The presentation will be a review of the NCCN guidelines for chordoma management. This review will include the rationale and an overview of chordoma. Characterization of genetic variants in the T gene in familial and sporadic chordoma Chordoma is a rare bone tumor that is believed to originate from notochordal remnants. T gene, which encodes Brachyury, is essential for the proper development an...

BACKGROUND Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically disti...

BACKGROUND Chordoma was a typically slow-growing tumor. The therapeutic approach to chordoma had traditionally relied mainly on surgical therapy. And the main reason for therapeutic failure was resistance to chemotherapy and radiotherapy. However the refractory mechanism was not clear. The aim of this study was to investigate the expression of three genes (MDR1, HIF-1α and MRP1) associated with...

Chordomas are primary malignant tumors of the notochord that are resistant to conventional chemotherapy. Expression of programmed cell death ligand 1 (PD-L1), prevalence of tumor-infiltrating lymphocytes (TILs), and their clinical relevance in chordoma remain unknown. We evaluated PD-L1 expression in three chordoma cell lines and nine chordoma tissue samples by western blot. Immunohistochemical...

Chordoma is a rare slow-growing neoplasm arising from notochordal remnants. In the United States, the annual incidence of chordoma is 0.08 per 100,000 and is more common in men than in women. The most common locations of chordoma are the cranial (32%), spinal (32.8%), and sacral (29.2%) regions [1]. We report an unusual case of pleural chordoma in a 45-year-old man.

Sarcomatous transformation (dedifferentiated chordoma) in chordoma is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma arising from the sacrococcygeal region of a 60-year-old man, in third tumor recurrence. This tumor showed features of sarcoma with areas more typical of chordo...

Chordoma, a rare bone tumor derived from the notochord, has been shown to be resistant to conventional therapies. Checkpoint inhibition has shown great promise in immune-mediated therapy of diverse cancers. The anti-PD-L1 mAb avelumab is unique among checkpoint inhibitors in that it is a fully human IgG1 capable of mediating antibody-dependent cell-mediated cytotoxicity (ADCC) of PD-L1-expressi...

BACKGROUND Chordoma pathogenesis remains poorly understood. In this study, we aimed to evaluate the relationships between microRNA-155 (miR-155) expression and the clinicopathological features of chordoma patients, and to evaluate the functional role of miR-155 in chordoma. METHODS The miRNA expression profiles were analyzed using miRNA microarray assays. Regulatory activity of miR-155 was as...

Chondroid chordoma is a variant of chordoma; it may rarely involve the petrous temporal bone and has a high propensity for recurrence. Chordoma rarely metastasizes but it is, nevertheless, associated with a poor outcome. We report a rare case of chondroid chordoma with extensive recurrence and pulmonary metastases.

Dedifferentiated chordoma, i.e. chordoma with a sarcomatous component, is a rare bone tumor. This case report is of a dedifferentiated chordoma in the sacrococcygeal region in a 50-year-old male. The patient was initially diagnosed as having a leiomyosarcoma at the first medical consultation, but after the tumor recurred it was proven to be a dedifferentiated chordoma. The primary tumor, which ...