Chordoma is a relatively rare primary osseous tumor arising from the remnants of the primitive notochord and located along the axial skeleton.
It is characterized by its slow growth, high frequency to invade and destroy bone by direct extension, local recurrence after surgical excision and distant metastasis. It accounts for 1 to 4% of all malignant bone tumors.
Approximately 50% of chordomas arise in the sacrococcygeal region, 35% at the clivus, and 15% in the vertebral column above the sacrum. The tumor tends to predominate in males, with roughly a 2:1 male-to-female ratio, and occurs predominantly in
the
fifth through seventh decades of life.
Pain is the most common symptom in the chordomas of the spine, and symptoms of constipation, urinary frequency, or nerve root compression may appear before patients present to their physician. Many patients, therefore, are misdiagnosed as having
degenerative disc disease, coccygodynia, or hemorrhoids. A firm, fixed presacral mass can usually be palpated on rectal examination.
Surgical extirpation of the tumor is the only curative procedure and the indicated surgical procedure for sacrococcygeal chordoma is a high sacral amputation, maintaining a cuff of normal tissue over the tumor. Radiation therapy seems to be
effective
with adjuvant or palliative aims.
We report a case of large sacral chordoma detected in a 60-year-old female in the course of a clinical examination, which was successfully treated by high sacral resection by a posterior approach and repair of the sacral bone defect with bone
cement.
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