Chordoma is a congenital malignant tumor originating from remnants of embryonic notochord tissue. One of its pathological characteristics is lobulated growth with bubble-like nuclei and mucinous stroma. The majority of cases occur in the spine and cranial base, with the sacrococcygeal region being the most commonly affected site.
Clinical Features
The primary symptoms include pain and the presence of a mass. Compression-related symptoms may arise, such as difficulty or incontinence in urination and defecation when the sacral nerves are affected, or corresponding symptoms when the rectum and bladder are compressed. Typical X-ray findings show a unilocular, centrally located, lytic lesion in axial bones, often accompanied by a soft-tissue mass and scattered calcifications. The cortical bone may appear thinned, presenting as an expansile lesion, without periosteal reaction.
Treatment
Surgery is the mainstay of treatment. For tumors that are unresectable or incompletely resected, radiotherapy can be utilized, although the recurrence rate is high. Chemotherapy is generally ineffective.