Clival Chondroid Chordoma: A Case Report and Review of the Literature

Abstract

Chordomas are rare, slow-growing, and locally aggressive malignant neoplasms derived from primitive notochord remnants. The chondroid variety represents 14% of all chordomas mainly developing in the spheno-occipital region and presenting between the third and fifth decades of life. When developing intracranially, symptoms can range from headaches and neck pain to cranial nerve neuropathies and facial numbness. We illustrate a case of an adolescent woman who presented with excruciating facial pain, otalgia, decreased visual acuity, quadriparesis, headache, nausea, and dysphagia. Radiological studies revealed a large heterogeneous mass in the spheno-occipital region with clivus destruction. Biopsy and histopathology confirmed the diagnosis. Proper identification with prompt surgical resection and adjuvant radiotherapy remains critical to prevent complications.

Systematic Review Comparing Open versus Endoscopic Surgery in Clival Chordomas and a 10-Year Single-Center Experience

“…10 Additionally, chemotherapy has not been shown to be particularly effective in chordomas, with its use being limited to palliative treatment. 11,12 Due to the radioresistant nature of chordomas, high levels of radiation are often needed, which can affect surrounding healthy structures.…”

Chondroid chordoma of the parapharyngeal space: A case report and review of literature

“…Chordomas are rare primary tumor of the bone that arise from the remnants of the embryonic notochord, along the midline craniovertebral axis, most commonly in clivus and sacrococcygeal regions. They are slow-growing and locally aggressive malignant neoplasms [9]. The majority of head and neck chordomas arise in the skull base with a small minority arising along the cervical spine.…”

“…These tumors appear hypointense on T1-weighted images and hyperintense on T2-weighted sequences. Since they are resistant to chemotherapy, the main treatment modality is complete surgical resection usually followed by adjuvant radiotherapy [9]. Molecular targeted therapy (MTT), e.g.…”

Clival chordoma presenting as a parapharyngeal mass: a diagnostic challenge

“…1 During the intracranial growth phase, it can cause a myriad of symptoms. 2 There are scant isolated reports documented regarding the same. 3 As they are difficult to access and give adequate clearance, it requires the close long term monitoring due to their progressive nature.…”