Chordoma

- arises from remnants of primitive notochord found in midline at either end of the spine (sacrum > shnooccipital region) in middle-aged men

Signs & Symptoms:
- gradual onset of pain & usu long duration prior to diagnosis
- large presacral rectal mass
- bowel or bladder dysfunction

Xrays:
- destructive bony lesion associated with an expansile soft tissue mass

Histology:
- polyhedral cells with eosinophilic cytoplasm & many vacuoles which displace the nucleus = physaliphorous cells
- cells disposed in cords, trabeculae or sheets which are separated by abundant mucoid & myxoid matrix

Treatment:
- wide excision (may have to sacrifice sacral roots)
- radiation if not resectable
- frequent metastases to lungs, liver & other tissues