A rare case of multifocal chordoma involving the lower clivus and multiple spine levels: illustrative case

• Published in: Journal of neurosurgery. Case lessons Vol. 6; no. 9
• Main Authors: Alsavaf, Mohammad Bilal, Salem, Eman H, Jawad, Basit A, Mongkolkul, Kittichai, Carrau, Ricardo L, Prevedello, Daniel M
• Format: Journal Article
• Language: English
• Published: American Association of Neurological Surgeons 28.08.2023
• Subjects: Case Lesson
• ISSN: 2694-1902; 2694-1902
• DOI: 10.3171/CASE23177

BACKGROUND Chordomas are rare bone tumors originating from notochord remnants with a predilection for the median axis of the body, primarily in the sacrococcygeal spine, skull base, and cervical spine. This paper presents the first reported case of simultaneous multilevel chordomas with various enhancements. OBSERVATIONS A 40-year-old woman presented with right-sided otalgia, headache, nasal obstruction, and facial pressure. The patient’s head and spinal magnetic resonance imaging (MRI) demonstrated a midline lesion in the clivus, C2, and T1–5 spinal levels. All lesions were T1 isointense and T2 hyperintense, consistent with chordoma. Interestingly, the clivus lesion was enhancing, whereas the C2 lesion was only slightly enhancing, and the rest of the lesions showed no enhancement. The patient underwent endoscopic endonasal transclival approach surgery for the clival lesion, followed by radiation to the surgical bed and the C2 lesion. The remaining lesions were monitored. Follow-up imaging showed stable disease in the C2 lesion and other lower lesions. The patient’s severe pain in the posterior cervical spine was managed with cervical fusion, pain management, and physical therapy. LESSONS This case underscores the importance of comprehensive spinal MRI in patients with chordomas, as multiple concurrent primaries may be present. The article summarizes the fundamental distinctions between ecchordosis physaliphora and chordoma and casts doubt on the capacity to always differentiate them.