Chordoma

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