Chordoma in the sella turcica

Abstract

A chordoma is a slow-growing malignant tumour with a metastatic potential developing from notochord remnants. The incidence is about 1 % of all malignant bone tumours with a male to female ration of 1 : 1. Most patients are in their fifth to seventh decades but patients presenting with skull or vertebral tumours are younger. Chordomas have a protracted course and symptoms are usually present for more than 1 year. The symptoms related to the size and location of the tumour are varied: headache, increased intracranial pressure or cranial nerve involvement for sphenooccipital tumor, pain and neurologic symptoms for vertebral tumours and pain, mass, neurologic symptoms or rectal and bladder dysfunction for sacral and sacrococcygeal tumours.

Intracranial chordomas have been described associated with Maffucci's syndrome, hemangiopericytomas, neurofibromatosis or tuberous sclerosis or presenting as a second primary malignant lesion [2].

DOI: 10.3329/pulse.v3i1.6554

Pulse Vol.3(1) July 2009 p33-34