Clinical outcomes and complications associated with surgical resection of chest wall sarcoma.

Introduction: Chest wall sarcomas are rare malignant mesenchymal tumors arising from soft tissue, cartilage and bones. Wide resection is usually the treatment option but often results in postoperative complications after resection of the chest wall. In this study, we reviewed cases of chest wall sarcoma treated with resection of the sternum or ribs and examined the factors that could cause complications.

Epidemiology and Outcomes of Retroperitoneal Sarcoma: An Analysis of the Population-Based Cancer Registry in Japan 2016-2019.

Background: Retroperitoneal sarcoma (RPS) is a rare cancer, so few reports have previously characterized its national profiles. The capture rate of RPS in the Bone and Soft Tissue Tumor Registry was only 20%. The present study aimed to clarify the characteristics and clinical outcomes of RPS using the National Cancer Registry (NCR), which contains nationwide population-based data from Japan.

The role of radiotherapy and chemotherapy in extraskeletal myxoid chondrosarcoma.

Background: Extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare subtype of sarcoma characterized by NR4A3 gene rearrangement. Despite being considered slowly progressive sarcomas, EMCs tend to have local recurrences and distant metastases in the late stages. This study aimed to investigate the prognostic factors of EMCs, especially the effect of (neo)adjuvant radiotherapy or chemotherapy on localized EMCs and chemotherapy on advanced-stage EMCs.

Impact of Postoperative Infection on Lower Limb Function After Surgery for Malignant Bone and Soft Tissue Tumors: Data from a Nationwide Registry in Japan.

Background: Due to the scarcity of cases, adjustments for confounding factors in analyses of the effects of postoperative infections on limb function after surgery for malignant bone and soft tissue tumors were insufficient in previous studies. One solution is to use big data from a nationwide registry.

Methods: Data from the Bone and Soft Tissue Tumor Registry in Japan were used to examine the impact of postoperative infections on limb function after surgery for malignant bone and soft tissue tumors in the lower extremities.

Metabolome Profiling of Yokukansan in Preventing Postoperative Delirium in Elderly Cancer Patients: A Reverse Translational Study.

Aims: Postoperative delirium (PD) is a common and severe complication in older adult patients undergoing invasive cancer resections. This study explored the plasma metabolome associated with PD and evaluated the efficacy of Yokukansan (YKS), a traditional Japanese Kampo medicine, in preventing PD.

Methods: An ancillary study was conducted alongside a double-blind, placebo-controlled randomized clinical trial involving patients 65 years and older, focusing on patients older than 75 years as a primary analysis population.

A comparative analysis of hand sarcoma by anatomical site: A case series.

Hand sarcomas are very rare, and few studies have focused on their anatomical distribution, such as involvement of the fingers vs. the palm/dorsum (PD). The present study reviewed 24 cases of hand sarcomas identified from medical records of National Cancer Centre Hospital (Tokyo, Japan) between January 2010 and December 2022, examining sex, age, maximum tumour size, metastasis, histology, origin (bone vs.

Epidemiologic Features and Age-Related Differences in Management among Patients with Gastrointestinal Stromal Tumors in Japan: A National Cancer Registry Study.

Unlabelled: Data on the epidemiology of gastrointestinal stromal tumor (GIST) and differences in its management according to age group are limited in Japan. We aimed to conduct an epidemiologic evaluation and describe age-related differences in management using data from Japan's National Cancer Registry. We analyzed National Cancer Registry data of 21,426 patients with GIST between 2016 and 2019.

Establishment and characterization of two novel patient-derived cell lines from giant cell tumor of bone: NCC-GCTB11-C1 and NCC-GCTB12-C1.

Giant cell tumor of bone (GCTB) is locally aggressive and rarely metastasizing mesenchymal tumor, molecularly characterized by the presence of H3-3A mutation. The management of GCTB is problematic because of local recurrence after curative surgical treatment, and complex biological and molecular backgrounds of etiology and disease progression. Development of multidisciplinary therapy has been required in GCTB, and targeting treatments against nuclear factor kappa-B ligand and epidermal growth factor receptor to neoplastic stromal cells were applied to the clinical practice.

Favorable response to nivolumab combined with radiotherapy for retroperitoneal leiomyosarcoma.

Unlabelled: Retroperitoneal sarcoma (RPS) is a biologically heterogeneous tumor and rare malignant mesenchymal soft-tissue neoplasm. Although the 5-year overall survival rate for RPS is approximately 60%-70%, it is quite low for unresectable tumors. Surgery is a standard treatment for RPS.

Is surgical resection of the primary site associated with longer survival in patients with metastatic chondrosarcoma at initial diagnosis?

Purpose: Patients with metastatic chondrosarcoma at presentation have a poor prognosis. Owing to the lack of sensitivity to chemotherapy and radiotherapy, surgical resection is the mainstay of treatment of localized chondrosarcoma. The aim of this study was to investigate whether surgical resection of the primary site was associated with improved survival in patients with metastatic chondrosarcoma at presentation.

Results of a Phase II Trial for Intermediate-Risk Rhabdomyosarcoma Treatment Protocol JRS-I: A Report From the Japan Rhabdomyosarcoma Study Group.

Purpose: To evaluate the safety and efficacy of multimodality treatment with vincristine, actinomycin D, and cyclophosphamide (VAC) therapy, surgery, and radiotherapy according to the US Intergroup Rhabdomyosarcoma Study IV (IRS-IV), and to establish a central review system and standard treatment for intermediate-risk pediatric rhabdomyosarcoma in Japan.

Patients And Methods: The Japan Rhabdomyosarcoma Study-I (JRS-I) was a single-arm, phase II trial for intermediate-risk rhabdomyosarcoma treatment with open enrollment from June 2004 to March 2009. Patients received 12 cycles of VAC every 3 weeks for 42 weeks, with local therapy beginning after Week 12.

Establishment and characterization of NCC-EMC1-C1: a novel patient-derived cell line of extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma characterized by a myxoid matrix and a distinctive lobulated architecture, composed of cords and clusters of uniform round-to-rhabdoid cells. At the molecular level, EMC is defined by specific gene fusions involving NR4A3, most frequently EWSR1::NR4A3. The responses to conventional chemotherapy are limited, and the prognosis for patients with advanced or metastatic disease remains poor.

Pharmacokinomic Profiling Using Patient-Derived Cell Lines Predicts Sensitivity to Imatinib in Dermatofibrosarcoma Protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma, characterized by a fusion. Imatinib, a multi-target tyrosine kinase inhibitor, is a standard treatment of DFSP. However, resistance emerges in 10-50% of cases.

Incidence, risk factors, and prognostic impact of regional lymph node metastasis in bone sarcoma: a population-based cohort study.

Background: This study aimed to investigate the risk factors and prognostic impact of regional lymph node metastasis (RLNM) in patients with bone sarcoma.

Methods: This retrospective study analyzed data from a Japanese registry of patients with bone sarcoma (2006-19). Disease-specific overall survival was estimated using the Kaplan-Meier method.

Incidence and age- and site-specific characteristics of osteosarcoma: a population-based study using National Cancer Registry 2016-2019.

Background: No previous reports have characterized national profiles of osteosarcoma. In this study, we examined nationwide statistics for osteosarcoma in Japan using data from the National Cancer Registry (NCR), a population-based cancer registry launched in 2016.

Methods: We identified 1187 patients with osteosarcomas entered in the NCR during 2016-2019 using the cancer topography and morphology codes from the International Classification of Diseases for Oncology, Third Edition.

Osteosarcoma in Japan: report from the bone and soft tissue tumor registry 2006-2022.

Background: Osteosarcoma is the most common primary malignant bone tumor. Although previous studies reported genetic differences between younger and older patients, comprehensive nationwide data remain scarce. This study aimed to describe age-related differences in demographics, treatment, and survival using Japan's Bone and Soft Tissue Tumor (BSTT) Registry.

The prognostic factors and treatment patterns of the first recurrence of retroperitoneal liposarcoma.

Background: This study aimed to identify prognostic factors and treatment patterns at the first recurrence of retroperitoneal liposarcoma (LPS).

Methods: Medical records of 150 patients who underwent resection for primary retroperitoneal LPS were reviewed. Of the 94 patients with local or distant recurrence, prognostic factors and treatment approaches were retrospectively analyzed.

Establishment and characterization of NCC-MLPS4-C1: a novel patient-derived cell line of myxoid liposarcoma.

Myxoid liposarcoma (MLPS) is a malignant tumor composed of uniform, round-to-ovoid cells and small lipoblasts embedded in a myxoid stroma containing branching capillaries. MLPS is characterized by the presence of fusion genes, primarily FUS::DDIT3, with EWSR1::DDIT3 identified in a subset of cases. Although multimodal therapies have improved outcomes for localized MLPS, the prognosis for recurrent or metastatic MLPS cases remains poor, underscoring the need for novel therapeutic strategies.

Proposal for a New Classification of Rare Cancers Adopting Updated Histological Tumor Types.

Several classifications have been proposed to define rare cancers; however, the pathophysiological understanding of tumors evolves rapidly. We propose a New Classification of Rare Cancer (NCRC) using the updated International Classification of Diseases for Oncology 3.2 coding system and World Health Organization Classification of Tumors 5th edition.

Epigenetic disruption of adipogenic gene enhancers in dedifferentiated liposarcomas and its therapeutic value.

Liposarcoma (LPS) is the most common soft-tissue sarcoma in adults, and well-differentiated liposarcoma (WDLPS) and dedifferentiated liposarcoma (DDLPS) are the most frequent subtypes. These LPSs are considered to develop due to disturbances in the adipogenic differentiation of mesenchymal stem cells. However, the molecular mechanisms underlying the disturbances remain unclear.

The impact of adjuvant therapy in extraskeletal osteosarcoma: A propensity score-matched analysis of 226 cases from the bone and Soft Tissue Tumor Registry in Japan.

Background: Extraskeletal osteosarcoma (ESOS) is a rare malignant soft tissue tumor for which optimal treatment strategies remain unclear. Although several retrospective studies have suggested the potential efficacy of adjuvant therapies, selection bias has limited the reliability of these findings. This study analyzes the largest ESOS cohort using the Japanese Bone and Soft Tissue Tumor (BSTT) Registry, employing propensity score matching to evaluate treatment outcomes while minimizing selection bias.

Establishment and characterization of NCC-PS2-C1: a novel cell line of high-grade pleomorphic spindle cell sarcoma, most consistent with myxofibrosarcoma.

Pleomorphic sarcoma (PS) is a heterogeneous group of malignant mesenchymal tumors that lack specific histological differentiation. PS is characterized by genetic instability and diversity and unique histological features such as pronounced morphologic pleomorphism. PS is one of the most common soft tissue sarcomas.

Statistics of Primary Malignant Spine Tumors in Japan: A Retrospective Study Using the Bone and Soft Tissue Tumor Registry in Japan.

Study Design: A retrospective registry-based study.

Objective: To elucidate the nationwide epidemiology, treatment patterns, and prognosis of primary malignant spine tumors (PMST) using the Japanese Orthopaedic Association's Bone and Soft Tissue Tumor (BSTT) Registry.

Summary Of Background Data: PMSTs are rare, comprising a small proportion of primary malignant bone tumors.

Prognostic potential of fusion gene analysis using plasma cell-free RNA in malignant bone and soft tissue tumours.

Background: Liquid biopsy, which facilitates minimally invasive analysis of body fluid samples, has considerable potential as a diagnostic and prognostic tool in various cancers. Analysis of circulating tumour cells, circulating tumour DNA, and exosomes in liquid biopsies has advantages and disadvantages. However, their utility in rare cancers, such as malignant bone and soft tissue tumours, remains unknown.