Conventional Skull Base Chordoma in a Ugandan Adult Male, Presenting with Prolonged Headache: A Case Report

Chordomas are uncommon malignant neoplasms arising from notochordal remnants, most commonly affecting the axial skeleton, mainly the skull base and sacrococcygeal region. Skull base chordoma may be asymptomatic or cause progressive headache before focal symptoms prompt imaging. In resource-limited settings, diagnostic work-up is often prolonged as the patient is managed with analgesics without prompt and satisfactory investigation. We report a case of conventional Chordoma arising at the skull base, in a patient with long standing headache.

A 49-year-old man presenting with a more than one-year history of intermittent and progressive worsening headache. After he reported an episode of facial numbness, examination documented preserved facial sensation. There was no other focal neurological clinical abnormality. Head CT imaging revealed an expansile, destructive midline skull base mass (bone erosion) occupying the region of the skull base, sphenoid paranasal sinus extending into the ethmoid. An invasive biopsy through the sphenoid sinuses was obtained for histopathologic evaluation, which showed morphology consistent with chordoma, supported by IHC showing positivity for EMA, pan-cytokeratin, and S100, with negative CK7, CK20, and CD34. The Ki-67 proliferation index was less than 5%. The integrated findings strongly supported conventional chordoma. Brachyury was unavailable. At last documented review, the patient was clinically stable and surgical management was planned; however the patient was lost to follow-up on subsequent attempts.

Progressive headache with a newly reported focal neurological symptom warrants appropriate imaging and specialist assessment. In a destructive midline skull base mass, the combined morphology and immunophenotype strongly support chordoma, while the absence of brachyury confirmation remains a limitation.