotherapy; thus, surgical resection is the primary treatment modality.

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Synovial sarcoma (SS) is defined as a monomorphic blue spindle cell sarcoma showing variable epithelial differentiation, and is characterized by a specific fusion gene, SS18-SSX. Although SS is rare, it accounts for approximately 8% of all soft tissue sarcomas, which occupies a significant proportion of soft tissue tumors. The prognosis of SS is unfavorable, with 5-year survival rate of 50-60%, and only a few anti-cancer agents are recommended for its treatment.

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Article Synopsis
  • This study explored the clinical outcomes of 43 patients with bone sarcomas who underwent unplanned surgeries across 23 hospitals in Japan from 2006 to 2017, focusing on patterns and results.
  • The majority of patients had osteosarcoma or Ewing sarcoma, with common primary tumor locations being the femur and spine; unplanned procedures included curettage and surgical resections, often accompanied by additional treatments.
  • The 5-year disease-specific survival rates varied by tumor type, with a significant decrease in survival for patients who experienced local recurrences compared to those who did not.
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Dedifferentiated liposarcoma (DDLPS) is one of the four subtypes of liposarcomas; it is characterized by the amplification of the 12q13-15 region, which includes MDM2 and CDK4 genes. DDLPS has an extremely high local recurrence rate and is refractory to chemotherapy and radiation, which leads to poor prognosis. Therefore, a novel therapeutic strategy should be urgently established for improving the prognosis of DDLPS.

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Article Synopsis
  • Alveolar soft-part sarcoma is a rare mesenchymal cancer with a poor long-term prognosis due to late metastases, and current treatments have not been effective.
  • The study successfully established a new cell line (NCC-ASPS1-C1) derived from patient tumor tissue, which maintains the disease's unique genetic characteristics, including the ASPSCR1-TFE3 fusion gene.
  • Screening revealed that the MET inhibitor tivantinib and the multi-kinase inhibitor orantinib effectively inhibit the growth of these cells, suggesting potential new therapeutic avenues worth exploring in further research.
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Spindle-cell/sclerosing rhabdomyosarcoma (ssRMS) is a rare subtype of rhabdomyosarcoma, characterized by unique pathological features. Although distinctive molecular backgrounds such as frequent mutations in MyoD1 have been reported, optimized therapy has not been fully developed, and further investigations are required. Patient-derived cancer models are critical tools for basic and pre-clinical studies.

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Synovial sarcoma is a rare malignancy of mesenchymal origin, characterized by a chromosomal translocation, t(X;18) (p11.2;q11.2).

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CIC-DUX4 sarcoma (CDS), an aggressive soft tissue sarcoma, is characterized by a CIC and DUX4 rearrangement. It has a dismal clinical course and high metastatic rate and shows chemotherapy resistance; therefore, a novel therapeutic strategy is required. Patient-derived cell lines are indispensable tools for basic and preclinical research.

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