Chordoma

Introduction: The aims of our study were (1) to determine disease-specific and disease-free survival after the en-bloc resection of sacral chordomas and (2) to investigate potential risk factors for tumor recurrence and major postoperative wound-related complications. Methods: We retrospectively analyzed 27 consecutive patients with sacral chordomas who were surgically treated in our institution between 2004 and 2022. Three patients (11.1%) had a recurrent tumor and four patients (14.8%) had history of a second primary solid tumor prior to or after their sacral chordoma...

Chordomas are clinically aggressive tumors with a high rate of disease progression despite maximal therapy. Given the limited therapeutic options available, there remains an urgent need for the development of novel therapies to improve clinical outcomes. Cell surface proteins are attractive therapeutic targets yet are challenging to profile with common methods. Four chordoma cell lines were analyzed by quantitative proteomics using a differential ultracentrifugation organellar fractionation approach. A subtractive proteomics strategy was applied to select proteins that are plasma membrane enriched...

BACKGROUND: Total sacrectomy is a technically demanding surgery with substantial risks, including high morbidity and mortality due to the likelihood of exsanguination.1-3 Despite the evolution of surgical techniques,4,5 the incidence of postoperative complications remains significant.1 This study presents a systematic approach to total sacrectomy, with a particular focus on a modified technique for isolating the iliac vessels, aimed at effective management of complex sacrococcygeal masses and the reduction of operative complications...

Objective  Interaction of tumor cells with the surrounding environment is essential for tumor growth and progression that eventually leads to metastasis. Growing evidence shows that extracellular vesicles also known as exosomes play a crucial role in signaling between the tumor and its microenvironment. Tumor-derived exosomes have generally protumorigenic effects such as metastasis, hypoxia, angiogenesis, and epithelial-mesenchymal transition. Methods  In this study, exosomes were isolated from a chordoma cell line, MUG-Chor1, and characterized subsequently...

BACKGROUND AND PURPOSE: Nuclear interaction correction (NIC) and trichrome fragment spectra modelling improve relative biological effectiveness-weighted dose (DRBE ) and dose-averaged linear energy transfer (LETd ) calculation for carbon ions. The effect of those novel approaches on the clinical dose and LET distributions was investigated. MATERIALS AND METHODS: The effect of the NIC and trichrome algorithm was assessed, creating single beam plans for a virtual water phantom with standard settings and NIC + trichrome corrections...

BACKGROUND: The large soft-tissue defect after total or high sacrectomy for giant sacral tumor induces high incidence of wound complications. It remains a huge challenge to reconstruct the soft-tissue defect and achieve the preferred clinical outcome. METHODS: A total of 27 patients undergoing one-stage total or high sacrectomy for giant sacral tumors between 2016 and 2021 in a tertiary university hospital were retrospectively reviewed. Participants were divided into two groups...

PURPOSE: Cranial nerve involvement (CNI) influences the treatment strategies and prognosis of head and neck tumors. However, its incidence in skull base chordomas and chondrosarcomas remains to be investigated. This study evaluated the imaging features of chordoma and chondrosarcoma, with a focus on the differences in CNI. METHODS: Forty-two patients (26 and 16 patients with chordomas and chondrosarcomas, respectively) treated at our institution between January 2007 and January 2023 were included in this retrospective study...

INTRODUCTION: Because of the co-location of critical organs at risk, base of skull tumours require steep dose gradients to achieve the prescribed dosimetric criteria. When available, proton beam therapy (PBT) is often considered a desirable modality for these cases, but in many instances, compromises in target coverage are still required to achieve critical organ at risk (OAR) tolerance doses. A number of techniques have been proposed to further improve the penumbra of PBT. In the current study, we propose a novel, collimator-free treatment planning technique that combines high-energy shoot-through proton beams with conventional Bragg peak spot placement...

BACKGROUND: The current study aims to evaluate the occurrence of temporal lobe reactions and identify possible risk factors for patients who underwent particle therapy of the skull base. METHODS: 244 patients treated for skull base chordoma ( n = 144) or chondrosarcoma ( n = 100) at the Heidelberg Ion Beam Therapy Center (HIT) using a raster scan technique, were analyzed. Follow-up MRI-scans were matched with the initial planning images. Radiogenic reactions were contoured and analyzed based on volume and dose of treatment...

BACKGROUND: This study aimed to compare the outcomes of proton beam therapy (PBT) and carbon ion radiotherapy (CIRT) by a systematic review and meta-analysis of the existing clinical evidence. METHODS: A systematic literature search was performed to identify studies comparing the clinical outcomes of PBT and CIRT. The included studies were required to report oncological outcomes (local control [LC], progression-free survival [PFS], or overall survival [OS]) or adverse events...

PURPOSE OF REVIEW: This review aims to provide a comprehensive overview of mesenchymal sinonasal tract tumors (STTs), a distinct subset of STTs. Despite their rarity, mesenchymal STTs represent a unique clinical challenge, characterized by their rarity, often slow progression, and frequently subtle or overlooked symptoms. The complex anatomy of the sinonasal area, which includes critical structures such as the orbit, brain, and cranial nerves, further complicates surgical treatment options...

PURPOSE: There is no approved targeted therapy for chordoma at present. Although several preclinical studies have implied the potential applicability of CDK4/6 inhibitor for this rare tumor, no clinical evidence has been documented so far. The purpose of this study was to elucidate the therapeutic efficacy of CDK4/6 inhibitor for chordoma. METHODS: The next generation sequencing (as for whole-exome sequencing, WES assay) and immunohistochemical (IHC) staining of the chordoma tissue from a patient with an advanced lesion were performed before treatment...

BACKGROUND: En-bloc resection remains the cornerstone treatment for malignant bone tumors affecting the spine. The thoracic spine poses unique challenges due to the proximity of crucial structures. This study assesses outcomes of patients who underwent en-bloc spondylectomy for malignant bone tumors at the thoracic level. METHODS: We retrospectively reviewed 85 cases of primary and secondary bone tumors in the thoracic spine, undergoing en-bloc spondylectomy from 1996 to 2016...

INTRODUCTION: Chordomas are rare malignant neoplasms that can originate anywhere along the cerebrospinal axis. However, they are most commonly found in the spine, cranium, and sacrococcygeal region. Chordomas can manifest differently depending on their location and most symptoms are a result of local invasion. We present a rare case of intracranial clival chordoma that manifested as isolated unilateral cranial nerve XII (CN XII) palsy. PRESENTATION: A 53-year-old male presented to the neurosurgical clinic with headaches, dysarthria, and pharyngeal pain...

Chordomas are derived from embryonic notochord remnants and comprise 1%-4% of all bone tumors.1 Nearly all chordomas arise in the axial skeleton, with 50% in the sacrococcygeal region, 35% in the skull base and 15% within mobile spine vertebrae.1,2 Regional recurrence after en bloc surgery is common and 30%-40% of patients develop metastatic disease.3-6 In this operative video, we present a 41-year old man who previously underwent en bloc lateral L1 corpectomy and received high-dose hybrid photon and proton radiation therapy for treatment of his L1 chordoma...

Brachyury is an oncogenic transcription factor whose overexpression drives chordoma growth. The downmodulation of brachyury in chordoma cells has demonstrated therapeutic potential, however, as a transcription factor it is classically deemed "undruggable".  Given that direct pharmacological intervention against brachyury has proven difficult, attempts at intervention have instead targeted upstream kinases. Recently, afatinib, an FDA-approved kinase inhibitor, has been shown to modulate brachyury levels in multiple chordoma cell lines...

Rare cancers (RCs), which account for over 20% of cancer cases, face significant research and treatment challenges due to their limited prevalence. This results in suboptimal outcomes compared to more common malignancies. Rare bone tumors (RBTs) constitute 5-10% of rare cancer cases and pose unique diagnostic complexities. The therapeutic potential of anti-cancer drugs for RBTs remains largely unexplored. Identifying molecular alterations in cancer-related genes and their associated pathways is essential for precision medicine in RBTs...

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INTRODUCTION: The purpose is to clarify the safety and clinical contribution of computed tomography (CT)-guided percutaneous needle-biopsy for patients with cervical spine lesion. MATERIAL AND METHODS: Between June 2015 and August 2022, CT-guided percutaneous needle biopsies were performed for 15 cervical spine lesions of 15 patients (8 male, 7 female; 2-81 years old). The technical success, clinical contribution, and safety were evaluated. Technical success was defined as the completion of the biopsy procedure...

OBJECTIVE: This study was designed to delve into the complexities involved in diagnosing of benign and malignant bone tumors and to assess the potential of AI technologies like ChatGPT in improving diagnostic accuracy and efficiency. The study also explores the few-shot learning as a method to optimize ChatGPT's performance in specialized medical domains such as benign and malignant bone tumors diagnosis. METHODS: A total of 1366 benign and malignant bone tumors-related imaging reports were collected and diagnosed by 25 experienced physicians...