MS-stable/TMB-high pleomorphic liposarcoma successfully treated with pembrolizumab: a case report.

Pleomorphic liposarcoma (PLPS) is a rare and aggressive subtype of liposarcoma with limited treatment options. Despite studies on immune checkpoint inhibitors (ICIs) in sarcomas, there have been few reports involving PLPS. Furthermore, the significance of tumor mutation burden (TMB)-high, a known biomarker for ICIs in various solid tumors, remains unclear in sarcomas.

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.

Travel distance to tertiary sarcoma centres does not influence oncological presentation or outcomes.

Aims: Soft-tissue sarcomas (STSs) are rare cancers with centralized care advocated to consolidate resources and expertise. However, geographical challenges, particularly in countries like Canada, can increase travel distances for patients. The impact of travel distance on sarcoma presentation and outcomes remains unclear, particularly in single-payer healthcare systems with centralized care.

Identifying consensus and areas for future research in chondrosarcoma : a report from the Birmingham Orthopaedic Oncology Meeting.

Aims: The Birmingham Orthopaedic Oncology Meeting (BOOM), held in January 2024, convened 309 delegates from 53 countries to discuss and refine 21 consensus statements on the optimal management of chondrosarcoma.

Methods: With representation from Europe (43%; n = 133), North America (17%; n = 53), South America (16%; n = 49), Asia (13%; n = 40), Australasia (5%; n = 16), the Middle East (4%; n = 12), and Africa (2%; n = 6), the combined experience of treating bone sarcomas among attendees totalled approximately 30,000 cases annually, equivalent to 66 years of experience in the UK alone. The meeting's process began with the formation of a local organizing committee, regional leads, and a scientific committee comprising representatives from 150 specialist units across 47 countries.

Curative-intent surgery for solitary bone metastasis from extremity and trunk wall sarcoma: What are the outcomes and complications?

Introduction: Approximately 40-50 % of sarcoma patients will develop lung metastasis, but only 10 % will develop bone metastasis. The survival benefit of surgery for solitary bone metastasis remains unclear.

Methods: From 1987 to 2019, 47 patients who underwent curative-intent treatment for localized bone or soft tissue sarcoma in the extremities or trunk wall developed solitary bone metastases as the first distant recurrence.

The utility of intraoperative marrow margin frozen section in extremity bone sarcoma resection.

Background And Objectives: Intraoperative frozen section analysis is commonly used to evaluate marrow margins during extremity bone sarcoma resections, but its efficacy in the era of magnetic resonance imaging is debated. This study aimed to compare the accuracy of intraoperative frozen section assessment with final pathology, assess its correlation with gross intraoperative margin assessment, and evaluate its impact on surgical decision making.

Methods: Consecutive patients undergoing extremity bone sarcoma resections from 2010 to 2022 at a single sarcoma center were included.

Embryonal and alveolar rhabdomyosarcoma in adolescents/young adults, adults and older adults: a population-based cohort study.

Background: The clinical characteristics, outcomes, and prognostic factors of adult embryonal rhabdomyosarcomas (ERMS) and alveolar rhabdomyosarcomas (ARMS), particularly the differences among adolescents/young adults (AYA), adults, and older adults, remain unclear. We assessed the clinicopathological features and survival outcomes of adult patients with ERMS and ARMS in Japan and to compare these features among AYA, adult, and older adult patients.

Methods: We retrospectively analyzed data from the Bone and Soft Tissue Tumor Registry of Japan and enrolled patients aged ≥15 years with ERMS and ARMS.

Clinical genomic profiling of malignant giant cell tumor of bone: A retrospective analysis using a real‑world database.

Malignant giant cell tumor of bone (GCTB) is identified by the presence of multinucleated giant cells, with an aggressive behavior and a high risk of metastasis, which has not been genetically characterized in detail. H3 histone family member 3A () gene mutations are highly recurrent and specific in GCTB. The present study analyzed the clinical information and genomic sequencing data of eight cases of malignant GCTB (out of 384 bone sarcoma samples) using an anonymized genomic database.

Myeloid sarcoma and pathological fracture: a case report and review of literature.

Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML), myelodysplastic syndromes/neoplasms (MDS), chronic myeloid leukemia (CML), and myeloproliferative neoplasm (MPN). Myeloid sarcoma disrupts the normal architecture of its surrounding tissues. When it forms in long bones, it can cause their pathological fracture.

Atypical Neurofibromatous Neoplasm with Uncertain Biologic Potential in the Posterior Mediastinum of a Young Patient with Neurofibromatosis Type 1: A Case Report.

Atypical neurofibromatous neoplasm with unknown biological potential (ANNUBP), proposed in a recent NIH consensus overview, is a rare precursor entity of malignant peripheral nerve sheath tumor (MPNST) in neurofibromatosis type 1 (NF1) patients. Only one report on imaging findings of ANNUBP is available. Herein, we present the case of a 19-year-old female, diagnosed with a mediastinal tumor by chance, who visited to our hospital.

Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study.

Background: Primary tumor resection is the mainstay of treatment for malignant peripheral nerve sheath tumors. However, the efficacy of perioperative chemotherapy and radiotherapy for malignant peripheral nerve sheath tumors has not been elucidated.

Methods: This retrospective analysis based on a Japanese registry included patients with localized malignant peripheral nerve sheath tumors arising at the extremities and trunk wall.

Preoperative arterial embolization and wound complications after resection of malignant bone tumor in the pelvis: a nationwide database study.

Background: Massive intraoperative blood loss is common in pelvic malignant bone tumor surgery, and preoperative arterial embolization may be used in selected cases. Preoperative arterial embolization reportedly increases wound complications in pelvic fracture surgery, but little evidence is available regarding pelvic bone tumor surgery.

Methods: Using a Japanese nationwide database (Diagnosis Procedure Combination database), we searched for patients who underwent pelvic malignant bone tumor surgery between July 2010 and March 2018.

Clinical characteristics of undifferentiated pleomorphic sarcoma of bone and the impact of adjuvant chemotherapy on the affected patients: a population-based cohort study.

Background: Clinical characteristics of undifferentiated pleomorphic sarcoma of bone are not elucidated. Herein, we clarify its clinical features and analyze the efficacy of adjuvant chemotherapy in patients with undifferentiated pleomorphic sarcoma of bone.

Methods: Prognostic factors and estimated disease-specific survival in 247 patients with primary undifferentiated pleomorphic sarcoma of bone were identified from a registry in Japan.

Role of adjuvant chemotherapy in patients with localized, undifferentiated pleomorphic sarcoma of soft tissue: a population-based cohort study.

Background: Primary tumor resection is the mainstay of treatment for undifferentiated pleomorphic sarcoma (UPS); however, the necessity of adjuvant chemotherapy has been debated. We aimed to clarify the effect of adjuvant chemotherapy on survival rates in patients with UPS with localized and resectable primary lesions.

Methods: This retrospective analysis included data of 2112 patients with localized UPS arising in the extremities and trunk, extracted from a registry in Japan.

Comparison of clinical features and outcomes of patients with leiomyosarcoma of bone and soft tissue: a population-based cohort study.

Background: Leiomyosarcoma commonly occurs in soft tissue but rarely in the bone. Whether leiomyosarcoma of bone and soft tissue have similar clinical characteristics and outcomes remains unknown.

Methods: This retrospective analysis was based on data from the Bone and Soft Tissue Tumor Registry in Japan.

Hemangioma of the Rib Mimicking Chondrosarcoma: A Report of Two Cases and Literature Review.

Cases: Case 1 was a 58-year-old man who presented with an incidentally detected, slowly growing mass in the right hypochondrium area. An imaging study showed the mass arising from the 11th rib, with ill-defined margins and cortical destruction. Differential diagnoses included chondrosarcoma and metastatic malignant tumor.

Prognostic factors and impact of surgery in patients with metastatic soft tissue sarcoma at diagnosis: A population-based cohort study.

Background: Approximately 10% of the patients with soft tissue sarcoma show metastasis at initial diagnosis, and hence, poorer prognosis. However, the prognostic factors and whether definitive surgery for the primary lesion improves overall survival, especially when complete resection of metastasis is difficult, remain unclear.

Methods: This retrospective analysis was based on the Bone and Soft Tissue Tumor Registry in Japan.