Chordomas originate from remnants of the embryonic notochord. The degenerated notochord is typically embedded within the sphenoid and occipital bones, but some portions may fold ventrally into the pharyngeal wall. Cells at the site where the notochord fuses with the pharyngeal epithelium have the potential for rapid proliferation, and under certain inducing factors, they can form chordomas—a rare, low-grade malignant tumor. Pathologically, the tumor is composed of typical vacuolated cells and a mucoid matrix, characterized by a lobulated structure with cells growing in irregular cords, clusters, or pseudoglandular patterns, and may form syncytial cells with large vacuoles. It can occur at any age but is most common in middle-aged individuals, with a higher incidence in males than females.

bubble_chart Clinical Manifestations

Chordomas are mostly located in the nasopharynx. As the tumor grows, symptoms such as headache, progressive stuffy nose, purulent nasal discharge, snoring, decreased sense of smell, tinnitus, and deafness often occur. Those occurring in the oropharynx or hypopharynx are less common and may cause swallowing discomfort or difficulty breathing. If the tumor extends intracranially, symptoms of cranial nerve involvement may appear. Physical examination may reveal a broad-based, slightly hard mass with normal mucosa on the surface, protruding from the roof of the nasopharynx, posterior pharyngeal wall, or lateral wall.

bubble_chart Diagnosis

Diagnosis must be based on pathological findings. Skull base X-rays, frontal and lateral skull radiographs, CT, and MRI can reveal bone destruction and tumor shadows in areas such as the clivus, sella turcica, and petrous apex, aiding in diagnosis. Differentiation from other pharyngeal tumors, such as nasopharyngeal angiofibroma, nasopharyngeal carcinoma, and craniopharyngioma, is necessary.

The primary treatment is surgical, combined with radiotherapy. The tumor is located at the skull base, making surgery challenging, with significant risks and a high recurrence rate. Extracranial resection or combined craniofacial approach resection may be performed.