Surgical management of multiple schwannomas scattered on a single peripheral nerve.

Background: Schwannoma is a common benign tumor. Most schwannomas are sporadic, but approximately 5% of schwannomas are multifocal. Schwannomas are sometimes present in a skip-like pattern on a single continuous peripheral nerve (Multiple schwannomas scattered on a single peripheral nerve: MSSPN).

Methotrexate, Doxorubicin, and Cisplatin Versus Methotrexate, Doxorubicin, and Cisplatin + Ifosfamide in Poor Responders to Preoperative Chemotherapy for Newly Diagnosed High-Grade Osteosarcoma (JCOG0905): A Multicenter, Open-Label, Randomized Trial.

Purpose: Our previous NECO phase II studies on high-grade osteosarcoma suggested that administering ifosfamide (IF; 16 g/m [4g/m once on day 1, then 2g/m once on days 2-7] × six) to patients showing a poor response (PrRsp) to preoperative chemotherapy with methotrexate, doxorubicin, and cisplatin (MAP) improves their prognoses. In this Japan Clinical Oncology Group (JCOG) study, JCOG0905, we aimed to investigate the efficacy and safety of IF in patients with PrRsp.

Methods: JCOG0905 is a multicenter, open-label, multi-institutional, randomized trial.

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.

Gemcitabine-Based Chemotherapy for Advanced Soft Tissue Sarcoma: Identifying the Appropriate Dose and Schedule.

Background In 2024, reimbursement for gemcitabine-docetaxel therapy (GEM-DOC; gemcitabine 900 mg/m on days 1 and 8 and docetaxel 70 mg/m on day 8 every 21 days, GEM 900-DOC 70) to treat recurrent/metastatic soft tissue sarcoma (STS) was made in Japan. Methods We retrospectively reviewed clinical records of advanced/metastatic STS patients who underwent off-label gemcitabine-containing chemotherapy at the Cancer Institute Hospital of the Japanese Foundation for Cancer Research between February 2007 and October 2019. Results Of 115 enrolled patients, 51 were treated with GEM-DOC (26 patients received the dose as previously stated) and the other 64 with gemcitabine monotherapy.

Confirmatory diagnosis and successive chemotherapeutic treatments of metastatic skeletal sarcoma: A case report.

sarcoma is rare and its clinical features remain unclear. Given the similarity in presentation, it is possible that previously reported cases of Ewing-like adamantinoma may have been sarcoma. The present case report describes a tumor in a 55-year-old man that was originally thought to be a Ewing-like adamantinoma, but was recently found to be an sarcoma following direct sequencing.

Impact of Technological Advancements on Short-term Outcomes in Flap Reconstruction after Soft Tissue Sarcoma Resection: A Retrospective Comparative Analysis.

Background:  Soft tissue sarcomas (STS) are rare malignancies requiring extensive surgical resection, often leading to significant soft tissue defects. Flap reconstruction is crucial for restoring function and appearance. Recent reconstructive microsurgery advancements, including high-resolution indocyanine green (ICG) imaging and ultra-high frequency ultrasonography (UHFU), have revolutionized preoperative planning and intraoperative guidance.

Frequent copy number gain of MCL1 is a therapeutic target for osteosarcoma.

Osteosarcoma (OS) is a primary malignant bone tumor primarily affecting children and adolescents. The lack of progress in drug development for OS is partly due to unidentified actionable oncogenic drivers common to OS. In this study, we demonstrate that copy number gains of MCL1 frequently occur in OS, leading to vulnerability to therapies based on Mcl-1 inhibitors.

The association between CD34 expression status and the clinicopathological behavior of dedifferentiated liposarcoma.

Lipogenic and fibrous tumors are thought to originate from CD34-positive stromal fibroblastic/fibrocystic cells. Well-differentiated lipogenic tumors typically express CD34, whereas dedifferentiated liposarcoma (DDLPS) often loses it. We conducted survival analyses involving 59 patients with DDLPS.

Bipolar Head Perforation With Rhabdomyosarcoma of the Thigh: A Case Report With Literature Review.

Background/aim: High complication rates during the perioperative management of sarcomas around the pelvis have been reported; however, few include the detailed clinical course or complications in the late postoperative period. Radiotherapy is a multidisciplinary strategy for treating sarcomas. However, irradiated bone and soft tissues show a permanent loss of repair and immunocompetence.

Bone metastases with "false negative" findings on 18F-FDG PET/CT in patients with angiosarcoma: A case series with literature review.

Rationale: Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) is considered a reliable and indispensable imaging method when evaluating distant metastases and clinical staging of angiosarcomas. Here, we report 2 cases of angiosarcoma with bone metastases with "false negative" findings on 18F-FDG PET/CT.

Patient Concerns: Case 1, a 39-year-old woman, who had undergone mastectomy for primary angiosarcoma 2 years prior, presented with a 5-month history of right coxalgia.

Clinical Outcome of Low-Grade Myofibroblastic Sarcoma in Japan: A Multicenter Study from the Japanese Musculoskeletal Oncology Group.

This retrospective multicenter study aimed to analyze the clinical features and prognosis of 24 patients diagnosed with LGMS between 2002 and 2019 in the Japanese sarcoma network. Twenty-two cases were surgically treated and two cases were treated with radical radiotherapy (RT). The pathological margin was R0 in 14 cases, R1 in 7 cases, and R2 in 1 case.

ASPSCR1::TFE3 orchestrates the angiogenic program of alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is a soft part malignancy affecting adolescents and young adults. ASPS is characterized by a highly integrated vascular network, and its high metastatic potential indicates the importance of ASPS's prominent angiogenic activity. Here, we find that the expression of ASPSCR1::TFE3, the fusion transcription factor causatively associated with ASPS, is dispensable for in vitro tumor maintenance; however, it is required for in vivo tumor development via angiogenesis.

Clinicopathologic and genetic characterization of angiofibroma of soft tissue: a study of 12 cases including two cases with AHRR::NCOA3 gene fusion.

Aims: Angiofibroma of soft tissue (AFST) is a benign tumour characterised by prominent arborizing blood vessels throughout the lesion. Approximately two-thirds of AFST cases were reported to have AHRR::NCOA2 fusion, and only two cases have been reported to have other gene fusions: GTF2I::NCOA2 or GAB1::ABL1. Although AFST is included in fibroblastic and myofibroblastic tumours in the World Health Organization's 2020 classification, histiocytic markers, especially CD163, have been reported to be positive in almost all examined cases, and it still remains the possibility of a fibrohistiocytic nature of the tumour.

Clinical characteristics of sarcoma cases in which long-term disease control was achieved with trabectedin treatment: A retrospective study.

Trabectedin is a therapeutic option for patients with advanced sarcoma. While a randomized trial demonstrated its prolonged progression-free survival (PFS), the reported PFS was <6 months. Some patients can achieve long-term disease control with this treatment.

Imaging findings of NTRK‑rearranged spindle cell neoplasms: A case series.

NTRK-rearranged spindle cell neoplasms (NTRK-RSCNs) are a new category of soft tissue tumors with gene fusions. The present study aimed to investigate the radiological features of NTRK-RSCNs and their association with histopathological findings. The present study included six patients with NTRK-RSCNs, whose fusion genes were confirmed using next-generation sequencing.

The Clinical Features of Multicentric Extra-abdominal Desmoid Tumors.

Background: Extra-abdominal desmoid tumors often occur in the necks, shoulder, chest wall, back, arm, buttock, thigh and leg. Multicentric extra-abdominal desmoids are rather rare and seem to have other clinical features. The aim of our study was to investigate clinical features, especially multicentric occurrence of extra-abdominal desmoid tumors.

Changes in Neutrophil-to-lymphocyte Ratio Predict Efficacy of Trabectedin for Soft-tissue Sarcoma.

Background/aim: Trabectedin and eribulin are widely used for the treatment of soft-tissue sarcoma (STS). Previously it was shown that the baseline neutrophil-to-lymphocyte ratio (NLR) predicts the efficacy of eribulin for STS. However, prognostic factors for trabectedin on STS have not been identified to date.