AIM Chordoma is a rare, slow-growing primary malignant tumor of the axial skeleton, arising from the embryonic cells of primitive notochord. Chordomas may arise at different sites of the vertebral column simultaneously or more probably they may metastasise along the neural axis insidiously. Recurrence despite radical surgery and following adjuvant therapy is possible. MATERIAL AND METHODS A 4...

Chordoma is a malignant tumor thought to arise from remnants of the embryonic notochord, with its origin in the bones of the axial skeleton. Surgical resection is the standard treatment, usually in combination with radiation therapy, but neither chemotherapeutic nor targeted therapeutic approaches have demonstrated success. No animal model and only few chordoma cell lines are available for prec...

Chordomas are rare bone tumors, developed from the notochord and largely resistant to chemotherapy. A special feature of this tumor is the heterogeneity of its cells. By combining high pressure freezing (HPF) with electron tomography we were able to illustrate the connections within the cells, the cell-cell interface, and the mitochondria-associated endoplasmic reticulum membrane complex that a...

Chordomas are malignant tumors that originate in embryonic notochordal remnants. The sacrum and skull are the most common sites; the mobile spine and other bones are extremely rare sites. We describe a 45-year-old man who presented with a lytic lesion in his left ilium. Imaging and pathology of a biopsy specimen suggested a malignant bone tumor; wide resection was accordingly performed. The mor...

The molecular mechanisms underlying chordoma pathogenesis are unknown. We therefore sought to identify novel mutations to better understand chordoma biology and to potentially identify therapeutic targets. Given the relatively high costs of whole genome sequencing, we performed a focused genetic analysis using matrix-assisted laser desorption/ionization-time of flight mass spectrometer (Sequeno...

Skull base chordoma is a primary rare malignant bone-origin tumor showing relatively slow growth pattern and locally destructive lesions, which can only be characterized by histologic components. There is no available prognostic or therapeutic biomarker to predict clinical outcome or treatment response and the molecular mechanisms underlying chordoma development still remain unexplored. Therefo...

The classical sacrococcygeal chordoma tumor presents with a typical morphology of lobulated myxoid tumor tissue with cords, strands and nests of tumor cells. The population of cells consists of small non-vacuolated cells, intermediate cells with a wide range of vacuolization and large heavily vacuolated (physaliferous) cells. To date analysis was only performed on bulk tumor mass because of its...

Chordomas are malignant, nonepithelial neoplasms derived from notochordal tissue. A primary chordoma of the nasal cavities and paranasal sinuses is extremely rare compared with clival chordomas, which often present as nasal masses after spreading anteriorly. Only a few cases of primary chordoma of the nasal cavities and paranasal sinuses have been reported in the literature. We report a case of...

We explored the general feasibility of proton beam therapy for chordoma and chondrosarcoma of the skull base. Clinical records and treatment-planning data of patients with the pathological diagnosis of chordoma or chondrosarcoma were examined. Proton beam therapy was administered for gross tumor mass as well as microscopic residual disease after surgery. The prescribed dose was determined to ma...

PURPOSE Chordomas are rare, malignant bone neoplasms in which the pathogenic mechanisms remain unknown. Interestingly, tuberous sclerosis complex (TSC) is the only syndrome in which the incidence of chordomas has been described. We previously reported the pathogenic role of the TSC genes in TSC-associated chordomas. In this study, we investigated whether aberrant TSC/mammalian target of rapamyc...