Late recurrence of cervical spine chordoma: illustrative case

BACKGROUND The authors report the case of a 65-year-old male with a 22-year delayed recurrence of a spinal chordoma. The patient originally underwent resection of a C4–5 chordoma in 2001 in Boston followed by proton beam radiotherapy. OBSERVATIONS In August 2023, the patient presented with neck pain, shoulder pain, urinary frequency, and increased mechanical falls and was found to have a recurrence of chordoma. He underwent an anterior cervical corpectomy from C3 to C6, followed by C2–T2 posterior fusion and stereotactic body radiotherapy (SBRT). As of October 2024, there are no suspicious lesions or suspicious uptake on PET CT. LESSONS The case is notable due to the extreme length of time between the initial resection and the recurrence. The frequency of follow-up imaging is determined by how complete the resection was, but in general, chordomas should be imaged every 6 months for the first 5 years. After 5 years, annual MRI for at least 15 years is recommended by the Chordoma Foundation. However, this case shows that recurrence can occur later than 15 years, suggesting that annual MRI with and without contrast for the duration of life may be more prudent to detect late recurrence.