Sacrococcygeal Chordoma: A Diagnostic Challenge

Chordomas are rare, aggressive malignancies arising from notochordal remnants that mainly affect the sacrococcygeal region. Their insidious symptoms often delay the diagnosis. We reported a case of chordoma, emphasizing clinical, histological, and radiological features, and highlighting the diagnostic challenges. We present the case of a 73-year-old woman with progressive low back pain, bilateral thigh radiation, and new-onset urinary and bowel dysfunction culminating in cauda equina syndrome. Neurologic assessment demonstrated preserved lower extremity motor strength (5/5) and symmetric reflexes but identified saddle hypoesthesia (S3-S5 dermatomes). Spine MRI revealed a large sacrococcygeal lesion with T2 hyperintensity, osteolytic destruction, compressing nerve roots, displacing the rectum, and infiltrating pelvic muscles. The patient underwent surgery with near-total resection (R1 margin due to sphincter preservation). Histopathology findings confirmed the diagnosis of chordoma by showing vacuolated physaliphorous cells in myxoid stroma with positivity of panCK and S100. Adjuvant proton therapy was recommended, but the patient declined. During the follow-up, she regained bowel function and pain relief. In this case, sacrococcygeal chordoma was diagnosed at an advanced stage following the onset of neurological deficits, necessitating urgent surgical intervention. MRI was pivotal for both diagnosis and preoperative planning. Immunohistochemistry provided definitive pathological confirmation. Sphincter-preserving resection, in our patient, successfully maintained bowel function. Given our R1 resection margin, long-term surveillance remains crucial due to the high risk of local recurrence.